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Primary Immunodeficiency Syndromes and Their Manifestations in Lymph Nodes

  • H. Cottier
  • R. Kraft
  • F. Meister
Part of the Current Topics in Pathology book series (CT PATHOLOGY, volume 84/2)

Abstract

Originally, the term “immunity” designated a state in which the organism is resistant against commonly disease-inducing infectious agents. This notion was later expanded to include the preservation of “self” by elimination or at least containment of “nonself”, the latter encompassing all sorts of exogenous (“foreign”) materials as well as altered “self” cells/molecules. In the pathogenesis of infectious disease (for review, see Mims 1987), to be successful the attacking microbes must cross outer and/or inner body surfaces and survive in a normally hostile microenvironment, in which various types of host defense mechanism become active. So-called nonspecific immunity, the phylogenetically older principle, relies mainly on phagocytosis and intracellular killing of microorganisms (for references, see Van Der Valk and Herman 1987), especially by granulocytes (Elsbach and Weiss 1985) and monocytes/macrophages (Lowrie et al. 1985). Activation of complement. (reviewed by Williams et al. 1988) and the cooperative action of other microbicidal, microbistatic, and/or cytolytic substances (e.g., certain cytokines) are usually important in this process, which corresponds to an acute inflammatory reaction (for references, see Gallin et al. 1988). The most efficient defense mechanisms, however, are phylogenetically younger and show a marked antigen-directed response. This represents the basis of “specific immunity” which depends on the production of sensitized T lymphocytes [“cell-mediated (specific) immunity”] and/or of antibodies by plasma cells, the differentiated end cells of the B lymphocyte lineage [“humoral (specific) immunity”].

Keywords

Chronic Granulomatous Disease Severe Combine Immunodeficiency Common Variable Immunodeficiency Purine Nucleoside Phosphorylase Primary Immunodeficiency Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Abbas AK (1987) Cellular interactions in the immune response. The roles of B lymphocytes and interleukin-4. Am J Pathol 129: 26–33Google Scholar
  2. Adrian GS, Hutton JJ (1983) Adenosine deaminase messenger RNAs in lymphoblast cell lines derived from leukemic patients and patients with hereditary adenosine deaminase deficiency. J Clin Invest 71: 1649–1660PubMedGoogle Scholar
  3. Akbar AN, Terry L, Timms A, Beverley PCL, Janossy G (1988) Loss of CD45R and gain of UCHL1 reactivity is a feature of primed T cells. J Immunol 140: 2171–2178PubMedGoogle Scholar
  4. Alarcon B, Regueiro JR, Arnaiz-Villena A, Terhorst C (1988) Familial defect in the surface expression of the T-cell receptor-CD3 complex. N Engl J Med 319: 1203–1208PubMedGoogle Scholar
  5. Allende JE (1988) GTP-mediated macromolecular interactions: the common features of different systems. FASEB J 2: 2356–2367PubMedGoogle Scholar
  6. Almawi WY, Pope BL (1986) Induction of suppression by a murine nonspecific suppressor inducer cell line (M1–A5) II The role of prostaglandins. J Immunol 136: 1982–1987PubMedGoogle Scholar
  7. Andersson U, Sander B, Andersson J, Willer G (1988) Concomitant production of different lymphokines in activated T cells. Eur J Immunol 18: 2081–2084PubMedGoogle Scholar
  8. Angelini G, De Preval C, Gorski J, Mach B (1986) High-resolution analysis of the human HLADR polymorphism by hybridization with sequence-specific oligonucleotide probes. Proc Natl Acad Sci USA 83: 4489–4493PubMedGoogle Scholar
  9. Antonelli G, Dianzani F, Van Damme J, Amicucci P, De Marco F, Cefaro A (1988) A macrophage-derived factor different from interleukin 1 and able to induce interferon-y and lymphoproliferation in resting T lymphocytes. Cell Immunol 113: 376–386PubMedGoogle Scholar
  10. Arden Jones MP, Gunapala DE, Matutes E, Catovsky D, Coates ARM (1988) A novel human macrophage-activating factor: Distinction from interferon-y (IFN-y) and granulocyte-macrophage colony-stimulating factor (GMCSF). Cell Immunol 113: 361–375Google Scholar
  11. Asano Y, Tada T (1989) Generation of T cell repertoire. Tivo distinct mechanisms for generation of T suppressor cells, T helper cells, and T augmenting cells. J Immunol 142: 365–373PubMedGoogle Scholar
  12. Asherson GL (1988) Antigen-specific T-helper and -suppressor factors in the control of the immune response. Immunology Suppl 1: 53–56Google Scholar
  13. Aucouturier P, Lacombe C, Brémard C, Lebranchu Y, Seligmann M, Griscelli C, Preud’Homme J-L (1989) Serum IgG subclass levels in patients with primary immunodeficiency syndromes or abnormal susceptibility to infections. Clin Immunol Immunopathol 51: 22–37PubMedGoogle Scholar
  14. Baca ME, Mowat AMCI, Parrott DMV (1989) Immunological studies of NK cell-deficient beige mice II Analysis of T-lymphocyte functions in beige mice. Immunology 66: 131–137PubMedGoogle Scholar
  15. Bach M-A, Launois P (1988) Mechanisms of Mycobacterium leprae-specific T-cell deficiency in lepromatous leprosy. Biochimie 70: 1013–1018PubMedGoogle Scholar
  16. Bar RS, DeLor CJ, Clausen KP, Hurtubise P, Henle W, Hewetson JF (1974) Fatal infectious mononucleosis in a family. N Engl J Med 290: 363–367PubMedGoogle Scholar
  17. Barandun S, Cottier H, Hässig A, Riva G (eds) (1959a) Das Antikörpermangelsyndrom. Schwabe, BaselGoogle Scholar
  18. Barandun S, Stampfli K, Spengler GA, Riva G (1959b) Die Klinik des Antikörpermangelsyndroms. HeIv Med Acta 26: 163–367Google Scholar
  19. Barandun S, Morell A, Skvaril F, Oberdorfer A (1976) Deficiency of x-or.1-type immunoglobulins. Blood 47: 79–89PubMedGoogle Scholar
  20. Barbey-Morel C, Pierce JA, Campbell EJ, Perlmutter DH (1987) Lipopolysaccharide modulates the expression of at proteinase inhibitor and other serine proteinase inhibitors in human monocytes and macrophages. J Exp Med 166: 1041–1054PubMedGoogle Scholar
  21. Barrett MJ, Buckley RH, Schiff SE, Kidd PC, Ward FE (1988) Accelerated development of immunity following transplantation of maternal marrow stem cells into infants with severe combined immunodeficiency and transplacentally acquired lymphoid chimerism. Clin Exp Immunol 72: 118–123PubMedGoogle Scholar
  22. Bästlein C, Burlefinger R, Holzberg E, Voeth C, Garbrecht M, Ottenjann R (1988) Common variable immunodeficiency syndrome and nodular lymphoid hyperplasia in the small intestine. Endoscopy 20: 272–275PubMedGoogle Scholar
  23. Behlke MA, Chou HS, Huppi K, Loh DY (1986) Murine T-cell receptor mutants with deletions of ß-chain variable region genes. Proc Natl Acad Sci USA 83: 767–771PubMedGoogle Scholar
  24. Bélisle C, Sainte-Marie G, Peng F-S (1982) Tridimensional study of the deep cortex of the rat lymph node VI The deep cortex units of the germ-free rat. Am J Pathol 107: 70–78PubMedGoogle Scholar
  25. Benacerraf B, Germain RN (1978) The immune response genes of the major histocompatibility complex. Immunol Rev 38: 70–119PubMedGoogle Scholar
  26. Benestad HB, Laerum OD (1989) The neutrophilic granulocyte. Curr Top Pathol 79: 7–36PubMedGoogle Scholar
  27. Berger M, Sorensen RU (1989) Immune defects associated with recurrent infections. Adv Pediatr Infect Dis 4: 111–138PubMedGoogle Scholar
  28. Berkenbosch F, Van Oers J, Del Rey A, Tilders F, Besedovsky H (1987) Corticotropin-releasing factor-producing neurons in the rat activated by interleukin-1. Science 238: 524–526PubMedGoogle Scholar
  29. Bernier GM, Gunderman JR, Ruymann FB (1972) Kappa-chain deficiency. Blood 40: 795–805PubMedGoogle Scholar
  30. Bias WB, Reveille JD, Beaty TH, Meyers DA, Arnett FC (1986) Evidence that autoimmunity in man is a mendelian dominant trait. Am J Hum Genet 39: 584–602PubMedGoogle Scholar
  31. Bigbee WL, Langlois RG, Swift M, Jensen RH (1989) Evidence for an elevated frequency of in vivo somatic cell mutations in ataxia telangiectasia. Am J Hum Genet 44: 402–408PubMedGoogle Scholar
  32. Blackshear PJ, Nairn AC, Kuo JF (1988) Protein kinases 1988: a current perspective. FASEB J 2: 2957–2969PubMedGoogle Scholar
  33. Bloemendal H, Pieper FR (1989) Intermediate filaments: known structure, unknown function. Biochim Biophys Acta 1007: 245–253PubMedGoogle Scholar
  34. Blore J, Haeney MR (1989) Primary antibody deficiency and diagnostic delay. Br Med J [Clin Res] 298: 516–517Google Scholar
  35. Blue M-L, Daley JF, Levine H, Craig KA, Schlossman SF (1987a) Identification and isolation of a T4+ T8+ cell with high T3 expression in human thymus: a possible late intermediate in thymocyte differentiation. J Immunol 139: 1065–1069PubMedGoogle Scholar
  36. Blue M-L, Levine H, Daley JF, Craig KA, Schlossman SF (1987b) Development of natural killer cells in human thymocyte culture: regulation by accessory cells. Eur J Immunol 17: 669–673PubMedGoogle Scholar
  37. Bonagura VR, Cunningham-Rundles S, Edwards BL, Ilowite NT, Wedgwood JF, Valacer DJ (1989) Common variable hypogammaglobulinemia, recurrent Pneumocystis carinii pneumonia on intravenous y-globulin therapy, and natural killer deficiency. Clin Immunol Immunopathol 51: 216–231PubMedGoogle Scholar
  38. Bonney RJ, Opas EE, Humes JL (1985) Lipoxygenase pathways of macrophages. Fed Proc 44: 2933–2936PubMedGoogle Scholar
  39. Borish L, Audera C, Rosenbaum R, Rocklin RE (1988) Enhancement of the function of neutrophil type-1 and type-3 complement receptors by human leukocyte inhibitory factor ( LIF ). Cell Immunol 113: 320–328PubMedGoogle Scholar
  40. Bosma GC, Davisson MT, Ruetsch NR, Sweet HO, Shultz LD, Bosma MJ (1989) The mouse mutation severe combined immune deficiency (scid) is on chromosome 16. Immunogenetics 29: 54–57PubMedGoogle Scholar
  41. Bottaro A, De Marchi M, De Lange G, Boccazzi C, Caldesi F, Gallina R, Carbonara AO (1989) New types of multiple and single gene deletions in the human IgCH locus Immunogenetics 29: 44–48Google Scholar
  42. Böttger EC, Hoffmann T, Hadding U, Bitter-Suermann D (1985) Influence of genetically inherited complement deficiencies on humoral immune response in guinea pigs. J Immunol 135: 4100–4107PubMedGoogle Scholar
  43. Boyd RL, Oberhuber G, Hala K, Wick G (1984) Obese strain ( OS) chickens with spontaneous autoimmune thyroiditis have a deficiency in thymic nurse cells. J Immunol 132: 718–724PubMedGoogle Scholar
  44. Brandtzaeg P, Karlsson G, Hansson G, Petruson B, Björkander J, Hanson LA (1986) The clinical condition of IgA-deficient patients is related to the proportion of IgD- and IgM-producing cells in their nasal mucosa. Clin Exp Immunol 67: 626–636Google Scholar
  45. Brown AR, Claassen E (1988) Detection of antibody, idiotype, and anti-idiotype forming cells by in situ immunocytochemical staining. J Immunol Methods 109: 235–244PubMedGoogle Scholar
  46. Bruton OC (1952) Agammaglobulinemia. Pediatrics 9: 722–728PubMedGoogle Scholar
  47. Buckley RH (1986) Humoral immunodeficiency. Clin Immunol Immunopathol 40: 13–24PubMedGoogle Scholar
  48. Buckley RH (1987) Immunodeficiency diseases. JAMA 258: 2841–2850PubMedGoogle Scholar
  49. Burks AW, Sampson HA, Buckley RH (1986) Anaphylactic reactions after gamma globulin administration in patients with hypogammaglobulinemia. Detection of IgE antibodies to IgA. N Engl J Med 314: 560–564PubMedGoogle Scholar
  50. Businco L, Di Fazia A, Ziruolo MG et al. (1987) Clinical and immunological findings in four infants with Omenn’s syndrome: a form of severe combined immunodeficiency with phenotypically normal T cells, elevated IgE, and eosinophilia. Clin Immunol Immunopathol 44: 123–133PubMedGoogle Scholar
  51. Butcher EC, Weissman IL (1984) Lymphoid tissues and organs. In: Paul WE (ed) Fundamental immunology. Raven, New York, pp 109–127Google Scholar
  52. Byrne JA, Butler JL, Cooper MD (1988) Differential activation requirements for virgin and memory T cells. J Immunol 141: 3249–3257PubMedGoogle Scholar
  53. Campana D, Coustan-Smith E, Janossy G (1988) Double and triple staining methods for studying the proliferative activity of human B and T lymphoid cells. J Immunol Methods 107: 79–88PubMedGoogle Scholar
  54. Cantani A, Ferrara M (1988) The lung and primary immunodeficiency. Allergol Immunopathol (Madr) 16: 429–437Google Scholar
  55. Cardarelli PM, Pierschbacher MD (1987) Identification of fibronectin receptors on T lymphocytes. J Cell Biol 105: 499–506PubMedGoogle Scholar
  56. Carlsen E, Prydz H (1988) Activation of monocytes — more than one process. Differential effect of cytokines on monocytes. Scand J Immunol 27: 401–404PubMedGoogle Scholar
  57. Casey TT, Posey DH, McCurley TL (1986) OKT4 epitope deficiency in significant proportions of the black population A cause for underestimation of helper/suppressor lymphocyte ratios. Arch Pathol Lab Med 110: 702–704PubMedGoogle Scholar
  58. Casey TT, Cousar JB, Collins RD (1988) A simplified plastic embedding and immunohistologic technique for immunophenotypic analysis of human hematopoietic and lymphoid tissues. Am J Pathol 131: 183–189PubMedGoogle Scholar
  59. Caulfield MJ (1986) A framework hypothesis on the development of an immune system selected by histocompatibility antigens. Scand J Immunol 24: 491–502PubMedGoogle Scholar
  60. Chain BM, Kay PM, Feldmann M (1986) The cellular pathway of antigen presentation: biochemical and functional analysis of antigen processing in dendritic cells and macrophages. Immunology 58: 271–276PubMedGoogle Scholar
  61. Champion S, Imhof BA, Savagner P, Thiery JP (1986) The embryonic thymus produces chemotactic peptides involved in the homing of hemopoietic precursors. Cell 44: 781–790PubMedGoogle Scholar
  62. Chatila T, Wong R, Young M, Miller R, Terhorst C, Geha RS (1989) An immunodeficiency characterized by defective signal transduction in T lymphocytes. N Engl J Med 320: 696–702PubMedGoogle Scholar
  63. Chrétien J, Venet A (1988) Les infections pulmonaires opportunistes dans les déficits immunitaires. Prax Klin Pneumol 42: 197–200PubMedGoogle Scholar
  64. Christenson L, Aebischer P, Galletti PM (1988) Encapsulated thymic epithelial cells as a potential treatment for immunodeficiencies. ?Pans Am Soc Artif Intern Organs 34: 681–686Google Scholar
  65. Chu ET, Rosenwasser LJ, Dinarello CA, Rosen FS, Geha RS (1984) Immunodeficiency with defective T-cell response to interleukin 1. Proc Natl Acad Sci USA 81: 4945–4949PubMedGoogle Scholar
  66. Clark SC, Kamen R (1987) The human hematopoietic colony-stimulating factors. Science 236: 1229–1237PubMedGoogle Scholar
  67. Clement LT, Giorgi JV, Plaeger-Marshall S, Haas A, Stiehm ER, Martin AM (1988a) Abnormal differentiation of immunoregulatory T-lymphocyte subpopulations in the major histocompatibility complex ( MHC) class II antigen deficiency syndrome. J Clin Immunol 8: 503–512PubMedGoogle Scholar
  68. Clement LT, Plaeger-Marshall S, Haas A, Saxon A, Martin AM (1988b) Bare lymphocyte syndrome. Consequences of absent class II major histocompatibility antigen expression for B lymphocyte differentiation and function. J Clin Invest 81: 669–675PubMedGoogle Scholar
  69. Clerici M, Villa ML, Mantovani M, Rugarli C (1988) NK cell activity and monocyte dysfunctions in a patient with common variable hypogammaglobulinemia. J Clin Lab Immunol 27: 143–147PubMedGoogle Scholar
  70. Coleman TH, Forristal J, Kosaka T, West CD (1983) Inherited complement component deficiencies in membranoproliferative glomerulonephritis. Kidney Int 24: 681–690PubMedGoogle Scholar
  71. Collins RD (1985) Lymph node examination. What is an adequate workup? Arch Pathol Lab Med 109: 797–799PubMedGoogle Scholar
  72. Colten HR (1984) Expression of the MHC class III genes. Philos Trans R Soc Lond [Biol] 306: 355–366Google Scholar
  73. Colten HR (1985) Molecular basis of complement deficiency syndromes. Lab Invest 52: 468–474PubMedGoogle Scholar
  74. Conley ME, Cooper MD (1981) Immature IgA B cells in IgA-deficient patients. N Engl J Med 305: 495–497PubMedGoogle Scholar
  75. Conley ME, Puck JM (1988) Definition of the gene loci in X-linked immunodeficiencies. Immunol Invest 17: 425–463PubMedGoogle Scholar
  76. Conley ME, Ziegler MM, Borden S IV, Huff DS, Boyle JT (1988) Multifocal adenocarcinoma of the stomach in a child with common variable immunodeficiency. J Pediatr Gastroenterol Nutr 7: 456–460PubMedGoogle Scholar
  77. Cooper MD (1987) Current concepts B lymphocytes Normal development and function. N Engl J Med 317: 1452–1456PubMedGoogle Scholar
  78. Cooper NR, Nemerow GR (1985) Complement effector mechanisms in health and disease. J Invest Dermatol 85 [1 Suppl]: 39s - 46sPubMedGoogle Scholar
  79. Cottier H (1957) Zur Histopathologie des Antikörpermangelsyndroms. In: Transactions of the 6th Congress of the European Society of Haematology, S. Karger, Basel, pp 41–46Google Scholar
  80. Cottier H, Barandun S (1959) Morphologische Pathologie des Antikörpermangelsyndroms. Helv Med Acta 26: 461–539PubMedGoogle Scholar
  81. Cottier H, Hassig A (1986) Introduction: immunodeficiency and ineffective immune responses. In: Morell A, Nydegger UE (eds) Clinical use of intravenous immunoglobulins. Academic, London, pp 1–13Google Scholar
  82. Cottier H, Hess MW, Roos B, Grétillat PA (1969) Regeneration, Hyperplasie and Onkogenese der lymphoretikularen Organe. In: Altmann H-W, Büchner F, Cottier H et al. (eds) Handbuch der Allgemeinen Pathologie, vol 6/2. Springer, Berlin Heidelberg New York, pp 495–766Google Scholar
  83. Cottier H, Turk J, Sobin L (1972) A proposal for a standardized system of reporting human lymph node morphology in relation to immunological function. Bull WHO 47: 375–408PubMedGoogle Scholar
  84. Cottier H, Hess MW, Keller H-U (1980) Structural basis for lymphoid tissue functions: established and disputable sites of antigen-cell and cell-to-cell interactions in vivo. Monogr Allergy 16: 50–71PubMedGoogle Scholar
  85. Cunningham-Rundles C, Siegal FP, Smithwick EM et al. (1984) Efficacy of intravenous immunoglobulin in primary humoral immunodeficiency disease. Ann Intern Med 101: 435–439PubMedGoogle Scholar
  86. Curnutte JT, Scott PJ, Mayo LA (1989) Cytosolic components of the respiratory burst oxidase: resolution of four components, two of which are missing in complementing types of chronic granulomatous disease. Proc Natl Acad Sci USA 86: 825–829PubMedGoogle Scholar
  87. Cuthbertson RA, Klintworth GK (1988) Transgenic mice — a gold mine for furthering knowledge in pathobiology. Lab Invest 58: 484–502PubMedGoogle Scholar
  88. Czuprynski CJ, Canono BP, Henson PM, Campbell PA (1985) Genetically determined resistance to listeriosis is associated with increased accumulation of inflammatory neutrophils and macrophages which have enhanced listericidal activity. Immunology 55: 511–518PubMedGoogle Scholar
  89. Dahinden CA, Zingg J, Maly FE, de Weck AL (1988) Leukotriene production in human neutrophils primed by recombinant human granulocyte/macrophage colony-stimulating factor and stimulated with the complement component C5A and FMLP as second signals. J Exp Med 167: 1281–1295PubMedGoogle Scholar
  90. De Mey J, Hacker GW, De Waele M, Springall DR (1986) Gold probes in light microscopy. In: Polak JM, Van Noorden S (eds) Immunocytochemistry. Modern methods and applications. 2nd edn. Wright, Bristol, pp 71–88Google Scholar
  91. Denning SM, Tuck DT, Singer KH, Haynes BF (1987) Human thymic epithelial cells function as accessory cells for autologous mature thymocyte activation. J Immunol 138: 680–686PubMedGoogle Scholar
  92. De Vaal OM, Seynhaeve V (1959) Reticular dysgenesia. Lancet II: 1123–1125Google Scholar
  93. Dexter TM (1987) Growth factors involved in haemopoiesis. J Cell Sci 88: 1–6PubMedGoogle Scholar
  94. DiGeorge AM (1965) A new concept of the cellular basis of immunity. Discussion. J Pediatr 67: 907–908Google Scholar
  95. Dimanche-Boitrel M, Guyot A, De Saint-Basile G, Fischer A, Griscelli C, Lisowska-Grospierre B (1988) Heterogeneity in the molecular defect leading to the leukocyte adhesion deficiency. Eur J Immunol 18: 1575–1579PubMedGoogle Scholar
  96. Dinarello CA, Mier JW (1987) Lymphokines. N Engl J Med 317: 940–945PubMedGoogle Scholar
  97. Dinauer MC, Orkin SH (1988a) Chronic granulomatous disease. Molecular genetics. Hematol Oncol Clin North Am 2: 225–240PubMedGoogle Scholar
  98. Dinauer MC, Orkin SH (1988b) Molecular genetics of chronic granulomatous disease. Immunodeficiency Rev 1: 55–69Google Scholar
  99. Djeu JY, Blanchard DK (1987) Regulation of human polymorphonuclear neutrophil (PMN) activity against Candida albicans by large granular lymphocytes via release of a PMN-activating factor. J Immunol 139: 2761–2767PubMedGoogle Scholar
  100. Doi S, Saiki O, Tanaka T et al. (1988) Cellular and genetic analyses of IL-2 production and IL-2 receptor expression in a patient with familial T-cell-dominant immunodeficiency. Clin Immunol Immunopathol 46: 24–36PubMedGoogle Scholar
  101. Donnér M, Schwartz M, Carlsson KU, Holmberg L (1988) Hereditary X-linked thrombocytopenia maps to the same chromosomal region as the Wiskott-Aldrich syndrome. Blood 72: 1849–1853PubMedGoogle Scholar
  102. Dorshkind K (1988) Interleukin-1 inhibition of B lymphopoiesis is reversible. Blood 72: 2053–2055PubMedGoogle Scholar
  103. Dosch HM, Mansour A, Cohen A, Shore A, Gelfand EW (1980) Inhibition of suppressor T-cell development following deoxyguanosine administration. Nature 285: 494–496PubMedGoogle Scholar
  104. Drayson MT, Ford WL (1984) Afferent lymph and lymph borne cells: their influence on lymph node function Immunobiology 168: 362–379PubMedGoogle Scholar
  105. Durham JC, Stephens DS, Rirnland D, Nassar VH, Spira TJ (1987) Common variable hypogammaglobulinemia complicated by an unusual T-suppressor/cytotoxic cell lymphoma. Cancer 59: 271–276PubMedGoogle Scholar
  106. Duse M, Plebani A (1987) Intractable seizures and IgG subclass deficiencies: response to gamma globulins (IVIG). Immunopathol Immunother Letter 3: 2–11Google Scholar
  107. Elsbach P, Weiss J (1985) Oxygen-dependent and oxygen-independent mechanisms of microbicidal activity of neutrophils. Immunol Lett 11: 159–163PubMedGoogle Scholar
  108. Enerbäck L, Norrby K (1989) The mast cells. Curr Top Pathol 79: 169–204PubMedGoogle Scholar
  109. Epstein CJ, Hofmeister BG, Yee D, Smith SA, Philip R, Cox DR, Epstein LB (1985) Stem cell deficiencies and thymic abnormalities in fetal mouse trisomy 16. J Exp Med 162: 695–712PubMedGoogle Scholar
  110. Erikson J, Martinis J, Croce CM (1981) Assignment of the genes for human í immunoglobulin chains to chromosome 22. Nature 294: 173–175PubMedGoogle Scholar
  111. Eskola J, Savilahti E, Nikoskelainen J, Ruuskanen O (1989) Regulatory T-cell function in primary humoral immunodeficiency states. J Clin Lab Immunol 28: 55–60PubMedGoogle Scholar
  112. Evans R, Duffy TM, Shultz LD (1988) The immunological mouse mutants nude (nu) and rhino (hrrh) generate cytotoxic effector cells following adoptive immunotherapy but fail to reject a transplanted tumor. Cancer Immunol Immunother 26: 35–42PubMedGoogle Scholar
  113. Evans SS, Ozer H (1987) Enhancement of a human antibody response in vitro mediated by interaction of interferon-a with T lymphocytes. J Immunol 138: 2451–2456PubMedGoogle Scholar
  114. Facchetti F, De Wolf-Peeters C, Mason DY, Pulford K, Van den Oord JJ, Desmet VJ (1988) Plasmacytoid T cells Immunohistochemical evidence for their monocyte/macrophage origin. Am J Pathol 133: 15–21PubMedGoogle Scholar
  115. Fasth A (1982) Primary immunodeficiency disorders in Sweden: cases among children, 1974–1979. J Clin Immunol 2: 86–92PubMedGoogle Scholar
  116. Fearon ER, Kohn DB, Winkelstein JA, Vogelstein B, Blaese RM (1988) Carrier detection in the Wiskott Aldrich syndrome. Blood 72: 1735–1739PubMedGoogle Scholar
  117. Feldbush TL, Hobbs MV, Severson CD, Ballas ZK, Weiler JM (1984) Role of complement in the immune response. Fed Proc 43: 2548–2552PubMedGoogle Scholar
  118. Fenton MJ, Vermeulen MW, Clark BD, Webb AC, Auron PE (1988) Human pro-IL-1ß gene expression in monocytic cells is regulated by two distinct pathways. J Immunol 140: 2267–2273PubMedGoogle Scholar
  119. Festenstein H, Awad J, Hitman GA et al. (1986) New HLA DNA polymorphisms associated with autoimmune diseases. Nature 322: 64–67PubMedGoogle Scholar
  120. Field KG, Olsen GJ, Lane DJ et al. (1988) Molecular phylogeny of the animal kingdom. Science 239: 748–753PubMedGoogle Scholar
  121. Filipovich AH, Spector BD, Kersey J (1980) Immunodeficiency in humans as a risk factor in the development of malignancy. Prey Med 9: 252–259Google Scholar
  122. Fischer A, Lisowska-Grospierre B, Anderson DC, Springer TA (1988) Leukocyte adhesion deficiency: molecular basis and functional consequences. Immunodeficiency Rev 1: 39–54Google Scholar
  123. Fleisher TA, White RM, Broder S et al. (1980) X-linked hypogammaglobulinemia and isolated growth hormone deficiency. N Engl J Med 302: 1429–1434PubMedGoogle Scholar
  124. Flohé L, Giertz H (1987) Endotoxins, arachidonic acid, and superoxide formation. Rev Infect Dis 9 [Suppl 5]: S553 - S561PubMedGoogle Scholar
  125. Forsyth K, Matthews C, Seshadri R, Heddle R (1988) Wiskott-Aldrich syndrome in identical twins: abnormality of CD4 and CD8 positive lymphocytes. Aust NZ J Med 18: 73–76Google Scholar
  126. Forsyth KD, Levinsky RJ (1989) Role of the LFA-1 adhesion glycoprotein in neutrophil adhesion to endothelium and plastic surfaces. Clin Exp Immunol 75: 265–268PubMedGoogle Scholar
  127. Fossum S (1989) The life history of dendritic leukocytes (DL). Curr Top Pathol 79: 101–124PubMedGoogle Scholar
  128. Franceschi C, Chiricolo M, Licastro F, Zannotti M, Masi M, Mocchegiani E, Fabris N (1988) Oral zinc supplementation in Down’s syndrome: restoration of thymic endocrine activity and of some immune defects. J Ment Defic Res 32: 169–181PubMedGoogle Scholar
  129. Frank MM, Joiner K, Hammer C (1987) The function of antibody and complement in the lysis of bacteria. Rev Infect Dis 9 [Suppl 5]: S537–S545PubMedGoogle Scholar
  130. Fràter-Schröder M, Hitzig WH, Sacher M (1981) Inheritance of transcobalamin II (TC II) in two families with TC II deficiency and related immunodeficiency. J Inherited Metab Dis 4: 165–166Google Scholar
  131. Freedman AS, Freeman G, Whitman J, Segil J, Daley J, Nadler LM (1989) Studies of in vitro activated CD5+ B cells. Blood 73: 202–208PubMedGoogle Scholar
  132. Freedom RM, Rosen FS, Nadas AS (1972) Congenital cardiovascular disease and anomalies of the third and fourth pharyngeal pouch. Circulation 156: 165–172Google Scholar
  133. Frenkel J, Neijens HJ, Den Hollander JC, WolversTettero ILM, Van Dongen JJM (1988) Oligoclonal T cell proliferative disorder in combined immunodeficiency. Pediatr Res 24: 622–627PubMedGoogle Scholar
  134. Friedl HP, Till GO, Trentz O, Ward PA (1989) Roles of histamine, complement and xanthine oxidase in thermal injury of skin. Am J Pathol 135: 203–217PubMedGoogle Scholar
  135. Fries LF, Siwik SA, Malbran A, Frank MM (1987) Phagocytosis of target particles bearing C3bIgG covalent complexes by human monocytes and polymorphonuclear leucocytes. Immunology 62: 45–51PubMedGoogle Scholar
  136. Fujita K, Kobayashi K, Okino F (1988) Juvenile rheumatoid arthritis in two siblings with congenital leucocyte adhesion deficiency. Eur J Pediatr 148: 118–119PubMedGoogle Scholar
  137. Galbraith GMP (1988) Chemotactic peptide-induced arachidonic acid mobilization in human polymorphonuclear leukocytes. Am J Pathol 133: 347–354PubMedGoogle Scholar
  138. Gallin JI, Goldstein IM, Snyderman R (eds) (1988) Inflammation. Basic principles and clinical correlates. Raven, New YorkGoogle Scholar
  139. Gao L, Malkovsky M, Webster ADB, Asherson GL (1985) Impaired lymphokine-activated killer-cell activity in patients with hypogammaglobulinaemia. Lancet 2: 340PubMedGoogle Scholar
  140. Garcia-Penarrubia P, Koster FT, Kelley RO, McDowell TD, Bankhurst AD (1989) Antibacterial activity of human natural killer cells. J Exp Med 169: 99–113PubMedGoogle Scholar
  141. Gathings WE, Kubagawa H, Cooper MD (1981) A distinctive pattern of B cell immaturity in perinatal humans. Immunol Rev 57: 107–126PubMedGoogle Scholar
  142. Gatti RA, Berkel I, Boder E et al. (1988) Localization of an ataxia-telangiectasia gene to chromosome 11g22–23. Nature 336: 577–580PubMedGoogle Scholar
  143. Gavinski S, Woloschak GE (1989) Expression of viral and virus-like elements in DNA repair-deficient/immunodeficient “wasted” mice. J Immunol 142: 1861–1866PubMedGoogle Scholar
  144. Geha RS, Leung DYM (1989) Hyperimmunoglobulin E syndrome. Immunodeficiency Rev 1: 155–172Google Scholar
  145. Geha RS, Reinherz E (1983) Identification of circulating maternal T and B lymphocytes in uncomplicated severe combined immunodeficiency by HLA typing of subpopulations of T cells separated by the fluorescence-activated cell sorter and of Epstein Barr virus-derived B cell lines. J Immunol 130: 2493–2495PubMedGoogle Scholar
  146. Geisler C, Pallesen G, Platz P et al. (1989) Novel primary thymic defect with T lymphocytes expressing y8 T cell receptor. J Clin Pathol 42: 705–711PubMedGoogle Scholar
  147. Geme StJW Jr, Prince JT, Burke BA, Good RA, Krivit W (1965) Impaired cellular resistance to herpes-simplex virus in Wiskott-Aldrich syndrome. N Engl J Med 273: 229–234Google Scholar
  148. Gerritsen EJA, Van den Berg H, Langlois van den Bergh R, Schellekens PTA, Van Tol MJD, Dooren LJ, Vossen JM (1988) Gecombineerde immunologische deficiëntie. Tijdschr Kindergeneeskd 56: 196–205PubMedGoogle Scholar
  149. Geursen RG, Weidmann E (1988) Il sospetto diagnostico di immunodeficienza. Ric Clin Lab 18 [Suppl 1]: 27–37PubMedGoogle Scholar
  150. Giblett ER, Anderson JE, Cohen F, Pollara B, Meuwissen HJ (1972) Adenosine-deaminase deficiency in two patients with severely impaired cellular immunity. Lancet II: 1067–1069Google Scholar
  151. Giblett ER, Ammann AJ, Wara DW, Sandman R, Diamond LK (1975) Nucleoside-phosphorylase deficiency in a child with severely defective T-cell immunity and normal B-cell immunity. Lancet 1: 1010–1013PubMedGoogle Scholar
  152. Glanzmann E, Riniker P (1950) Essentielle Lymphocytophthise. Ein neues Krankheitsbild aus der Säuglingspathologie. Ann Paediatr Int Rev Pediatr (Jahrbuch für Kinderheilkunde. Schweizerische Gesellschaft für Pädiatrie 175: 1–32Google Scholar
  153. Glass D, Raum D, Gibson D, Stillman JS, Schur PH (1976) Inherited deficiency of the second component of complement. Rheumatic disease associations. J Clin Invest 58: 853–861PubMedGoogle Scholar
  154. Gönczy P, Reith W, Barras E, Lisowska-Grospierre B, Griscelli C, Hadam MR, Mach B (1989) Inherited immunodeficiency with a defect in a major histocompatibility complex class II promoter-binding protein differs in the chromatin structure of the HLA-DRA gene. Mol Cell Biol 9: 296–302PubMedGoogle Scholar
  155. Good MF, Berzofsky JA, Maloy WL, Hayashi Y, Fujii N, Hockmeyer WT, Miller LH (1986) Genetic control of the immune response in mice to a Plasmodium falciparum sporozoite vaccine. Widespread nonresponsiveness to single malaria T epitope in highly repetitive vaccine. J Exp Med 164: 655–660PubMedGoogle Scholar
  156. Good RA (1954) Society transactions. Society for pediatric research. Agammaglobulinemia: an experimental study. Am J Dis Child 88: 625–626Google Scholar
  157. Good RA, Varco RL (1955) A clinical and experimental study of agammaglobulinemia. J Lancet 75: 245–271PubMedGoogle Scholar
  158. Good RA, Dalmasso AP, Martinez C, Archer OK, Pierce JC, Papermaster BW (1962) The role of the thymus in development of immunologic capacity in rabbits and mice. J Exp Med 116: 773–795PubMedGoogle Scholar
  159. Gordon DL, Rice J, Finlay-Jones JJ, McDonald PJ, Hostetter MK (1988) Analysis of C3 deposition and degradation on bacterial surfaces after opsonization. J Infect Dis 157: 697–704PubMedGoogle Scholar
  160. Gordon J, Paraskevas F, Lee S-T (1985) T-cell function in B-lymphocyte-deprived mice. Cell Immunol 94: 100–112PubMedGoogle Scholar
  161. Goverman J, Hunkapiller T, Hood L (1986) A speculative view of the multicomponent nature of T cell antigen recognition. Cell 45: 475–484PubMedGoogle Scholar
  162. Graziano RF, Looney RJ, Shen L, Fanger MW (1989) FcyR-mediated killing by eosinophils. J Immunol 142: 230–235PubMedGoogle Scholar
  163. Green AR (1989) Peptide regulatory factors: multifunctional mediators of cellular growth and differentiation. Lancet 1: 705–707PubMedGoogle Scholar
  164. Greenberg F, Elder FFB, Haffner P, Northrup H, Ledbetter DH (1988) Cytogenetic findings in a prospective series of patients with DiGeorge anomaly. Am J Hum Genet 43: 605–611PubMedGoogle Scholar
  165. Gregersen PK, Shen M, Song Q-L et al. (1986) Molecular diversity of HLA-DR4 haplotypes. Proc Natl Acad Sci USA 83: 2642–2646PubMedGoogle Scholar
  166. Grierson HL, Skare J, Purtilo DT (1989) Phenotypes of males in the registry of the X-linked lymphoproliferative syndrome (XLP). Lab Invest 89: 35AGoogle Scholar
  167. Griscelli C, Lisowska-Grospierre B (1989) Combined immunodeficiency with defective expression in MHC class II genes. Immunodeficiency Rev 1: 135–153Google Scholar
  168. Griscelli C, Lisowska-Grospierre B, Le Deist F et al. (1989) Combined immunodeficiency with abnormal expression of MHC class II genes. Clin Immunol Immunopathol 50: S140 - S148PubMedGoogle Scholar
  169. Haar JL, Popp JD, Shultz LD (1989) Defective in vitro migratory capacity of bone marrow cells from viable motheaten mice in response to normal thymus culture supernatants. Exp Hematol 17: 21–24PubMedGoogle Scholar
  170. Hagiya M, Davis DD, Shultz LD, Sakano H (1986) Non-germ-line elements (NGE) are present in the T cell receptor ß-chain genes isolated from the mutant mouse, motheaten (me/me). J Immunol 136: 2697–2700PubMedGoogle Scholar
  171. Halperin JA, Nicholson-Weller A (1989) Paroxysmal nocturnal hemoglobinuria. A complement-mediated disease. Complement Inflamm 6: 65–72PubMedGoogle Scholar
  172. Hamblin AS (1988) Lymphokines and interleukins. Immunology Suppl 1: 39–41Google Scholar
  173. Hammarström L, Lönnqvist B, Ringdén O, Smith CIE, Wiebe T (1985) Transfer of IgA deficiency to a bone-marrow-grafted patient with aplastic anaemia. Lancet 1: 778–781PubMedGoogle Scholar
  174. Hanson LA, Björkander J, Söderström R, Söderström T (1988) Clinical significance of IgG subclass and IgA deficiency. In: Webster ADB (ed) Immunodeficiency and disease. Kluwer Academic, Dordrecht, pp 99–111Google Scholar
  175. Harland C, Shah T, Webster AD, Peters TJ (1988) Dipeptidyl peptidase IV — subcellular localization, activity and kinetics in lymphocytes from control subjects, immunodeficient patients and cord blood. Clin Exp Immunol 74: 201–205PubMedGoogle Scholar
  176. Hässig A, Cottier H (1986) Hypothesis: selective ineffective humoral immune responsiveness as a possible cause of certain chronic inflammatory disorders. Theoretical considerations and rationale for treatment. Klin Wochenschr 64: 401–404PubMedGoogle Scholar
  177. Hauptmann G, Tâppeiner G, Schifferli JA (1988) Inherited deficiency of the fourth component of human complement. Immunodeficiency Rev 1: 3–22Google Scholar
  178. Hayakawa H, Iwata T, Yata J, Kobayashi N (1981) Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome. J Clin Immunol 1: 31–39PubMedGoogle Scholar
  179. Hayakawa H, Matsumoto S, Kobayashi N, Yata J (1987) Genetically determined immunodeficiencies. In: Good RA, Lindenlaub E (eds) The nature, cellular, and biochemical basis and management of immunodeficiencies, Symposia Medica Hoechst 21. Schattauer, Stuttgart, pp 39–60Google Scholar
  180. Hayashi S-I, Witte PL; Kincade PW (1989) The XID mutation affects hemopoiesis in long term cultures of murine bone marrow. J Immunol 142: 444–451PubMedGoogle Scholar
  181. Haynes BF, Warren RW, Buckley RH et al. (1983) Demonstration of abnormalities in expression of thymic epithelial surface antigens in severe cellular immunodeficiency diseases. J Immunol 130: 1182–1188PubMedGoogle Scholar
  182. Hedrick SM, Cohen DI, Nielsen EA, Davis MM (1984) Isolation of cDNA clones encoding T cell-specific membrane-associated proteins. Nature 308: 149–153PubMedGoogle Scholar
  183. Henderson LM, Arlett CF, Harcourt SA, Lehmann AR, Broughton BC (1985) Cells from an immunodeficient patient (46BR) with a defect in DNA ligation are hypomutable but hypersensitive to the induction of sister chromatid exchanges. Proc Natl Acad Sci USA 82: 2044–2048PubMedGoogle Scholar
  184. Hertel-Wulff B, Strober S (1988) Immunosuppressive lymphokine derived from natural suppressor cells. J Immunol 140: 2633–2638PubMedGoogle Scholar
  185. Herzog WR, Meade R, Pettinicchi A, Ptak W, Askenase PW (1989) Nude mice produce a T cell-derived antigen-binding factor that mediates the early component of delayed-type hypersensitivity. J Immunol 142: 1803–1812PubMedGoogle Scholar
  186. Hess MW (1968) Experimental thymectomy. Possibilities and limitations. Exp Med Pathol Klin 25: 1–105Google Scholar
  187. Hess MW, Mueller C, Schaffner T, Gerber HA, Eggli P, Cottier H (1985) Thymic lymphopoiesis: protected from, or influenced by, external stimulation? Ann NY Acad Sci 459: 14–21PubMedGoogle Scholar
  188. Hiernaux JR, Goidl EA, Martin McEvoy SJ, Stashak PW, Baker PJ, Holmes KL (1989) Characterization of the immunodeficiency of RIIIS/J mice. I. Association with the CD5 (Ly-1) B cell lineage. J Immunol 142: 1813–1817PubMedGoogle Scholar
  189. High KA (1988) Antithrombin III, protein C, and protein S. Naturally occurring anticoagulant proteins. Arch Pathol Lab Med 112: 28–36PubMedGoogle Scholar
  190. Hirayama K, Matsushita S, Kikuchi I, luchi M, Ohta N, Sasazuki T (1987) HLA-DQ is epistatic to HLA-DR in controlling the immune response to schistosomal antigen in humans Nature 327: 426–430PubMedGoogle Scholar
  191. Hirschhorn R, Ellenbogen A (1986) Genetic heterogeneity in adenosine deaminase (ADA) deficiency: five different mutations in five new patients with partial ADA deficiency. Am J Hum Genet 38: 13–25PubMedGoogle Scholar
  192. Hobbs JR, Milner RDG, Watt PJ (1967) Gamma-M deficiency predisposing to meningococcal septicaemia. Br Med J 4: 583–586PubMedGoogle Scholar
  193. Hoffman T, Winchester R, Schulkind M, Frias JL, Ayoub EM, Good RA (1977) Hypoimmunoglobulinemia with normal T cell function in female siblings. Clin Immunol Immunopathol 7: 364–371PubMedGoogle Scholar
  194. Hong R, Horowitz S, Moen R et al. (1987) Thymus and B cell reconstitution in severe combined immunodeficiency after transplantation of monoclonal antibody depleted parental mismatched bone marrow. Bone Marrow Transplant 1: 405–409PubMedGoogle Scholar
  195. Hood L, Kronenberg M, Hunkapiller T (1985) T cell antigen receptors and the immunoglobulin supergene family. Cell 40: 225–229PubMedGoogle Scholar
  196. Hormaeche CE, Harrington KA, Joysey HS (1985) Natural resistance to salmonellae in mice: con- trol by genes within the major histocompatibility complex. J Infect Dis 152: 1050–1056PubMedGoogle Scholar
  197. Huber R (1986) Structural basis for antigen-antibody recognition. Science 233: 702–703PubMedGoogle Scholar
  198. Ichihara Y, Matsuoka H, Tsuge I, Okada J, Torii S, Yasui H, Kurosawa Y (1988) Abnormalities in DNA rearrangements of immunoglobulin gene loci in precursor B cells derived from X-linked agammaglobulinemia patient and a severe combined immunodeficiency patient. Immunogenetics 27: 330–337PubMedGoogle Scholar
  199. Insel RA, Anderson PW (1986) Response to oligosaccharide-protein conjugate vaccine against Hemophilus influenzae b in two patients with IgG2 deficiency unresponsive to capsular polysaccharide vaccine. N Engl J Med 315: 499–503PubMedGoogle Scholar
  200. Ioachim HL (1987) Neoplasms associated with immune deficiencies. Pathol Annu 22 (Pt 2): 177–222PubMedGoogle Scholar
  201. Iwata T, Incefy GS, Good RA et al. (1983) Circulating thymic hormone levels in severe combined immunodeficiency. Clin Exp Immunol 53: 1–9PubMedGoogle Scholar
  202. Janossy G, Campana D, Akbar A (1989a) Kinetics of T lymphocyte development. Curr Top Pathol 79: 59–99PubMedGoogle Scholar
  203. Janossy G, Bofill M, Rowe D, Muir J, Beverley PCL (1989b) The tissue distribution of T lymphocytes expressing different CD45 polypeptides. Immunology 66: 517–525PubMedGoogle Scholar
  204. Jaspers NGJ, Bootsma D (1982) Genetic heterogeneity in ataxia-telangiectasia studied by cell fusion. Proc Natl Acad Sci USA 79: 2641–2644PubMedGoogle Scholar
  205. Jeannet M (1986) Système HLA et contrôle génétique de la réponse immune cellulaire. Schweiz Med Wochenschr 116: 1464–1466PubMedGoogle Scholar
  206. Johnson E, Hetland G (1988) Mononuclear phagocytes have the potential to synthesize the complete functional complement system. Scand J Immunol 27: 489–493PubMedGoogle Scholar
  207. Jondal M (1987) The human NK cell — a short over-view and a hypothesis on NK recognition. Clin Exp Immunol 70: 255–262PubMedGoogle Scholar
  208. Jouan H, Le Deist F, Nézelof C (1987) Omenn’s syndrome — pathologic arguments in favor of a graft versus host pathogenesis. Hum Pathol 18: 1101–1108PubMedGoogle Scholar
  209. Junker AK, Chan KW, Massing BG (1989) Clinical and immune recovery from Omenn syndrome after bone marrow transplantation. J Pediatr 114: 596–600PubMedGoogle Scholar
  210. Kakinuma M, Yamamoto K (1985) Strain differences in lung granuloma formation in response to a BCG cell-wall vaccine in mice. Demonstration of two types of low responders. Immunology 55: 91–95PubMedGoogle Scholar
  211. Kamel-Reid S, Dick JE (1988) Engraftment of immune-deficient mice with human hematopoietic stem cells. Science 242: 1706–1709PubMedGoogle Scholar
  212. Kaminsky SG, Milisauskas V, Chen PB, Nakamura I (1987) Defective differentiation of natural killer cells in SJL mice. Role of the thymus. J Immunol 138: 1020–1025PubMedGoogle Scholar
  213. Kaplan AP, Silverberg M (1987) The coagulation-kinin pathway of human plasma. Blood 70: 1–15PubMedGoogle Scholar
  214. Kappler JW, Staerz U, White J, Marrack PC (1988) Self-tolerance eliminates T cells specific for Mls-modified products of the major histocompatibility complex. Nature 332: 35–40PubMedGoogle Scholar
  215. Karagogeos D, Wortis HH (1987) Thymus grafts induce B cell development in nude, X-linked immune deficient mice. Eur J Immunol 17: 141–144PubMedGoogle Scholar
  216. Karlsson G, Brandtzaeg P, Hansson G, Petruson B, Björkander J, Hanson LA (1987) Immunohistochemical study of nasal mucosa in patients with common variable immunodeficiency. Int Arch Allergy Appl Immunol 82: 481–482PubMedGoogle Scholar
  217. Keever CA, Flomenberg N, Brochstein J et al. (1988) Tolerance of engrafted donor T cells following bone marrow transplantation for severe combined immunodeficiency. Clin Immunol Immunopathol 48: 261–276PubMedGoogle Scholar
  218. Keppen LD, Fasules JW, Burks AW, Gollin SM, Sawyer JR, Miller CH (1988) Confirmation of autosomal dominant transmission of the DiGeorge malformation complex. J Pediatr 113: 506–508PubMedGoogle Scholar
  219. Kersey JH, Shapiro RS, Filipovich AH (1988) Relationship of immunodeficiency to lymphoid malignancy. Pediatr Infect Dis J 7:S10–SI2Google Scholar
  220. Kindler V, Sappino A-P, Grau GE, Piguet P-F, Vassalli P (1989) The inducing role of tumor necrosis factor in the development of bactericidal granulomas during BCG infection. Cell 56: 731–740PubMedGoogle Scholar
  221. Kinlen LJ, Webster ADB, Bird AG, Haile R, Peto J, Soothill JF, Thompson RA (1985) Prospective study of cancer in patients with hypogammaglobulinaemia. Lancet 1: 263–266PubMedGoogle Scholar
  222. Kirsch IR, Morton CC, Nakahara K, Leder P (1982) Human immunoglobulin heavy chain genesmap to a region of translocations in malignant B lymphocytes. Science 216: 301–303PubMedGoogle Scholar
  223. Kishi T, Sakura N, Ito K, Usui T (1987) High risk screening of adenosine deaminase and purine nucleoside phosphorylase deficiency in Japan. Acta Paediatr Jpn Overseas Ed 29: 152–154Google Scholar
  224. Kitas GD, Salmon M, Emery P (1989) Immune dysfunction in patients not responding to hepatitis B vaccination. Lancet 1: 551PubMedGoogle Scholar
  225. Klaus GGB, Humphrey JH (1977) The generation of memory cells. I. The role of C3 in the generation of B memory cells. Immunology 33: 31–40PubMedGoogle Scholar
  226. Knowles DM II, Pelicci P-G, Dalla-Favera R (1986) T-cell receptor beta chain gene rearrangements: genetic markers of T-cell lineage and clonality. Hum Pathol 17: 546–551PubMedGoogle Scholar
  227. Konttinen YT, Segerberg-Konttinen M, Nordström D, Bergroth V, Scheinin T, Saari H (1988) An immunoperoxidase-autoradiography double labeling method for analysis of lymphocyte activation markers and DNA synthesis. J Immunol Methods 110: 19–27PubMedGoogle Scholar
  228. Kraft R, Cottier H (1986) Die Funktion der Makrophagen unter besonderer Berücksichtigung der Immunregulation. Nova Acta Leopoldina NF 59, 263: 45–69Google Scholar
  229. Kraft R, Bachmann M, Bachmann K, Buerki H, Hess MW, Cottier H, Stoner RD (1987) Satisfactory primary tetanus antitoxin responses but markedly reduced germinal centre formation in first draining lymph nodes of ageing mice. Clin Exp Immunol 67: 447–453PubMedGoogle Scholar
  230. Kraft R, Buerki H, Schweizer T, Hess MW, Cottier H, Stoner RD (1989) Tetanus toxoid complexed with heterologous antibody can induce germinal centre formation and B cell memory in mice without evoking a detectable anti-toxin response. Clin Exp Immunol 76: 138–143PubMedGoogle Scholar
  231. Krieger JI, Grammer SF, Grey HM, Chesnut RW (1985) Antigen presentation by splenic B cells: resting B cells are ineffective, whereas activated B cells are effective accessory cells for T cell responses. J Immunol 135: 2937–2945PubMedGoogle Scholar
  232. Krown SE (1986) Interferons: not just alpha. Cancer Treat Rep 70: 1353–1355PubMedGoogle Scholar
  233. Krueger GRF (1985) Klinische Immunpathologie. Kohlhammer, Stuttgart, pp 187–288Google Scholar
  234. Kwan SP, Kunkel L, Bruns G, Wedgwood RJ, Latt S, Rosen FS (1986) Mapping of the X-linked agammaglobulinemia locus by use of restriction fragment-length polymorphism. J Clin Invest 77: 649–652PubMedGoogle Scholar
  235. Kwitko AO, Roberts-Thomson PJ, Shearman DJC (1982) Low molecular weight IgM in selective IgA deficiency. Clin Exp Immunol 50: 198–202PubMedGoogle Scholar
  236. Kyewski B (1988) Unravelling the complexity of intrathymic cell-cell interactions. APMIS 96: 1049–1060PubMedGoogle Scholar
  237. Lackie JM (1988) The behavioural repertoire of neutrophils requires multiple signal transduction pathways. J Cell Sci 89: 449–452PubMedGoogle Scholar
  238. Lane P, MacLennan I (1986) Impaired lung function in patients with IgA deficiency and low levels of IgG2 or IgG3. N Engl J Med 314: 924–925Google Scholar
  239. Latron F, Jotterand-Bellomo M, Maffei A, Scarpellino L, Bernard M, Strominger JL, Accolla RS (1988) Active suppression of major histocompatibility complex class II gene expression during differentiation from B cells to plasma cells. Proc Natl Acad Sci USA 85: 2229–2233PubMedGoogle Scholar
  240. Lau YL, Levinsky RJ (1988) Prenatal diagnosis and carrier detection in primary immunodeficiency disorders. Arch Dis Child 63: 758–764PubMedGoogle Scholar
  241. Lau YL, Shields JG, Levinsky RJ, Callard RE (1989) Epstein-Barr-virus-transformed lymphoblastoid cell lines derived from patients with X-linked agammaglobulinaemia and WiskottAldrich syndrome: responses to B cell growth and differentiation factors. Clin Exp Immunol 75: 190–195PubMedGoogle Scholar
  242. Lebovitz RM (1986) Oncogenes as mediators of cell growth and differentiation. Lab Invest 55: 249–251PubMedGoogle Scholar
  243. Ledbetter JA, Tonks NK, Fischer EH, Clark EA (1988) CD45 regulates signal transduction and lymphocyte activation by specific association with receptor molecules on T or B cells. Proc Natl Acad Sci USA 85: 8628–8632PubMedGoogle Scholar
  244. Le Meur M, Gerlinger P, Benoist C, Mathis D (1985) Correcting an immune-response deficiency by creating E a gene transgenic mice. Nature 316: 38–42PubMedGoogle Scholar
  245. Lennert K (1961) Lymphknoten: Diagnostik in Schnitt und Ausstrich. In: Lubarsch O, Henke F, Rössle R, Uehlinger E (eds) Handbuch der speziellen pathologischen Anatomie und Histologie, vol I/3a. Springer, Berlin Göttingen Heidelberg, pp 86–102Google Scholar
  246. Leung DYM, Geha RS (1988) Clinical and immunologic aspects of the hyperimmunoglobulin E syndrome. Hematol Oncol Clin North Am 2: 81–100Google Scholar
  247. Levin S, Hahn T (1985) Interferon deficiency syndrome. Clin Exp Immunol 60: 267–273PubMedGoogle Scholar
  248. Levitt D, Olmstead L (1987) B-cell stimulation by T-cell-secreted proteoglycan. Cell Immunol 110: 425–430PubMedGoogle Scholar
  249. Levy Y, Hershfield MS, Fernandez-Mejia C, Polmar SH, Scudiery D, Berger M, Sorensen RU (1988) Adenosine deaminase deficiency with late onset of recurrent infections: response to treatment with polyethylene glycol-modified adenosine deaminase. J Pediatr 113: 312–317PubMedGoogle Scholar
  250. Lichtman AH, Chin J, Schmidt JA, Abbas AK (1988) Role of interleukin 1 in the activation of T lymphocytes. Proc Natl Acad Sci USA 85: 9699–9703PubMedGoogle Scholar
  251. Lin C Y, Kuo Y-C, Lin C-C, Ou B-R (1988) Enhancement of interleukin-2 and y-interferon production in vitro on cord blood lymphocytes and in vivo on primary cellular immunodeficiency patients with thymic extract (thymostimulin). J Clin Immunol 8: 103–107PubMedGoogle Scholar
  252. Litwin SD (1979) Immunodeficiency with thymoma: failure to induce Ig production in immunodeficient lymphocytes cocultured with normal T cells. J Immunol 122: 728–732PubMedGoogle Scholar
  253. Löffler W (1951) Skoda im Wendepunkt der Medizin. Wien Med Wochenschr 41: 771–777Google Scholar
  254. Login GR, Dvorak AM (1988) Microwave fixation provides excellent preservation of tissue, cells and antigens for light and electron microscopy. Histochem J 20: 373–387PubMedGoogle Scholar
  255. Lowrie DB, Jackett PS, Andrew PW (1985) Activation of macrophages for antimycobacterial activity. Immunol Lett 11: 195–203PubMedGoogle Scholar
  256. Luscieti P, Hubschmid T, Cottier H, Hess MW, Sobin LH (1980) Human lymph node morphology as a function of age and site. J Clin Pathol 33: 454–461PubMedGoogle Scholar
  257. Luzi G, Businco L, Aiuti F (1983) Primary immunodeficiency syndromes in Italy: a report of the National Register in children and adults. J Clin Immunol 3: 316–320PubMedGoogle Scholar
  258. MacDonald HR, Schneider R, Lees RK et al. (1988) T-cell receptor Vß use predicts reactivity and tolerance to Mlsa-encoded antigens. Nature 332: 40–45PubMedGoogle Scholar
  259. MacLennan ICM, Oldfield S, Liu Y-J, Lane PJL (1989) Regulation of B-cell populations. Curr Top Pathol 79: 37–57PubMedGoogle Scholar
  260. Malcolm S, Barton P, Murphy C, Ferguson-Smith MA, Bentley DL, Rabbitts TH (1982) Localization of human immunoglobulin K light chain variable region genes to the short arm of chromosome 2 by in situ hybridization. Proc Natl Acad Sci USA 79: 4957–4961PubMedGoogle Scholar
  261. Male DK (1986) Idiotypes and autoimmunity. Clin Exp Immunol 65: 1–9PubMedGoogle Scholar
  262. Malech HL, Gallin JI (1987) Neutrophils in human diseases. N Engl J Med 317: 687–694PubMedGoogle Scholar
  263. Maleckar JR, Sherman LA (1987) The composition of the T cell receptor repertoire in nude mice. J Immunol 138: 3873–3876PubMedGoogle Scholar
  264. Malkovsky M, Jira M, Gao L, Loveland B, Malkovska V, Dalgleish AG, Webster ADB (1986) Reduced expression of interleukin-2 receptors in hypogammaglobulinaemia: a possible cause of higher cancer incidence. Lancet 1: 1442–1443PubMedGoogle Scholar
  265. Malynn BA, Blackwell TK, Fulop GM et al. (1988) The scid defect affects the final step of the immunoglobulin VDJ recombinase mechanism. Cell 54: 453–460PubMedGoogle Scholar
  266. Mankarious S, Lee M, Fischer S, Pyun KH, Ochs HD, Oxelius VA, Wedgwood RJ (1988) The half-lives of IgG subclasses and specific antibodies in patients with primary immunodeficiency who are receiving intravenously administered immunoglobulin. J Lab Clin Med 112: 634–640PubMedGoogle Scholar
  267. Margolick JB, Ambrus JL Jr, Volkman DJ, Fauci AS (1986) Human T4+ lymphocytes produce a phagocytosis-inducing factor (PIF) distinct from interferon-a and interferon-y. J Immunol 136: 546–554PubMedGoogle Scholar
  268. Markert ML, Hershfield MS, Wiginton DA et al. (1987) Identification of a deletion in the adenosine deaminase gene in a child with severe combined immunodeficiency. J Immunol 138: 3203–3206PubMedGoogle Scholar
  269. Marlin SD, Morton CC, Anderson DC, Springer TA (1986) LFA-1 immunodeficiency disease. Definition of the genetic defect and chromosomal mapping of a and ß subunits of the lymphocyte function-associated antigen 1 (LFA-1) by complementation in hybrid cells. J Exp Med 164: 855–867PubMedGoogle Scholar
  270. Marx JL (1987) Receptors highlighted at NIH symposium. Science 238: 615–616PubMedGoogle Scholar
  271. Mason DY, Krissansen GW, Davey FR, Crumpton MJ, Gatter KC (1988) Antisera against epitopes resistant to denaturation on T3 (CD3) antigen can detect reactive and neoplastic T cells in paraffin embedded tissue biopsy specimens. J Clin Pathol 41: 121–127PubMedGoogle Scholar
  272. Matheson DS, Green BJ (1987) Defect in production of B cell differentiation factor-like activity by mononuclear cells from a boy with hypogammaglobulinemia. J Immunol 138: 2469–2472PubMedGoogle Scholar
  273. Matsuura M, Kikkawa Y, Kitagawa T, Tanaka S (1989) Modulation of immunological abnormalities of growth hormone-deficient children by growth hormone treatment. Acta Paediatr Jpn Overseas Ed 31: 53–57Google Scholar
  274. Mayer L, Kwan SP, Thompson C, Ko HS, Chiorazzi N, Waldmann T, Rosen F (1986) Evidence for a defect in “switch” T cells in patients with immunodeficiency and hyperimmunoglobulinemia M. N Engl J Med 314: 409–413PubMedGoogle Scholar
  275. McGeady SJ (1987) Transient hypogammaglobulinemia of infancy: need to reconsider name and definition. J Pediatr 110: 47–50PubMedGoogle Scholar
  276. McGuire TC, Banks KL, Poppie MJ (1975) Combined immunodeficiency in horses: characterization of the lymphocyte defect. Clin Immunol Immunopathol 3: 555–566PubMedGoogle Scholar
  277. Medical Research Councils of Austria, Denmark, Finland, France, The Netherlands, Norway, Spain, Sweden, Switzerland, United Kingdom, and West Germany (1988) Gene therapy in man. Recommendations of European Medical Research Councils. Lancet 1: 1271–1272Google Scholar
  278. Ménard M, Meyers KM (1988) Storage pool deficiency in cattle with the Chédiak-Higashi syndrome results from an absence of dense granule precursors in their megakaryocytes. Blood 72: 1726–1734PubMedGoogle Scholar
  279. Mensink EJBM, Thompson A, Schot JDL, van de Greef WMM, Sandkuyl LA, Schuurman RKB (1986) Mapping of a gene for X-linked agammaglobulinemia and evidence for genetic heterogeneity. Hum Genet 73: 327–332PubMedGoogle Scholar
  280. Merigan TC (1988) Human interferon as a therapeutic agent. A decade passes. N Engl J Med 318: 1458–1460PubMedGoogle Scholar
  281. Metcalf D (1985) The granulocyte-macrophage colony stimulating factors. Cell 43: 5–6PubMedGoogle Scholar
  282. Metzger H, Kinet J-P (1988) How antibodies work: focus on Fc receptors. FASEB J 2: 3–11PubMedGoogle Scholar
  283. Meuer SC, Dumann H, Meyer zum Büschenfelde K-H, Köhler H (1989) Low-dose interleukin-2 induces systemic immune responses against HBsAg in immunodeficient non-responders to hepatitis B vaccination. Lancet 1: 15–18PubMedGoogle Scholar
  284. Migone N, Oliviero S, De Lange G et al. (1984) Multiple gene deletions within the human immunoglobulin heavy-chain cluster. Proc Natl Acad Sci USA 81: 5811–5815PubMedGoogle Scholar
  285. Miller JFAP (1961) Immunological function of the thymus. Lancet 11: 748–749Google Scholar
  286. Milon G, Lebastard M, Marchal G (1985) T-dependent production and activation of mononuclear phagocytes during murine BCG infection. Immunol Lett 11: 189–194PubMedGoogle Scholar
  287. Mims CA (1987) The pathogenesis of infectious disease, 3rd edn. Academic, LondonGoogle Scholar
  288. Mizushima Y, Saitoh M, Ogata M et al. (1989) Thymic stroma-derived T cell growth factor (TSTGF). IV. Capacity of TSTGF to promote the growth of L3T4 Lyt-2 thymocytes by synergy with phorbol myristate acetate or various IL. J Immunol 142: 1195–1202PubMedGoogle Scholar
  289. Mollines TE, Lachmann PJ (1988) Regulation of complement. Scand J Immunol 27: 127–142Google Scholar
  290. Moreau J-F, Donaldson DD, Bennett F, Witek-Giannotti JA, Clark SC, Wong GG (1988) Leukaemia inhibitory factor is identical to the myeloid growth factor human interleukin for DA cells. Nature 336: 690–692PubMedGoogle Scholar
  291. Morell A, Keller H (1988) Immundefekt and andere parathymische Syndrome bei Thymom. Ergeb Inn Med Kinderheilkd 57: 185–216PubMedGoogle Scholar
  292. Morell A, Nydegger UE (eds) (1986) Clinical use of intravenous immunoglobulins. Academic, London.Google Scholar
  293. Morgan EL, Weigle WO, Hugh TE (1984) Anaphylatoxin-mediated regulation of human and murine immune responses. Fed Proc 43: 2543–2547PubMedGoogle Scholar
  294. Morris AG (1988) Interferons. Immunology Suppl 1: 43–45Google Scholar
  295. Mossalayi MD, Lecron JC, Goube de Laforest P, Janossy G, Debré P, Tanzer J (1988) Characterization of prothymocytes with cloning capacity in human bone marrow. Blood 71: 1281–1287PubMedGoogle Scholar
  296. Movat HZ, Burrowes CE, Cybulsky MI, Dinarello CA (1987) Acute inflammation and a Shwartzman-like reaction induced by interleukin-1 and tumor necrosis factor. Synergistic action of the cytokines in the induction of inflammation and microvascular injury. Am J Pathol 129: 463–476PubMedGoogle Scholar
  297. Mueller-Eberhard HJ (1985) The killer molecule of complement. J Invest Dermatol 85 [1 Suppl]: 47s - 52sGoogle Scholar
  298. Nakagawa T, Nakagawa N, Ambrus JL Jr, Fauci AS (1988) Differential effects of interleukin 2 vs B cell growth factor on human B cells. J Immunol 140: 465–469PubMedGoogle Scholar
  299. Neugebauer E, Lorenz W, Beckurts T, Maroske D, Merte H (1987) Significance of histamine formation and release in the development of endotoxic shock: proof of current concepts by randomized controlled studies in rats. Rev Infect Dis 9 [Suppl 5]: 5585–S593Google Scholar
  300. Nézelof C, Jammet ML, Lortholary P, Labrune B, Lamy M (1964) L’hypoplasie héréditaire du thymus. Sa place et sa responsabilité dans une observation d’aplasie lymphocytaire normoplasmocytaire et normoglobulinémique du nourrison. Arch Fr Pediatr 21: 897–920PubMedGoogle Scholar
  301. Nossal GJV (1987) The basic components of the immune system. N Engl J Med 316: 1320–1325PubMedGoogle Scholar
  302. Nunoi H, Yanabe Y, Higuchi S et al. (1988) Severe hypoplasia of lymphoid tissues in Mol deficiency. Hum Pathol 19: 753–759PubMedGoogle Scholar
  303. Nusinow SR, Zuraw BL, Curd JG (1985) The hereditary and acquired deficiencies of complement. Med Clin North Am 69: 487–504PubMedGoogle Scholar
  304. Ochs HD, Wedgwood RJ, Frank MM, Heller SR, Hosea SW (1983) The role of complement in the induction of antibody responses. Clin Exp Immunol 53: 208–216PubMedGoogle Scholar
  305. Ohno T, Fujii H, Kanoh T, Uchino H, Kuribayashi K, Masuda T, Watanabe Y (1987a) Selective deficiency in IL-2 production and refractoriness to extrinsic IL-2 in immunodeficiency with hyper-IgM. Clin Immunol Immunopathol 45: 471–480Google Scholar
  306. Ohno T, Inaba M, Kuribayashi K, Masuda T, Kanoh T (1987b) Selective IgM deficiency in adults: phenotypically and functionally altered profiles of peripheral blood lymphocytes. Clin Exp Immunol 68: 630–637PubMedGoogle Scholar
  307. Ottenhoff THM, Torres P, De las Aguas JT, Fernandez R, Van Eden W, De Vries RRP, Stanford JL (1986) Evidence for an HLA-DR4-associated immune-response gene for Mycobacterium tuberculosis. Lancet 1: 310–312Google Scholar
  308. Out TA, Kallenberg CGM, Van Munster PJJ (1988) De betekenis van de bepaling van humorale factoren van de afweer: Ig-isotypen, specifieke antistoffen en complement. Tijdschr Kindergeneeskd 56: 192–196PubMedGoogle Scholar
  309. Ownby DR, Pizzo S, Blackmon L, Gall SA, Buckley RH (1976) Severe combined immunodeficiency with leukopenia (reticular dysgenesis) in siblings: immunologic and histopathologic findings. J Pediatr 89: 382–387PubMedGoogle Scholar
  310. Pachman LM, Lynch PA, Silver RK, Ozog DL, Poznanski AK (1989) Primary immunodeficiency disease in children: an update. Curr Probl Pediatr 19: 1–64PubMedGoogle Scholar
  311. Pallant A, Eskenazi A, Mattei M-G, Fournier REK, Carlsson SR, Fukuda M, Frelinger JG (1989) Characterization of cDNAs encoding human leukosialin and localization of the leukosialin gene to chromosome 16. Proc Natl Acad Sci USA 86: 1328–1332PubMedGoogle Scholar
  312. Papadea C, Check IJ (1989) Human immunoglobulin G and immunoglobulin G subclasses: biochemical, genetic, and clinical aspects. CRC Crit Rev Clin Lab Sci 27: 27–56Google Scholar
  313. Patarroyo M, Prieto J, Beatty PG, Clark EA, Gahmberg CG (1988) Adhesion-mediating molecules of human monocytes. Cell Immunol 113: 278–289PubMedGoogle Scholar
  314. Paul WE (1987) Interleukin 4/B cell stimulatory factor 1: one lymphokine, many functions. FASEB J 1: 456–461PubMedGoogle Scholar
  315. Perlmutter DH, Colten HR (1989) Molecular basis of complement deficiencies. Immunodeficiency Rev 1: 105–133Google Scholar
  316. Perry KJ, Filteau SM, Woodward B (1988) Dissociation of immune capacity from nutritional sta- tus by triiodothyronine supplements in severe protein deficiency. FASEB J 2: 2609–2612PubMedGoogle Scholar
  317. Perryman LE, McGuire TC, Banks KL (1983) Animal model of human disease. Infantile X-linked agammaglobulinemia. Agammaglobulinemia in horses. Am J Pathol 111: 125–127PubMedGoogle Scholar
  318. Plebani A, Ugazio AG, Monafo V, Burgio GR (1986) Clinical heterogeneity and reversibility of selective immunoglobulin A deficiency in 80 children. Lancet 1: 829–831PubMedGoogle Scholar
  319. Pollard K, Lunny D, Holgate CS, Jackson P, Bird CC (1987) Fixation, processing, and immunochemical reagent effects on preservation of T-lymphocyte surface membrane antigens in paraffin-embedded tissue. J Histochem Cytochem 35: 1329–1338PubMedGoogle Scholar
  320. Popa V (1988) Lymphocytic interstitial pneumonia of common variable immunodeficiency. Ann Allergy 60: 203–206PubMedGoogle Scholar
  321. Porteu F, Fischer A, Descamps-Latscha B, Halbwachs-Mecarelli L (1986) Defective complement receptors (CR1 and CR3) on erythrocytes and leukocytes of factor I (C3b-inactivator) deficient patients. Clin Exp Immunol 66: 463–471PubMedGoogle Scholar
  322. Puck JM, Nussbaum RL, Smead DL, Conley ME (1989) X-linked severe combined immunodeficiency: localization within the region Xg13.1–821.1 by linkage and deletion analysis. Am J Hum Genet 44: 724–730PubMedGoogle Scholar
  323. Purtilo DT (1985) Epstein-Barr virus-induced diseases in the X-linked lymphoproliferative syndrome and related disorders. Biomed Pharmacother 39: 52–58PubMedGoogle Scholar
  324. Purtilo DT (1987) Opportunistic cancers in patients with immunodeficiency syndromes. Arch Pathol Lab Med 111: 1123–1129PubMedGoogle Scholar
  325. Purtilo DT, Okano M, Grierson H et al. (1989) Molecular pathology of X-linked lymphoproliferative syndrome (XLP). Lab Invest 60 (1): 74AGoogle Scholar
  326. Pyke KW, Dosch H-M, Ipp MM, Gelfand EW (1975) Demonstration of an intrathymic defect in a case of severe combined immunodeficiency disease. N Engl J Med 293: 424–428PubMedGoogle Scholar
  327. Radford DJ, Thong YH (1989) Facial and immunological anomalies associated with tetralogy of Fallot. Int J Cardiol 22: 229–236PubMedGoogle Scholar
  328. Radford DJ, Perkins L, Lachman R, Thong YH (1988) Spectrum of DiGeorge syndrome in pa- tients with truncus arteriosus: expanded DiGeorge syndrome. Pediatr Cardiol 9: 95–101PubMedGoogle Scholar
  329. Ratech H, Greco MA, Gallo G, Rimoin DL, Kamino H, Hirschhorn R (1985) Pathologic findings in adenosine-deaminase-deficient severe combined immunodeficiency. I. Kidney, adrenal, and chondro-osseous tissue alterations. Am J Pathol 120: 157–169PubMedGoogle Scholar
  330. Raulf M, König W (1988) Modulation of leukotriene release from human polymorphonuclear leucocytes by PMA and arachidonic acid. Immunology 64: 51–59PubMedGoogle Scholar
  331. Ravetch JV, Luster AD, Weinshank R et al. (1986) Structural heterogeneity and functional domains of murine immunoglobulin G Fc receptors. Science 234: 718–725PubMedGoogle Scholar
  332. Reed SG, Andrade ZA, Roters SB, Inverso JA, Sadigursky M (1986) Leishmania mexicana amazonensis infections in “resistant” inbred mice following removal of the draining lymph node. Clin Exp Immunol 64: 8–13Google Scholar
  333. Reid KBM, Day AJ (1989) Structure-function relationships of the complement components. Immunol Today 10: 177–180PubMedGoogle Scholar
  334. Reynolds HY (1988) Immunoglobulin G and its function in the human respiratory tract. Mayo Clin Proc 63: 161–174PubMedGoogle Scholar
  335. Rich KC, Arnold WJ, Palella T, Fox IH (1979) Cellular immune deficiency with autoimmune hemolytic anemia in purine nucleoside phosphorylase deficiency. Am J Med 67: 172–176PubMedGoogle Scholar
  336. Richie ER, McEntire B, Crispe N, Kimura J, Lanier LL, Allison JP (1988) α/ß T-cell antigen receptor gene and protein expression occurs at early stages of thymocyte differentiation. Proc Natl Acad Sci USA 85: 1174–1178Google Scholar
  337. Roberton DM, Björkander J, Henrichsen J, Söderström T, Hanson LA (1989) Enhanced IgG1 and IgG3 responses to pneumococcal polysaccharides in isolated IgA deficiency. Clin Exp Immunol 75: 201–205PubMedGoogle Scholar
  338. Roder JC, Todd RF, Rubin P et al. (1983) The Chediak-Higashi gene in humans. III. Studies on the mechanisms of NK impairment. Clin Exp Immunol 51: 359–368PubMedGoogle Scholar
  339. Rosen FS (1978) The immunodeficiency syndromes. In: Samter M (ed) Immunological diseases, 3rd edn, vol 1. Little, Brown and Company, Boston, pp 472–498Google Scholar
  340. Rosen FS, Alper CA (1985) Genetic deficiencies of the complement system. Clin Immunol Allergy 5: 371–377Google Scholar
  341. Rosen FS, Cooper MD, Wedgwood RJP (1984a) The primary immunodeficiencies (first of two parts). N Engl J Med 311: 235–242PubMedGoogle Scholar
  342. Rosen FS, Cooper MD, Wedgwood RJP (1984b) The primary immunodeficiencies (second of two parts). N Engl J Med 311: 300–310PubMedGoogle Scholar
  343. Ross SC, Densen P (1984) Complement deficiency states and infection: epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency. Medicine (Baltimore) 63: 243–273Google Scholar
  344. Royer HD, Reinherz EL (1987) T lymphocytes: ontogeny, function, and relevance to clinical disorders. N Engl J Med 317: 1136–1142PubMedGoogle Scholar
  345. Ryan JJ, Mond JJ, Finkelman FD (1988) Genetic analysis of the presentation of minor lymphocyte stimulating determinants. I. Combined importance of MHC and non-MHC influences. J Immunol 141: 1063–1073PubMedGoogle Scholar
  346. Ryser O, Morell A, Hitzig WH (1988) Primary immunodeficiencies in Switzerland: first report of the National Registry in adults and children. J Clin Immunol 8: 479–485PubMedGoogle Scholar
  347. Sachs L (1987) The molecular control of blood cell development. Science 238: 1374–1379PubMedGoogle Scholar
  348. Sainte-Marie G, Peng F-S (1985) Lymph nodes of the N: NIH(S)II-nu/nu mouse. Lab Invest 52: 631–637PubMedGoogle Scholar
  349. Sainte-Marie G, Peng F-S (1987) The formation of “compartment replicas” in the lymph nodes of athymic animals. Cell Tissue Res 248: 323–333PubMedGoogle Scholar
  350. Sainte-Marie G, Peng F-S, Pelletier M (1986) Morphological anomalies in the lymph nodes of 13-month-old thymus-grafted nude mice. Thymus 8:77–90Google Scholar
  351. Sakiyama Y, Takahashi Y, Matsumoto S (1987) IL2 production and IL2 receptor expression in primary immunodeficiency diseases. Acta Paediatr Jpn Overseas Ed 29: 678–679Google Scholar
  352. Sauter H, Paige CJ (1987) Detection of normal B-cell precursors that give rise to colonies produc- ing both x and.l light immunoglobulin chains. Proc Natl Acad Sci USA 84: 4989–4993PubMedGoogle Scholar
  353. Savino W, Dardenne M (1984) Thymic hormone-containing cells. VI. Immunohistologic evidence for the simultaneous presence of thymulin, thymopoietin and thymosin al in normal and pathological human thymuses. Eur J Immunol 14: 987–991PubMedGoogle Scholar
  354. Schaffner T, Mueller J, Hess MW, Cottier H, Sordat B, Ropke C (1974) The bursa of Fabricius: a central organ providing for contact between the lymphoid system and intestinal content. Cell Immunol 13: 304–312PubMedGoogle Scholar
  355. Scher I, Titus JA, Finkelman FD (1983) The ontogeny and distribution of B cells in normal and mutant immune-defective CBA/N mice: two-parameter analysis of surface IgM and IgD. J Immunol 130: 619–625PubMedGoogle Scholar
  356. Schinzel A (1988) Microdeletion syndromes, balanced translocations, and gene mapping. J Med Genet 25: 454–462PubMedGoogle Scholar
  357. Schmalstieg FC (1988) Leukocyte adherence defect. Pediatr Infect Dis J 7: 867–872PubMedGoogle Scholar
  358. Schmitt M, Keller HU, Cottier H (1986) Qualitative and quantitative assessment of human polymorphonuclear leukocyte ( PMNL) functions. Beitr Infusionsther Klin Ernahr 15: 196–230Google Scholar
  359. Schooley JC, Kelly LS (1961) The thymus in lymphocyte production. Fed Proc 20: 71Google Scholar
  360. Schreiber JR, Barrus V, Cates KL, Siber GR (1986) Functional characterization of human IgG, IgM, and IgA antibody directed to the capsule of Haemophilus influenzae type b. J Infect Dis 153: 8–16PubMedGoogle Scholar
  361. Schulte-Wissermann H, Lemmel E-M, Opferkuch W, Grosse-Wilde H, Mempel W, Kössling FK, Cottier H (1973) Immunmangelsyndrom mit Dysgammaglobulinämie und lymphatischer Hyperplasie. Helv Paediatr Acta 28: 569–581PubMedGoogle Scholar
  362. Schuurman H-J, Vaessen LMB, Broekhuizen R, Brandt CJWM, Holewijn MC, Vos JG, Rozing J (1987) Implantation of cultured thymic fragments in congenitally athymic (nude) rats. Influx of lymphocytes and dendritic cells. Scand J Immunol 26: 129–139PubMedGoogle Scholar
  363. Schwaber J, Koenig N, Girard J (1988a) Correction of the molecular defect in B lymphocytes from X-linked agammaglobulinemia by cell fusion. J Clin Invest 82: 1471–1476PubMedGoogle Scholar
  364. Schwaber J, Payne J, Chen R (1988b) B lymphocytes from X-linked agammaglobulinemia. Delayed expression of light chain and demonstration of lyonization in carriers. J Clin Invest 81: 514–522PubMedGoogle Scholar
  365. Schwaber JF, Lazarus H, Rosen FS (1981) IgM-restricted production of immunoglobulin by lymphoid cell lines from patients with immunodeficiency with hyper IgM (dysgammaglobulinemia). Clin Immunol Immunopathol 19: 91–97PubMedGoogle Scholar
  366. Schwartz RS, Rose NR (eds) (1986) Autoimmunity: experimental and clinical aspects. Ann NY Acad Sci 475: 1–427Google Scholar
  367. Seemayer TA (1987) Molecular basis of selected primary immunodeficiency disorders. Arch Pathol Lab Med 111: 1114–1117PubMedGoogle Scholar
  368. Segal A, Walport M (1988) Neutrophil and complement defects: recent advances. In: Webster ADB (ed) Immunodeficiency and disease. Kluwer Academic, Dordrecht, pp 144–179Google Scholar
  369. Segal AW (1987) Absence of both cytochrome b _245 subunits from neutrophils in X-linked chronic granulomatous disease. Nature 326: 88–91PubMedGoogle Scholar
  370. Shah T, Webster ADB, Peters TJ (1983) Lymphocyte enzyme activities in immunodeficiency syndromes with particular reference to common variable hypogammaglobulinaemia. Clin Exp Immunol 53: 413–422PubMedGoogle Scholar
  371. Shanon A, Levin S, Holtzman F, Brock-Sinai F, Abu-Said A (1988) Combined familial adenosine deaminase and purine nucleoside phosphorylase deficiencies. Arch Dis Child 63: 931–934PubMedGoogle Scholar
  372. Sharp JG, Crouse DA, Purtilo DT (1987) Ontogeny and regulation of the immune system. Arch Pathol Lab Med 111: 1106–1113PubMedGoogle Scholar
  373. Sherr E, Adelman DC, Saxon A, Gilly M, Wall R, Sidell N (1988) Retinoic acid induces the differentiation of B cell hybridomas from patients with common variable immunodeficiency. J Exp Med 168: 55–71PubMedGoogle Scholar
  374. Sideras P, Funa K, Zalcberg-Quintana I, Xanthopoulos KG, Kisielow P, Palacios R (1988) Analysis by in situ hybridization of cells expressing mRNA for interleukin 4 in the developing thymus and in peripheral lymphocytes from mice. Proc Natl Acad Sci USA 85: 218–221PubMedGoogle Scholar
  375. Siegel JP (1988) Effects of interferon-y on the activation of human T lymphocytes. Cell Immunol 111: 461–472PubMedGoogle Scholar
  376. Siegel RL, Issekutz T, Schwaber J, Rosen FS, Geha RS (1981) Deficiency of T helper cells in transient hypogammaglobulinemia of infancy. N Engl J Med 305: 1307–1313PubMedGoogle Scholar
  377. Sing GK, Keller JR, Ellingsworth LR, Ruscetti FW (1988) Transforming growth factor ß selectively inhibits normal and leukemic human bone marrow cell growth in vitro. Blood 72: 1504–1511PubMedGoogle Scholar
  378. Sjöholm AG, Hammarström L, Smith CIE, Kjellman N-IM (1985) Immunoglobulin synthesis in a patient with C4 deficiency: functional immaturity of peripheral blood lymphocytes. Acta Pathol Microbiol Scand [C] 93: 169–173Google Scholar
  379. Smith CIE (1988) Molecular basis of the immune response. Scand J Immunol 27:485–487Google Scholar
  380. Smith CIE, Hammarström L (1986) Gene abnormalities in human immunoglobulin deficiency disorders. Clin Immunol Newsletter 7: 145–149Google Scholar
  381. Smith CIE, Hammarström L (1987) Cellular basis of immunodeficiency. Ann Clin Res 19: 220–229PubMedGoogle Scholar
  382. Smith CIE, Brémard-Oury C, Le Deist F et al. (1988) The antibody spectrum in individuals with defect expression of HLA class II and the LFA-1 glycoprotein family genes. Clin Exp Immunol 74: 449–453PubMedGoogle Scholar
  383. Smith KA (1988) Interleukin-2: inception, impact, and implications. Science 240: 1169–1176PubMedGoogle Scholar
  384. Smith PJ, Makinson TA (1989) Cellular consequences of overproduction of DNA topoisomerase II in an ataxia-telangiectasia cell line. Cancer Res 49: 1118–1124PubMedGoogle Scholar
  385. Snyderman R, Verghese MW (1987) Leukocyte activation by chemoattractant receptors: roles of a guanine nucleotide regulatory protein and polyphosphoinositide metabolism. Rev Infect Dis 9 [Suppl 5]: S562–5569PubMedGoogle Scholar
  386. Spangrude GJ, Heimfeld S, Weissman IL (1988) Purification and characterization of mouse hematopoietic stem cells. Science 241: 58–62PubMedGoogle Scholar
  387. Späth PJ (1987) Inhibitors of complement activation and complement breakdown products. Plasma Ther Transfus Technol 8: 324–332Google Scholar
  388. Spector BD, Perry GS III, Kersey JH (1978) Genetically determined immunodeficiency diseases (GDID) and malignancy: Report from the immunodeficiency-cancer registry. Clin Immunol Immunopathol 11: 12–29PubMedGoogle Scholar
  389. Sporn MB, Roberts AB (1988) Peptide growth factors are multifunctional. Nature 332: 217–219PubMedGoogle Scholar
  390. Stavnezer-Nordgren J, Kekish O, Zegers BJM (1985) Molecular defects in a human immunoglobulin rc chain deficiency. Science 230: 458–461PubMedGoogle Scholar
  391. Steffen M, Ottmann OG, Moore MAS (1988) Simultaneous production of tumor necrosis factor-a and lymphotoxin by normal T cells after induction with IL-2 and anti-T3. J Immunol 140: 2621–2624PubMedGoogle Scholar
  392. Stein H, Dallenbach F, Dienemann D (1988) Differenzierungslinien physiologischer and maligner Zellen des lymphatischen Systems. Verh Dtsch Ges Pathol 72:57–85PubMedGoogle Scholar
  393. Stoolman LM (1989) Adhesion molecules controlling lymphocyte migration. Cell 56:907–910PubMedGoogle Scholar
  394. Strasser-Vogel B, Belohradsky BH (1988) Asplenismus and Hyposplenismus als Immundefektsyndrom. Monatsschr Kinderheilkd 136: 795–807PubMedGoogle Scholar
  395. Strauss PR, Henderson JF, Goodman MG (1985) Nucleosides and lymphocytes — an overview. Proc Soc Exp Biol Med 179: 413–418PubMedGoogle Scholar
  396. Sullivan JL, Byron KS, Brewster FE, Baker SM, Ochs HD (1983) X-linked lymphoproliferative syndrome. Natural history of the immunodeficiency. J Clin Invest 71: 1765–1778PubMedGoogle Scholar
  397. Suthanthiran M (1988) T-cell differentiation antigen cluster 2 (CD2) is a receptor for accessory cells and can generate and/or transduce accessory signals. Cell Immunol 112: 112–122PubMedGoogle Scholar
  398. Suzuki Y, Kobayashi A (1985) Macrophage-mediated suppression of immune responses in Toxoplasma-infected mice. II. Both H-2-linked and -nonlinked control of induction of suppressor macrophages. Cell Immunol 91: 375–384PubMedGoogle Scholar
  399. Syrjänen K, Gissmann L, Koss LG (eds) (1987) Papillomaviruses and human disease. Springer, Berlin Heidelberg New YorkGoogle Scholar
  400. Szakal AK, Kosco MH, Tew JG (1988) A novel in vivo follicular dendritic cell-dependent iccosome-mediated mechanism for delivery of antigen to antigen-processing cells. J Immunol 140: 341–353PubMedGoogle Scholar
  401. Tacchini-Cottier FM, Jones PP (1988) Defective EQ expression in three mouse H-2 haplotypes results from aberrant RNA splicing. J Immunol 141: 3647–3653PubMedGoogle Scholar
  402. Takatsu K (1988) B-cell growth and differentiation factors (42732A). Proc Soc Exp Biol Med 188: 243–258PubMedGoogle Scholar
  403. Talmadge JE, Herberman RB (1986) The preclinical screening laboratory: evaluation of immunomodulatory and therapeutic properties of biological response modifiers. Cancer Treat Rep 70: 171–182PubMedGoogle Scholar
  404. Taubman MA, Ebersole JL, Smith DJ (1986) Immunoglobulin levels of congenitally athymic rats immunized with thymus-dependent-independent antigens. Immunology 58: 145–150PubMedGoogle Scholar
  405. Tesch H, Müller W, Rajewsky K (1986) Lymphokines regulate immunoglobulin isotype expression in an antigen-specific immune response. J Immunol 136: 2892–2895PubMedGoogle Scholar
  406. Tew JG, Mandel TE, Phipps RP, Szakal AK (1984) Tissue localization and retention of antigen in relation to the immune response. Am J Anat 170: 407–420PubMedGoogle Scholar
  407. Thompson CB, Lindsten T, Ledbetter JA et al. (1989) CD28 activation pathway regulates the production of multiple T-cell-derived lymphokines/cytokines. Proc Natl Acad Sci USA 86: 1333–1337PubMedGoogle Scholar
  408. Thompson LF, Seegmiller JE (1980) Adenosine deaminase deficiency and severe combined immunodeficiency disease. Adv Enzymol 51: 167–210PubMedGoogle Scholar
  409. Thompson RA (guest ed) (1985) Clinics in immunology and allergy: laboratory investigation of immunological disorders, vol 5/No. 3. WB Saunders, LondonGoogle Scholar
  410. Thompson RA (1988) Immunodeficiency due to defects of polymorphonuclear leukocyte function Immunol Invest 17: 85–92PubMedGoogle Scholar
  411. Tiller TL Jr, Buckley RH (1978) Transient hypogammaglobulinemia of infancy: review of the literature, clinical and immunologic features of 11 new cases, and long-term follow-up. J Pediatr 92: 347–353PubMedGoogle Scholar
  412. Timens W, Boes A, Rozeboom-Uiterwijk T, Poppema S (1988) Immuno-architecture of human fetal lymphoid tissues. Virchows Arch [A] 413: 563–571Google Scholar
  413. Tite JP, Kaye J, Saizawa KM, Ming J, Katz ME, Smith LA, Janeway CA Jr (1986) Direct interactions between B and T lymphocytes bearing complementary receptors. J Exp Med 163: 189–202PubMedGoogle Scholar
  414. Tonegawa S (1983) Somatic generation of antibody diversity. Nature 302: 575–581PubMedGoogle Scholar
  415. Toossi Z, Kleinhenz ME, Ellner JJ (1986) Defective interleukin 2 production and responsiveness in human pulmonary tuberculosis. J Exp Med 163: 1162–1172PubMedGoogle Scholar
  416. Touraine JL, Bétuel H, Souillet G, Jeune M (1978) Combined immunodeficiency disease associated with absence of cell-surface HLA-A and -B antigens. J Pediatr 93: 47–51PubMedGoogle Scholar
  417. Touraine J-L, Marseglia G-L, Betuel H (1985) Thirty international cases of bare lymphocyte syndrome: biological significance of HLA antigens. Exp Hematol [Suppl 17] 13: 86–87Google Scholar
  418. Turck CW, Dohlman JG, Goetzl EJ (1987) Immunological mediators of wound healing and fibrosis. J Cell Physiol [Suppl] 5: 89–93Google Scholar
  419. Tykocinski M, Schinella RA, Greco MA (1983) Fibroblastic reticulum cells in human lymph nodes. An ultrastructural study. Arch Pathol Lab Med 107: 418–422PubMedGoogle Scholar
  420. Tzoneva M, Ganev V, Galabov A, Georgieva K (1988) Selective immunodeficiency with defect in interferon-gamma induction in two sibs with recurrent infections. Clin Genet 33: 454–456PubMedGoogle Scholar
  421. Umetsu DT, Ambrosino DM, Quinti I, Siber GR, Geha RS (1985) Recurrent sinopulmonary infection and impaired antibody response to bacterial capsular polysaccharide antigen in children with selective IgG-subclass deficiency. N Engl J Med 313: 1247–1251PubMedGoogle Scholar
  422. Umetsu DT, Katzen D, Jabara HH, Geha RS (1986) Antigen presentation by human dermal fibroblasts: activation of resting T lymphocytes. J Immunol 136: 440–445PubMedGoogle Scholar
  423. Unanue ER, Allen PM (1986) Biochemistry and biology of antigen presentation by macrophages. Cell Immunol 99: 3–6PubMedGoogle Scholar
  424. Unanue ER, Allen PM (1987) The basis for the immunoregulatory role of macrophages and other accessory cells. Science 236: 551–557PubMedGoogle Scholar
  425. Van den Brande P, Geboes K, Vantrappen G, Van den Eeckhout A, Vertessen S, Stevens EAM, Ceuppens JL (1988) Intestinal nodular lymphoid hyperplasia in patients with common variable immunodeficiency: local accumulation of B and CD8(+) lymphocytes. J Clin Immunol 8: 296–306PubMedGoogle Scholar
  426. Van der Valk P, Herman CJ (1987) Leukocyte functions. Lab Invest 56: 127–137PubMedGoogle Scholar
  427. Van der Valk P, Meijer CJLM (1987) The histology of reactive lymph nodes. Am J Surg Pathol 11: 866–882PubMedGoogle Scholar
  428. Van Epps DE, El-Naggar A, Ochs HD (1983) Abnormalities of lymphocyte locomotion in immunodeficiency disease. Clin Exp Immunol 53: 678–688Google Scholar
  429. Van Furth R (1989) Origin and turnover of monocytes and macrophages. Curr Top Pathol 79: 125–150PubMedGoogle Scholar
  430. Vetvicka V, Lee G, Kincade PW (1986) Intrinsic B lymphocyte and macrophage defects in C3H/HeJ mice. J Immunol 136: 2370–2374PubMedGoogle Scholar
  431. Vossen JM, Zegers BJM (1988) Diagnostisch onderzoek bij patiënten met een verdenking op een immunologische afweerstoornis. Tijdschr Kindergeneeskd 56: 174–184PubMedGoogle Scholar
  432. Vu TH, Tacchini-Cottier FM, Day CE, Begovich AB, Jones PP (1988) Molecular basis for the defective expression of the mouse E717 gene. J Immunol 141: 3654–3661PubMedGoogle Scholar
  433. Waldmann TA (1988) Immunodeficiency diseases: primary and acquired. In: Samter M (ed) Immunological diseases, 4th edn, vol 1. Little, Brown and Company, Boston, pp 411–465Google Scholar
  434. Wang JC (1987) Recent studies of DNA topoisomerases. Biochim Biophys Acta 909: 1–9PubMedGoogle Scholar
  435. Warren HS, Chedid LA (1987) Strategies for the treatment of endotoxemia: significance of the acute-phase response. Rev Infect Dis 9 [Suppl 5]: S630–5638PubMedGoogle Scholar
  436. Watanabe S, Iwata M, Maeda H, Ishibashi Y (1987) Immunohistochemical studies of major histocompatibility antigens in a case of the bare lymphocyte syndrome without immunodeficiency. J Am Acad Dermatol 17: 895–902PubMedGoogle Scholar
  437. Weaver D, Baltimore D (1987) B lymphocyte-specific protein binding near an immunoglobulin rcchain gene J segment. Proc Natl Acad Sci USA 84: 1516–1520PubMedGoogle Scholar
  438. Webster ADB (ed) (1988) Immunodeficiency and disease. Kluwer Academic, DordrechtGoogle Scholar
  439. Webster ADB, Spickett GP (1988) Hypogammaglobulinemia: recent advances. In: Webster ADB (ed) Immunodeficiency disease. Kluwer Academic, Dordrecht, pp 25–42Google Scholar
  440. Weemaes CMR, The TH, Van Munster PJJ, Bakkeren JAJM (1984) Antibody responses in vivo in chromosome instability syndromes with immunodeficiency. Clin Exp Immunol 57: 529–534PubMedGoogle Scholar
  441. Weinberg GA, Granoff DM, Nahm MH, Shackelford PG (1986) Functional activity of different IgG subclass antibodies against type b capsular polysaccharide of Haemophilus influenzae. J Immunol 136: 4232–4236PubMedGoogle Scholar
  442. Weisdorf SA, Krivit W (1982) Paucity of splenic germinal centers: a new and unique splenomegaly syndrome including dysfunctional immune system. Clin Immunol Immunopathol 23: 492–500PubMedGoogle Scholar
  443. Weissman IL, Peacock M, Eltringham JR (1973) Regional lymph node irradiation: effect on local and distant generation of antibody forming cells. J Immunol 110: 1300–1306PubMedGoogle Scholar
  444. West CD (1989) The complement profile in clinical medicine. Inherited and acquired conditions lowering the serum concentrations of complement component and control proteins. Complement Inflamm 6: 49–64PubMedGoogle Scholar
  445. White AG, Raju KT, Abouna GM (1988) A six year experience with recurrent infection and immunodeficiency in children in Kuwait. J Clin Lab Immunol 26: 97–101PubMedGoogle Scholar
  446. White CJ, Gallin JI (1986) Phagocyte defects. Clin Immunol Immunopathol 40: 50–61PubMedGoogle Scholar
  447. Williams LW, Burks AW, Steele RW (1988) Complement: function and clinical relevance. Ann Allergy 60: 293–300PubMedGoogle Scholar
  448. Wintzer H-O, Amberg R, von Kleist S, Forster B (1988) Immunohistological demonstration of lymphocyte surface antigens in postmortem lymphoid tissues. Virchows Arch [A] 413: 581–589Google Scholar
  449. Wise R (1987) Antimicrobial agents: a widening choice. Lancet 11: 1251–1254Google Scholar
  450. Wood MJ, Geddes AM (1987) Antiviral therapy. Lancet II: 1189–1192Google Scholar
  451. Wood PR, Spanidis V, Frangos K, Cheers C (1986) The in vitro bactericidal activity of peritoneal and spleen cells from Listeria-resistant and -susceptible mouse strains. Cell Immunol 99: 160–169PubMedGoogle Scholar
  452. World Health Organization (1983) Primary immunodeficiency diseases. Report prepared for the WHO by a scientific group on immunodeficiency. Clin Immunol Immunopathol 28: 450–475Google Scholar
  453. World Health Organization (1986) Primary immunodeficiency diseases. Report of a World Health Organization scientific group. Clin Immunol Immunopathol 40: 166–196Google Scholar
  454. World Health Organization (1989) Primary immunodeficiency diseases. Report of a WHO sponsored meeting. Immunodeficiency Rev 1: 173–205Google Scholar
  455. Yamamoto H, Tsutsui T, Mayumi M, Kasakura S (1989) Immunodeficiency associated with selective loss of helper/inducer T cells and hypogammaglobulinaemia in a child with intestinal lymphangiectasia. Clin Exp Immunol 75: 196–200PubMedGoogle Scholar
  456. Yancopoulos GD, Alt FW (1988) Reconstruction of an immune system. Science 241: 1581–1583PubMedGoogle Scholar
  457. Yount WJ, Hong R, Seligmann M, Good R, Kunkel HG (1970) Imbalances of gamma globulin subgroups and gene defects in patients with primary hypogammaglobulinemia. J Clin Invest 49: 1957–1966PubMedGoogle Scholar
  458. Yunis EJ (1988) MHC haplotypes in biology and medicine. Am J Clin Pathol 89: 268–280PubMedGoogle Scholar
  459. Zegers BJM, Stoop JW (1988) Metabolic causes of immune deficiency: mechanisms and treat- ment. In: Webster ADB (ed) Immunodeficiency and disease. Kluwer Academic, Dordrecht, pp 113–131Google Scholar
  460. Zegers BJM, Maertzdorf WJ, Van Loghem E et al. (1976) Kappa-chain deficiency. An immunoglobulin disorder. N Engl J Med 294: 1026–1030PubMedGoogle Scholar
  461. Zubler RH (1989) Les principes fondamentaux de la réponse immune de type humoral. Schweiz Med Wochenschr 119: 3–8PubMedGoogle Scholar

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© Springer-Verlag Berlin Heidelberg 1991

Authors and Affiliations

  • H. Cottier
  • R. Kraft
  • F. Meister

There are no affiliations available

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