Abstract
Study of the primary immunodeficiency diseases has had major consequences for the practice of medicine. New knowledge of the mechanisms responsible for these disorders has contributed not only to our ability to treat these relatively few patients but also to our understanding of the immunity systems themselves in relation to the rest of the body economy. In turn, the growing fund of information about cells of the lymphoid system, their function and organization, has extended our clinical resources. This ongoing mutual enrichment between basic science and clinical analysis is our best hope as we search for ways to control the suffering caused by the primary immunodeficiencies and other human diseases.
The studies on which this review is based were supported by USPHSR Grants CA-19267, CA-08748, CA-17404, AI-11843, and NS-11457, and by the Judith Harris Selig Memorial Fund, the Zelda R. Weintraub Cancer Fund, and the Fund for the Advanced Study of Cancer.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Ackert, C., Plüss, H. J., and Hitzig, W. H., 1976, Hereditary severe combined immunodeficiency and adenosine deaminase deficiency, Pediatr. Res. 10: 67.
Aiuti, F., LaCava, V., Garofalo, J. A., D’Amelio, R., and D’Asero, C., 1973, Surface markers on human lymphocytes: Studies of normal subjects and of patients with primary immunodeficiencies, Clin. Exp. Immunol. 15: 43.
Aiuti, F., Cerottini, J.-C., Coombs, R. R. A., Cooper, M., Dickler, H. B., Fryiland, S., Fudenberg, H. H., Greaves, M. F., Grey, H. M., Kunkel, H. G., Natvig, J., Preud’homme, J.-L., Rabellino, E., Ritts R. E., Rowe, D. S., Seligmann, M., Siegal, F. P., Stjernsward, J., Terry, W. D., and Wybran, J., 1974, Special technical report: Identification, enumeration and isolation of B and T lymphocytes from human peripheral blood. Report of a WHO/IARC-sponsored workshop on human B and T cells, London, July, 1974, Scand, J. Immunol. 3: 521.
Aiuti, F., Ammirati, P., Fiorelli, M., Franchi, F., Calvani, N., and Businco, L., 1979, Immunologic and clinical investigations on a bovine thymic extract. Therapeutic applications in primary immunodeficiencies, Pediatr. Res. 13: 797.
Alper, C. A., Cotten, H. R., Rosen, F. S., Rabson, A. R., Macnab, G. M., and Gear, J. S. S., 1972, Homozygous deficiency of the third component of complement (C3) in a patient with repeated infections, Lancet 2: 1179.
Ammann, A. J., and Hong, R., 1973, Selective IgA Deficiency, in: Immunologic Disorders in Infants and Children ( E. R. Stiehm and V. Fulginiti, eds.), Saunders, Philadelphia.
Anderson, I. M., 1975, Bone marrow transplants, Proc. R. Soc. Med. 68: 577.
Archer, O. K., and Pierce, J. C., 1961, Role of the thymus in development of the immune response, Fed. Proc. 20: 26.
Atwater, J. S., and Tomasi, T. B., 1978, Suppressor cells in IgA deficiency, Clin. Immunol. Immunopathol. 9: 379.
August, C. S., Rosen, F. S., Filler, R. M., Janeway, C. A., Markowski, A., and Kay, H. E. M., 1968, Implantation of a foetal thymus restoring immunological competence in a patient with thymic aplasia (DiGeorge syndrome), Lancet 2: 1210.
August, C. S., Levey, R. H., Berkej, A. I., Rosen, F. S., and Kay, H. E. M., 1970, Establishment of immunological competence in a child with congenital thymic aplasia by a graft of fetal thymus, Lancet 1: 1080.
Bach, F. H., Albertini, R. J., Joo, P., Anderson, J. J., and Bortin, M. M., 1968, Bone marrow transplantation in a patient with Wiskott-Aldrich syndrome, Lancet 2: 1364.
Bachman, R., 1965, Studies on the serum Y-A-globulin level, III. The frequency of A-Y A-globulinemia, Scand. J. Clin. Lab. Invest. 17: 316.
Baehner, R. L., and Karnovsky, M. L., 1968, Deficiency of reduced nicotinamide adenine dinucleotide oxidase in chronic granulomatous disease, Science 162: 1277.
Baehner, R. L., and Nathan, D. G., 1967, Leukocyte oxidase: Defective activity in chronic granulomatous disease, Science 155: 835.
Ballet, J. J., Griscelli, C., Coutris, C., Milhaud, G. and Maroteaux, P., 1977, Bone marrow transplantation in osteopetrosis (letter), Lancet 2: 1137.
Ballow, M., and Hyman, L. R., 1977, Combination immúnotherapy in chronic mucocutaneous candidiasis: Synergism between transfer factor and fetal thymus tissue, Clin. Immunol. Immunopathol. 8: 504.
Ballow, M., Day, N. K., Biggar, W. D., Park, B. H., Yount, W. J., and Good, R. A., 1973, Reconstitution of Clq following bone marrow transplantation in patients with severe combined immunodeficiency, Clin. Immunol. Immunopathol. 2: 28.
Ballow, M., Shirer, J. E., Harden, L., Yang, S. Y., and Day, N. K., 1975, Complete absence of the third component of complement in man, J. Clin. Invest. 56: 703.
Barandun, S., Huser, H. J., and Hassiz, A., 1958, Klinische Erscheinungsformen des Antikbrpermangelsyndroms, Schweiz. Med. Wochenschr. 88: 78.
Barandun, S., Riva, G., and Spengler, G. A., 1968, Immunologic deficiency diagnosis; forms and current treatment, Birth Defects, 4 (1): 40.
Beck, P., Willis, D., Davies, G. T., Lachmann, P. J., and Sussman, M., 1973, A family study of hereditary angioneurotic oedema, Q. J. Med. 42: 317.
Berendes, H., Bridges, R. A., and Good, R. A., 1957, Fatal granulomatous disease of childhood: Clinical study of a new syndrome, Minn. Med. 40: 309.
Biggar, W. D., Stutman, O., and Good, R. A., 1972, Morphological and functional studies of fetal thymus transplants in mice. J. Exp. Med. 135: 793.
Biggar, W. D., Park, B. H., and Good, R. A., 1975a, Compatible bone marrow transplantation and immunologic reconstitution of combined immunodeficiency disease, Birth Defects 11 (1): 385.
Biggar, W. D., Park, B. H., Stutman, O., Gajl-Peczalska, K., and Good, R. A., 1975b, Fetal thymus transplantation: Experimental and clinical observations, Birth Defects 11 (1): 361.
Blaese, R. M., Strober, W., Brown, R. S., and Waldmann, T. A., 1968, The Wiskott-Aldrich syndrome: A disorder with a possible defect in antigen processing or recognition, Lancet 1: 1056.
Blaese, R. M., Strober, W., Levy, A. L., and Waldmann, T. A., 1971, Hypercatabolism of IgG, IgA, IgM and albumin in the Wiskott-Aldrich syndrome, J. Clin. Invest. 50: 2331.
Blaese, R. M., Strober, W., and Waldmann, T. A., 1975, Immunodeficiency in the Wiskott-Aldrich syndrome, Birth Defects 11 (1): 250.
Goder, E., and Sedgwick, R. P., 1957, Ataxia-telangiectasia: A familial syndrome of progressive cerebellar ataxia, oculocutaneous telangiectasia and frequent pulmonary infection, Univ. S. Calif. Med. Bull. 9: 15.
Goder, E., and Sedgwick, R. P., 1962, Ataxia-telangiectasia: A review of 101 cases, in: Cerebellum, Posture and Cerebral Palsy ( G. Walsh, ed.), Heinemann Medical Books, London.
Bortin, M. M., and Rimm, A. A., 1977, Severe combined immunodeficiency. Characterization of the disease and results of transplantation, J. Am. Med. Assoc. 238: 591.
Boxer, L. A., Watanabe, A. M., Rister, M., Besch, H. R., Jr., Allen, J., and Baehner, R. L., 1976, Correction of leukocyte function in Chediak-Higashi syndrome by ascorbate, N. Engl. J. Med. 295: 1041.
Bayer, J. T., Gall, E. P., Norman, M. E., Nilsson, U. R., and Zimmerman, T. S., 1975, Hereditary deficiency of the seventh component of complement, J. Clin. Invest. 56: 905.
Bridges, R. A., Berendes, H., and Good, R. A., 1959, A fatal granulomatosis of childhood. The clinical, pathological, and laboratory features of new syndrome, Am. J. Dis. Child. 97: 387.
Broder, S., Edelson, R. L., Lutzner, M. A., Nelson, D. L., MacDermott, R. P., Durm, M. E., Goldman, C. K., Meade, B. D., and Waldmann, T. A., 1976, The Sézary Syndrome. A malignant proliferation of helper T cells, J. Clin. Invest. 58: 1297.
Broom, B. D., de la Concha, E. G., Webster, A. D. B., Janossy, G. J., and Asherson, G. L., 1976, Intracellular immunoglobulin production in vitro by lymphocytes from patients with hypogammaglobulinemia and their effect on normal lymphocytes, Clin. Exp. Immunol. 23: 77.
Bruton, O. C., 1952, Agammaglobulinemia, Pediatrics 9: 722.
Buckley, R. H., 1972, Plasma therapy in immunodeficiency disease, Am. J. Dis. Child. 124: 376.
Buckley, R. H., Whisnant, J. K., Schiff, R. I., Gilbertsen, R. B., Huang, A. T., and Platt, M. S., 1976, Correction of severe combined immunodeficiency by fetal liver cells. N. Engl. J. Med. 294: 1076.
Buckley, R. M., and Dees, S. C., 1969, Correlation of milk precipitins with IgA deficiency, N. Engl. J. Med. 281: 465.
Carson, M. J., Chadwick, D. L., Brubaker, C. A., Clelan, R. S., and Landing, B. H., 1968, Thirteen boys with progressive septic granulomatosis, Pediatrics 35: 405.
Cassidy, J. T., Burt, A., Petty, P., and Sullivan, D., 1969, Selective IgA deficiency in connective tissue diseases, N. Engl. J. Med. 280: 275.
Chilgren, R. A., Quie, P. G., Meuwissen, H. J., and Hong, R., 1967, Chronic mucocutaneous candidiasis, deficiency of delayed hypersensitivity, and selective local antibody defect, Lancet 2: 688.
Chilgren, R. A., Meuwissen, H. J., Good, R. A., and Hong, R., 1969, The cellular immune defect in chronic mucocutaneous candidiasis, Lancet 1: 1286.
Clark, R. A., and Kimball, H. R., 1971, Defective granulocyte chemotaxis in the Chediak-Higashi syndrome, J. Clin. Invest. 50: 2645.
Cleveland, W. W., 1975, Immunologic reconstitution in the DiGeorge syndrome by fetal thymic transplant, Birth Defects 11 (1): 352.
Cleveland, W. W., Fogel, B. J., Brown, W. T., and Kay, H. E. M., 1968, Foetal thymic transplant in a case of DiGeorge syndrome, Lancet 2: 1211.
Coccia, P. S., Cervenka, J., Teitelbaum, S. L., Kahn, A., Clawson, C. C., and Brown, D. M., 1979, Reversal of human osteopetrosis by bone marrow transplantation: Etiologic implications, Clin Res. 27: 483A.
Choen, G., and Peterson, A., 1972, Treatment of hereditary angioedema with frozen plasma, Ann. Allergy 30: 690.
Conn, H. O., and Quintiliani, R., 1966, Severe diarrhea controlled by gamma globulin in a patient with agammaglobulinemia, amyloidosis, and thymoma, Ann. Intern. Med. 65: 526.
Conn, H. O., Binder, H., and Burns, B., 1968, Pernicious anemia and immunologic deficiency, Ann. Intern. Med. 68: 603.
Cooper, M. D., and Lawton, A. R., 1972, Circulating B cells in patients with immunodeficiency, Am. J. Pathol. 69: 513.
Cooper, M. D., Chase, H. P., Lowman, J. T., Krivit, W., and Good, R. A., 1968, Immunologic defects in patients with Wiskott-Aldrich syndrome, Birth Defects 4 (1): 378.
Cooper, M. D., Lawton, A. R., and Bockman, D. E., 1971, Agammaglobulinemia with B lymphocytes. Specific defect of plasma cell differentiation, Lancet 2: 791.
Cooper, M. R, DeChatelet, L. R., Metcall, C. E., La Via, M. F., Spurr, C. L., and Baehner, R. L., 1970, Leukocyte G.-6-P.D. deficiency, Lancet 2: 110.
Copenhagen Study Group of Immunodeficiencies, 1976, Bone marrow transplantation from parent to child (both DW-2 homozygous), in: International Cooperative Group for Bone Marrow Transplantation in Man, Third Workshop. Tarrytown, N.Y.
Cottier, H., 1958, Zur Histopathologie des Antikörpermangelsyndroms, Tr. 6th Congr. Europ. Soc. Haematol., Copenhagen, 1957. Karger, Basel.
Cunningham-Rundles, C., and Good, R. A., 1979, Selective IgA deficiency, Birth Defects Compendium, Second Edition (D. Bergsma, ed.), p. 580, Alan R. Liss, New York.
Cunningham-Rundles, C., Brandeis, W. E., Good, R. A., and Day, N. K., 1978, Milk precipitins, circulating immune complexes and IgA deficiency, Proc. Natl. Acad. Sci. USA 75: 3387.
Cunningham-Rundles, C., Brandeis, W. B., Pudifin, D. J., Day, N. K., and Good, R. A., 1980, Autoimmunity in selective IgA deficiency: Relationship to anti-bovine protein antibodies, circulating immune complexes, and arthritis (submitted).
Cunningham-Rundles, C., Brandeis, W. E., Safai, B., O’Reilly, R., Day, N. K. and Good, R. A., 1979a, Selective IgA deficiency and circulating immune complexes containing bovine proteins in a child with chronic graft-vs.-host disease, Am. J. Med. 67: 883.
Cunningham-Rundles, C., Brandeis, W. E., Good, R. A. and Day, N. K., 1979b, Bovine antigens and the formation of circulating immune complexes in selective immunoglobulin A deficiency, J. Clin. Invest. 64: 272.
Cunningham-Rundles, C., Brandeis, W. E., Pudifin, D. J., Day, N. K., and Good, R. A., 1979c, Autoimmunity, arthritis, milk precipitins, and circulating immune complexes in selective IgA deficiency, Clin. Res. 27: 475A.
Day, N. K., and Good, R. A., 1975, Deficiencies of the complement system in man, Birth Defects 11 (1): 306.
Day, N. K., and Good, R. A., 1980, The complement system in human disease, in: Infections in the Abnormal Host (M. H. Grieco, ed.), p. 38, Yorke Medical Books, New York.
Day, N. K., Geiger, H., Stroud, R., de Bracco, M., Moncada, B., Windhorst, D., and Good, R. A., 1972, Clr deficiency: An inborn error associated with cutaneous and renal disease, J. Clin. Invest. 51: 1102.
Day, N. K., Geiger, H., McLean, R., Michael, A., and Good, R. A., 1973, C2 deficiency. Development of lupus erythematosus, J. Clin. Invest. 52: 1601.
Day, N. K., Degos, L., Beth, M., Sasportes, M., Gharbi, R., and Giraldo, G., 1976a, C8 deficiency in a family with xeroderma pigmentosum. Lack of linkage to the HL-A region, in: HLA and Disease, Vol. 58, INSERM, Paris, p. 197 (abstr.).
Day, N. K., Jersild, C., Rubinstein, P., and Good, R. A., 1976b, Inherited complement deficiencies and the major histocompatibility system in man, in: Clinical Aspects of the Complement System, Report of the International Symposium, Bochum (W. Opferkuch, K. Rother, and D. R. Schultz, eds.), pp. 187–199, Georg Thieme, Stuttgart.
Day, N. K., Moncada, B., and Good, R. A., 1977, Inherited deficiencies of the complement system, in: Comprehensive Immunology, Vol. 2, Biological Amplification Systems in Immunology ( N. K. Day and R. A. Good, eds.), p. 229, Plenum Press, New York.
de Vaal, D. M. and Seynhaeve, V., 1959, Reticular dysgenesia, Lancet 2: 1123.
DiGeorge, A. M., 1965, A new concept of the cellular basis of immunity, J. Pediatr. 67: 907.
DiGeorge, A. M., 1968, Congenital absence of the thymus and its immunologic consequences: Concurrence and congenital hypoparathyroidism, Birth Defects, 4 (1): 116.
Dosch, H. M., and Gelfand, E. W., 1976, In vitro antibody synthesis and suppression in humoral immunodeficiency states, Clin. Res. 25: 418A.
Dosch, H., Percy, M. E. and Gelfand, E. E., 1977, Functional differentiation of B lymphocytes in congenital agammaglobulinemia, J. Immunol. 119: 1959.
Dupont, B., Hansen, J., Good, R. A., and O’Reilly, R. J., 1977, Histocompatibility testing for clinical bone marrow transplantation, in: HLA System—New Aspects, ( G. B. Ferrara, ed.), p. 153 Elsevier/North Holland Biomedical Press, New York.
Dupont, B., O’Reilly, R. J., Pollack, M. S., and Good, R. A., 1979, Use of HLA genotypically different donors in bone marrow transplantation, Transplant. Proc. 11: 219.
Fogh, J. E., and Giovanella, B. C., eds., 1978, The Nude Mouse in Experimental and Clinical Research, Academic Press, New York.
Foroozonfar, N., Hobbs, J. R., Hugh-Jones, K., Humble, J. G., James, D. C. O., Selwyn, S., Watson, J. G., and Yamamura, M., 1977, Bone marrow transplant from an unrelated donor for chronic granulomatous disease, Lancet 1: 210.
Forssman, O., and Herner, B., 1964, Acquired agammaglobulinemia and malabsorption, Acta Med. Scand. 176: 779.
FrqSland, S. S., and Natvig, J. B., 1973, Identification of three different human lymphocyte populations by surface markers, Transplant. Rev. 16: 114.
Fudenberg, H. H., Good, R. A., Goodman, H. C., Hitzig, W., Kunkel, H. G., Roitt, I. M., Rosen, F. S., Rowe, D. S., Seligmann, M., and Soothill, J. R., 1971, Primary immunodeficiencies, Bull. WHO 45: 125.
Gajl-Peczalska, K., Park, B. H., Biggar, W., and Good, R. A., 1973, B and T lymphocytes in primary immunodeficiency disease in man, J. Clin. Invest. 52: 919.
Gallin, J. I., Klimerman, J. A., Padgett, G. A., and Wolff, S. M., 1975, Defective mononuclear leukocyte chemotaxis in the Chediak-Higashi syndrome of humans, mink, and cattle, Blood 45: 863.
Gallin, J. I., Elin, R. J., Hubert, R. T., Fauci, A. S., Kaliner, M. A., and Wolff, S. M., 1979, Efficacy of ascorbic acid in Chediak-Higashi syndrome (CHS): Studies in humans and mice, Blood 53: 226.
Gatti, R. A., and Good, R. A., 1971, Occurrence of malignancy in immunodeficiency disease: A literature review, Cancer 28: 89.
Gatti, R. A., Meuwissen, H. J., Allen, H. D., Hong, R., and Good, R. A., 1968, Immunologic reconstitution of sex-linked lymphopenic immunologic deficiency, Lancet 2: 1366.
Gatti, R. A., Platt, N., Hong, R., Langer, L. O., Kay, H. E. M., and Good, R. A., 1969, Hereditary lymphopenic agammaglobulinemia associated with a distinctive form of short-limbed dwarfism and ectodermal dysplasia, J. Pediatr. 75: 675.
Gatti, R. A., Gershanik, J. J., Levkoff, A. H., Wertelecki, W., and Good, R. A., 1972, Digeorge syndrome associated with combined immunodeficiency, J. Pediatr. 81: 920.
Geha, R. S., 1976, Is the B cell abnormality secondary to T cell abnormality in severe combined immunodeficiency? Clin. Immunol. Immunopathol. 6: 102.
Geha, R. S., Rosen, F. S., and Merler, E., 1973, Identification and characterization of subpopulations of lymphocytes in human peripheral blood after fractionation on discontinuous gradients of albumin. The cellular defect in X-linked agammaglobulinemia, J. Clin. Invest. 52: 1726.
Geha, R. S., Gatien, J. G., Merler, E., and Rosen, F. S., 1975, Assessment of the B lymphocyte population in agammaglobulinemia, Birth Defects 11 (1): 36.
Geha, R. S., Malakian, A., Le Franc, G., Chayban, D., and Serre, J. L., 1976, Immunologic reconstitution in severe combined immunodeficiency following transplan - tation with parental bone marrow, Pediatrics 58: 451.
Gelfand, E. W., Dosch, H. M., Huber, J., and Shore, A., 1977, In vitro and in vivo reconstitution of severe combined immunodeficiency disease with thymic epithelium, Clin. Res. 25: 358A.
Gelfand, J. A., Sherins, R. J., Allings, D. W., and Frank, M. M., 1976, Treatment of hereditary angioedema and Danazol. Reversal of clinical and biochemical abnormalities, N. Engl. J. Med. 295: 1444.
Gelfand, J. A., Boss, G. R., Reinhart, R., and Frank, M. M., 1979, Acquired Cl esterase inhibitor deficiency and angioedema: A review, Medicine 58: 321.
Gelzayd, E. A., McCleery, J. L., Melnyk, C. S., and Kraft, S. C., 1971, Intestinal malabsorption and immunoglobulin deficiency, Arch. Intern. Med. 127: 141.
Giblett, E. R., Anderson, J. E., Cohen, F., Pollara, B., and Meuwissen, H. J., 1972, Adenosine deaminase deficiency in two patients with severely impaired cellular immunity, Lancet 2: 1067.
Giblett, E. R., Ammann, A. J., Wara, D. W., Sandman, R., and Diamond, L. K., 1975, Nucleoside phosphorylase deficiency in a child with severe defective T cell immunity, Lancet 1: 1010.
Glanzman, E., and Riniker, P., 1950, Essentielle Lymphocytophthise. Ein neues Krankheitsbild aus der Säuglings-Pathologie, Ann. Paediatr. 175: 1.
Goldstein, A. L., Geha, A., Katz, M. M., Hardy, M. A., and White, A., 1972, Purification and biological activity of thymosin, a hormone of the thymus gland, Proc. Natl. Acad. Sci. USA 69: 1800.
Goldstein, G., 1975, The isolation of thymopoietin (thymin), Ann. N. Y. Acad. Sci. 249: 177.
Good, R. A., 1954a, Agammaglobulinemia: A provocative experiment of nature, Bull. Univ. Minn. Hosp. Minn. Med. Found. 26: 1.
Good, R. A., 1954b, Agammaglobulinemia: An experimental study, Am. J. Dis. Child. 88: 625.
Good, R. A., 1955, Studies on agammaglobulinemia. II. Failure of plasma cell formation in bone marrow and lymph nodes of patients with agammaglobulinemia, J. Lab. Clin. Med. 46: 167.
Good, R. A., 1969, Immunologic reconstitution: The achievement and its meaning, Hosp. Pract. 4: 41.
Good, R. A., 1972, Studies on immunodeficiency in man, Am. J. Pathol. 69: 484.
Good, R. A., 1973, Immunodeficiency in developmental perspective, Harvey Lect. 67: 1.
Good, R. A., 1977, Cellular engineering, Clin. Bull. Memorial Sloan-Kettering Cancer Center 7: 33.
Good, R. A., 1979, Immunodeficiency, common variable, in: Birth Defects Compendium, 2nd ed. ( D. Bergsma, ed.), p. 574, Alan R. Liss, New York.
Good, R. A., and Rötstein, J., 1960, Rheumatoid arthritis and agammaglobulinemia, Bull. Rheum. Dis. 10: 203.
Good, R. A., and Varco, R. L., 1955, A clinical and experimental study of agammaglobulinemia, Lancet 75: 245.
Good, R. A., Bridges, R. A., Zak, S. J., and Pappenheimer, A. M., Jr., 1959, Delayed hypersensitivity in agammaglobulinemia, in: Mechanisms of Hypersensitivity (J. J. Shaffer, G. LoGrippo, and M. W. Chase, eds.), p. 467, Little, Brown, Boston.
Good, R. A., Dalmasso, A. P., Martinez, C., Archer, O. K., Pierce, J. C., and Papermaster, B. W., 1962, The role of the thymus in development of immunologic capacity in rabbits and mice, J. Exp. Med. 116: 773.
Gupta, S., Good, R. A., and Siegal, F. P., 1976, Rosette-formation with mouse erythrocytes. III. Studies in patients with primary immunodeficiency and lymphoproliferative disorders, Clin. Exp. Immunol. 26: 204.
Haliotis, R., Roder, J., Klein, M., Ortaldo, J., Fauci, A. S., and Herberman, R. B., 1980, Chédiak—Higashi gene in humans. I. Impairment of the natural-killer cell function, J. Exp. Med. 151: 1039.
Hantley, C. C., Thorpe, D. P., Lyerly, A. D., and Kelsey, W. M., 1967, Rheumatoid arthritis with IgA deficiency, Am. J. Dis. Child. 113: 411.
Hathaway, W. E., Brangle, R. W., Nelson, T. L., and Roeckel, I. E., 1966, Aplastic anemia and alymphocytosis in an infant with hypogammaglobulinemia: Graf tversus-host reaction ? J. Pediatr. 68: 713.
Hathaway, W. E., Fulginiti, V. A., Pierce, C. W., Githen, J. H., Pearlman, D. S., Muschenheim, F., and Kempe, C. H., 1967, Graft-versus-host reaction following a single blood transfusion, J. Am. Med. Assoc. 201: 1015.
Hauptman, G., Grosshans, E., and Heid, E., 1974, Lupos erythemateux aigus et déficits héréditaires en complement: A propos d’un cas par déficit complement en C, Ann. Dermatol. Syphiligr. 101: 479.
Hayward, A. R., and Greaves, M. F., 1975, Central failure of B-lymphocyte induction in pan-hypogammaglobulinemia, Clin. Immunol. Immunopathol. 3: 461.
Hecht, F., and McCaw, B. K., 1977, Chromosome instability syndromes, in: Genetics of Human Cancer ( J. Mulvihill, R. W. Miller, and F. Fraumeni, eds.), p. 105. Raven Press, New York.
Hermans, P. E., 1967, Nodular lymphoid hyperplasia of the small intestine and hypogammaglobulinemia: Theoretical and practical considerations, Fed. Proc. 26: 1606.
Hermans, P. E., and Huizenga, K. A., 1972, Association of gastric carcinoma with idiopathic late-onset immunoglobulin deficiency, Ann. Intern. Med. 76: 605.
Hermans, P. E., Diaz-Buxo, J. A., and Stobo, J. D., 1976, Idiopathic late-onset immunoglobulin deficiency: Clinical observations in 50 patients, Am. J. Med. 61: 221.
Hitzig, W. H., 1973, Congenital thymic and lymphocytic deficiency disorders, in: Immunologic Disorders in Infants and Children ( E. R. Stiehm and V. A. Fulginiti, eds.), p. 214, Saunders, Philadelphia.
Hitzig, W. H., and Munterer, U., 1975, Conventional immunoglobulin therapy, Birth Defects 11 (1): 339.
Hitzig, W. H., and Willi, H., 1961, Hereditäre lymphoplasmocytäre Dysgenesie, Schweiz. Med. Wochenschr. 91: 1625.
Hitzig, W. H., Dohmann, V., Pliiss, H. J., and Vischer, D., 1974, Hereditary transcobalamin II deficiency: Clinical findings in a new family, J. Pediatr. 85: 662.
Hohn, D. C., and Lehrer, R. I., 1975, NADPH-oxidase deficiency in X-linked chronic granulomatous disease, J. Clin. Invest. 55: 707.
Holmes, B., Quie, P. G., Windhorst, D. B., and Good, R. A., 1966, Fatal granulomatous disease of childhood, an inborn abnormality of phagocytic function, Lancet 1: 1225.
Holmes, B., Park, B. H., Malawista, S. E., Quie, P. G., Nelson, D. L., and Good, R. A., 1970, Chronic granulomatous disease in females: A deficiency of leukocyte glutathione peroxidase, N. Engl. J. Med. 283: 217.
Hong, R., Ammann, A. J., Huang, S. W., Levy, R. L., Davenport, G., Bach, M. L., Bach, F. H., Bortin, M. M., and Kay, H. E. M., 1972, Cartilage-hair hypoplasia: Effect on thymus transplants, Clin. Immunol. Immunopathol. 1: 15.
Hong, R., Santosham, M., Schulte-Wissermann, H., Horowitz, S., Hsu, S. H., and Winkelstein, J. A., 1976, Reconstitution of B and T lymphocytes function in severe combined immunodeficiency disease after transplantation with thymic epithelium, Lancet 2: 1270.
Hong, R., Schulte-Wisserman, H., Horowitz, S., Borzy, M., and Finlay, J., 1978, Cultured thymic epithelium in severe combined immunodeficiency, Transplant. Proc. 10: 201.
Horowitz, S. D., and Hong, R., 1977, The pathogenesis and treatment of immunodeficiency, in: Monographs in Allergy, Vol. 10 ( P. Kallos, T. M. Inderbitzin, Z. Trnka, A. L. de Weck, and B. H. Waksman, eds.), p. 101, Karger, Basel.
Huff, D. S., and Lischner, H. W., 1971, The lymph nodes in the imcomplete III-IV pharyngeal pouch syndrome, Am. J. Pathol. 62: 199.
Hughes, W. S., Cerda, J. J., Holtzapple, P., and Brooks, F. P., 1971, Primary hypogammaglobulinemia and malabsorption, Ann. Intern. Med. 74: 903.
Huizenga, K. A., Wollaeger, E. E., Green, P. A., and McKenzie, B. F., 1961, Serum globulin deficiencies in non-tropical sprue, with report of two cases of acquired agammaglobulinemia, Am. J. Med. 31: 572.
Huntley, C. C., Thorpe, D. P., Lyerly, A. D., and Kelsey, W. M., 1967, Rheumatoid arthritis with IgA deficiency, Am. J. Dis. Child. 113: 411.
Inai, S., Kitamura, H., Hiramatsu, S., and Nagaki, K., 1979, Deficiency of the ninth component of complement in man (Brief communications), J. Clin. Lab. Immunol. 2: 85.
Incefy, G. S., Dardenne, M., Pahwa, S., Grimes, E., Pahwa, R., Smithwick, E., O’Reilly, R., and Good, R. A., 1977, Thymic activity in severe combined immunodeficiency, Proc. Natl. Acad. Sci. USA 74: 1250.
Iwata, T., Incefy, G. S., Cunningham-Rundles, S., Cunningham-Rundles, C., Smithwick, E., O’Reilly, R., and Good, R. A., 1980, Circulating thymic hormone activity (FTS) in patients with primary and secondary immunodeficiency diseases (submitted).
Jaffee, C. J., Atkinson, J. P., Gelfand, J. A., and Frank, M. M., 1975, Hereditary angioedema: The use of fresh frozen plasma for prophylaxis in patients undergoing oral surgery, J. Allergy Clin. Immunol. 55: 386.
Jeunet, F. S., and Good, R. A., 1968, Thymoma, immunologic deficiencies and hematological abnormalities, Birth Defects 4 (1): 192.
Jeunet, I., 1965, Thymome et syndrome de carence en immunoglobulines, Schweiz. Med. Wochenschr. 95: 1419.
Johnson, S. M., North, M. E., Asherson, G. L., Allsop, J., Watts, R. W. E., and Webster, A. D. B., 1977, Lymphocyte purine 5’ nucleotidase deficiency in primary hypogammaglobulinemia, Lancet 1: 168.
Keczkes, K., Bilimoria, S., and Piercy, D. M., 1979, Pernicious anemia and granulomatous skin lesions in a case of common variable hypogammaglobulinemia, Br. J. Dermatol. 101: 211.
Keightley, R. G., Lawton, A. R., Cooper, M. D., and Yunis, E. J., 1975, Successful fetal liver transplantation in a child with severe combined immunodeficiency, Lancet 2: 850.
Kirkpatrick, C. H., and Smith, T. K., 1974, Chronic mucocutaneous candidiasis: Immunologic and antibiotic therapy, Ann. Intern. Med. 80: 310.
Kirkpatrick, C. H., and Windhorst, D. B., 1979, Mucocutaneous candidiasis and thymoma, Am. J. Med. 66: 939.
Kirkpatrick C. H., Ottenson, E. A., Smith, T. K., Wells, S. A., and Burdick, J. F., 1976, Reconstitution of defective cellular immunity with foetal thymus and dialyzable transfer factor: Long-term studies in a patient with chronic mucocutaneous candidiasis, Clin. Exp. Immunol. 23: 414.
Koch, C., Henriksen, K:, Juhl, F., Wiik, A., Faber, V., Andersen, V., Dupont, B., Hansen, G. S., Svejgaard, A., Thomsen, M., Ernst, P., Killmann, S. A., Good, R. A., Jensen, K., and Müller-Berat, N., 1973, Bone marrow transplantation from an HL-A non-identical but mixed-lymphocyte culture identical donor, Lancet 1: 1146.
Koistinen, J., 1975, Selective IgA deficiency in blood donors, Vox Sang. 29: 192.
Krivit, W., and Good, R. A., 1959, Aldrich’s syndrome (thrombocytopenia, eczema and infection in infants), Am. J. Dis. Child. 97: 137.
Larsson, S. O., Hagelquist, E., and Coster, C., 1961, Hypogammaglobulinaemia and pernicious anemia, Acta Haematol. 26: 50.
Leddy, J. P., Frank, M. M., Gaither, T., Baum, J., and Klemperer, M. R., 1974, Hereditary deficiency of the sixth component of complement in man, J. Clin. Invest. 53: 544.
Lee, F. I., Jenkins, G. C., Hughes, D. T. D., and Kazantzis, G., 1964, Pernicious anaemia, myxoedema, and hypogammaglobulinaemia: A family study, Br. Med. J. 1: 598.
Levy, R., Yuang, S. W., Bach, M. A., Bach, F. H., Hong, R., Ammann, A. J., Bortin, M., and Kay, H. E. M., 1972, Thymic transplantation in a case of chronic mucocutaneous candidiasis, Lancet 2: 1898.
Lewis, V., Twomey, J. J., Goldstein, G., O’Reilly, R., Smithwick, E., Pahwa, R., Pahwa, S., Good, R. A., Schulte-Wisserman, H., Horowitz, S., Hong, R., Jones, J., Sieber, O., Kirkpatrick, C., Polmar, S., and Bealmear, P., 1977, Circulating thymic hormone activity in congenital immunodeficiency, Lancet 2: 471.
Lint, T. F., Zeitz, H. J., and Gewurz, H., 1980, Inherited deficiency of the ninth component of complement (submitted).
Lopukhin, Y., Morosov, Y., and Petrov, R., 1973, Transplantation of neonate thymus-sternum complex in ataxia-telangiectasia, Transplant. Proc. 5: 823.
Lux, S. E., Johnston, R. B., August, C. S., Say, B., Penchaszadeh, V. B., Rosen, F. S., and McKusick, V. A., 1970, Chronic neutropenia and abnormal cellular immunity in cartilage-hair hypoplasia, N. Engl. J. Med. 282: 231.
MacLean, L. D., Zak, S. J., Varco, R. L., and Good, R. A., 1956, Thymic tumor and acquired agammaglobulinemia: A clinical and experimental study of the immune response, Surgery 40: 1010.
Malawista, S. E., and Gifford, R. H., 1975, Chronic granulomatous disease of childhood with leukocyte glutathione peroxidase deficiency in a brother and sister: A likely autosomal recessive inheritance, Clin. Res. 23: 416A.
Mancini, G., Carbonara, A. O., and Heremans, J. F., 1965, Immunochemical quantitation of antigens by single radial immunodiffusion, Immunochemistry 2: 235.
Marsh, W. L., Oyen, R., Nichols, M. E., and Allen, F. H., Jr., 1975a, Chronic granulomatous disease and the Kell blood groups, Br. J. Haematol. 29: 247.
Marsh, W. L., Uretsky, S. C., and Douglas, S. D., 1975b, Antigens of the Kell blood group system on neutrophils and monocytes: Their relation to chronic granuloma-tous disease, J. Pediatr. 87: 1117.
Matthews, L. W., Doershuk, C. F., Stern, R. C., Tucker, A. S., and Boat, T. F., 1973, Comprehensive and preventive treatment of cystic fibrosis, in: Fundamental Problems of Cystic Fibrosis and Related Diseases, Symposia Specialists, Miami, Fla.
Mazzitello, W. F., and Good, R. A., 1956, Agammaglobulinemia, Minn. Med. 39: 308.
McKenzie, M. R., and Paglieroni, T., 1975, Class specific Ig suppression in variable immunodeficiency, Blood 46: 1019.
McKusick, V. A., Eldridge, E., Hostetler, J. A., Ruangwit, V., and Egeland, J. A., 1964, Dwarfism in the Amish. II. Cartilage-hair hypoplasia, Bull. Johns Hopkins Hosp. 116: 285.
Meuwissen, H. F., Pollara, B., and Pickering, R. J., 1975, Combined immunodeficiency disease associated with adenosine deaminase deficiency, Report on a Workshop, Albany, 1973, J. Pediatr. 86: 169.
Meuwissen, J. H., Kieserman, M. A., Taft, E. G., Pollara, B., and Pickering, R. J., 1978, Marrow transplantation (MTP) in Wiskott-Aldrich syndrome (WAS): T cell engraftment with cyclophosphamide (CY), complete engraftment with total body irradiation, Pediatr. Res. 12: 483.
Miller, M. E., 1968, Graft-versus-host reactions in man with special reference to thymic dysplasia, Birth Defects 4 (1): 257.
Miller, M. E., and Koblenzer, P. J., 1972, Leiner’s disease and deficiency of C5, J. Pediatr. 80: 879.
Miller, M. E., and Nilsson, U. R., 1970, A familial deficiency of the phagocytosis-enhancing activity of serum related to a dysfunction of the fifth component of complement (C5), N. Engl. J. Med. 282: 354.
Mills, E. L., Rholl, K. S., and Quie, P. G., 1978, Chemiluminescence: A rapid sensitive method for detection of patients with chronic granulomatous disease and identification of carriers, Pediatr. Res. 12: 454 (abstr.).
Mills, E.. L., Rholl, K. S., and Quie, P. G., 1980, X-linked inheritance in females with chronic granuloma-tous disease (submitted).
Mills, J. V., Mochaeli, D., and Fudenberg, H. H., 1977, Antibodies to human collagen in subjects with selective IgA deficiency, Clin. Exp. Immunol. 13: 203.
Montes, L. F., Carter, R. E., Moreland, N., and Ceballos, R., 1968, Generalized cutaneous candidiasis associated with diffuse myopathy and thymoma, J. Am. Med. Assoc. 204: 351.
Montes, L. F., Ceballos, R., Cooper, M. D., Bradley, M. N., and Bockman, D. E., 1972, Chronic mucocutaneous candidiasis, myositis, and thymoma, J. Am. Med. Assoc. 222 (13): 1619.
Müller-Rucholtz, W., Wottge, H. U., and Müller-Hermelink, H. F., 1976, Bone marrow transplantation in rats across strong histocompatibility barriers by selective elimination of lymphoid cells in donor marrow, Transplant. Proc. 8: 537.
Nezelof, C., Jammet, M. L., Lortholary, P., Labrune, B., and Lamy, M., 1964, L’hypoplasie héréditaire du thymus. Sa place et sa résponsibilité dans une observation d’aplasie lymphocytaire normoplasmocytaire et normoglobulinémique du nourrisson, Arch. Fr. Pediatr. 21: 897.
Niethammer, D., Goldman, S. F., Haas, R. J., Dietrich, M., Flad, H. B., Fliedner, R. M., and Kleihaver, E., 1976, Bone marrow transplantation for severe combined immunodeficiency with the HLA-A incompatible but MLC identical mother as a donor, Transplant. Proc. 8: 623.
Oppenheim, J. J., Blaese, R. M., and Waldmann, T. A., 1970, Defective lymphocyte transformation and delayed hypersensitivity in the Wiskott-Aldrich syndrome, J. Immunol. 104: 835.
O’Reilly, R. J., Dupont, B., Pahwa, S., Grimes, E., Smithwick, E. M., Pahwa, R., Schwartz, S., Hansen, J. A., Siegal, F. P., Sorell, M., Svejgaard, A., Jersild, C., Thomsen, M., Platz, P., L’Esperance P., and Good, R. A., 1977, Reconstitution of severe combined immunodeficiency by transplantation of marrow from an unrelated donor, N. Engl. J. Med. 297: 1311.
O’Reilly, R. J., Pahwa, R., Dupont, B., and Good, R. A., 1978, Severe combined immunodeficiency: Transplantation approaches for patients lacking an HLA genotypically identical sibling, Transplant. Proc. 10(1): 187.
Osofsky, S. G., Thompson, B. H., Lint, T. F., and Gewurz, H., 1977, Hereditary deficiency of the third component of complement in a child with fever, skin rash and arthralgias in response to whole blood transfusion, J. Pediatr. 90: 180.
Page, A. R., Hansen, A. E., and Good, R. A., 1963, Occurrence of leukemia and lymphoma in patients with agammaglobulinemia, Blood 21: 197.
Pahwa, R., and Good, R. A., 1980, Immunologic reconstitution, in: Infections in the Abnormal Host ( M. H. Grieco, ed.), p. 951, Yorke Medical Books, New York.
Pahwa, R., Pahwa, S., Good, R. A., Incefy, G. S., and O’Reilly, R. J., 1977, Rationale for combined use of fetal liver and thymus for immunological reconstitution in patients with variants of severe combined immunodeficiency, Proc. Natl. Acad. Sci. USA 74: 3002.
Pahwa, R., Pahwa, S., O’Reilly, R. J., and Good, R. A., 1978, Treatment of immunodeficiency diseases—progress towards replacement therapy emphasizing cellular and macromolecular engineering, Springer Semin. Immunopathol. 1: 355.
Pahwa, R., Ikehara, S., Pahwa, S., and Good, R. A., 1979a, Thymic function in man, Thymus 1: 27.
Pahwa, R. N., Pahwa, S. G., and Good, R. A., 1979b, T-lymphocyte differentiation in vitro in severe combined immunodeficiency: defects of stem cells. J. Clin. Invest. 64: 1632.
Pahwa, S., and Good, R. A., 1980, In vitro B lymphocyte responses in patients with selective IgA deficiency, Pediatr. Res. 14:550 (abstr.)
Pahwa, B., Pahwa, R., Incefy, G., Reece, E., O’Reilly, R., Smithwick, E., and Good, R. A., 1978, Incomplete reconstitution following fetal thymus transplantation in DiGeorge syndrome, Clin. Res. 26: 383A.
Pahwa, S., Pahwa, R., Kapoor, N., and Good, R. A., 1979, Heterogeneity of B-lymphocyte differentiation in severe combined immunodeficiency disease (SCID), Pediatr. Res. 13: 453 (abstr.)
Parkman, R., Rappaport, J., Geha, R., Belli, J., Cassady, R., Levey, R., Nathan, D. G., and Rosen, F. S., 1978, Correction of the Wiskott-Aldrich syndrome by bone marrow transplantation, N. Engl. J. Med. 248: 921.
Pelkonen, R., Siurala, M., and Vuopio, P., 1963, Inherited agammaglobulinemia with malabsorption and marked alterations in the gastrointestinal mucosa, Acta Med. Scand. 173: 549.
Peterson, B. H., Graham, J. A., and Boroks, G. F., 1976, Human deficiency of the eighth component of complement: The requirement of C8 for serum Neisseria gonorrhoeae bactericidal activity, J. Clin. Invest. 57: 283.
Peterson, R. D. A., Kelly, W. D., and Good, R. A., 1964, Ataxia-telangiectasia: Its association with defective thymus, immunological deficiency disease and malignancy, Lancet 1: 1189.
Peterson, R. D. A., Cooper, M. D., and Good, R. A., 1965, Lymphoid tissue abnormalities associated with ataxia telangiectasia, Am. J. Med. 41: 342.
Pickering, R. J., Kelly, J. R., Good, R. A., and Gewurz, H., 1969, Replacement therapy in hereditary angioedema: Successful treatment of two patients with fresh frozen plasma, Lancet 1: 326.
Pickering, R. J., Naff, G. B., Stroud, R. M., Good, R. A., and Gewurz, H., 1970, Deficiency of C1r in human serum: Effects on the structure and function of macromolecular C 1, J. Exp. Med. 141: 803.
Pickering, R. J., Pollara„B., and Meuwissen, H. J., 1974, Meeting report. Workshop on severe combined immunological deficiency disease and adenosine deaminase deficiency, Clin. Immunol. Immunopathol. 3: 301.
Pitts, J. S., Donaldson, V. M., Forristal, J., and Wyatt, R. J., 1978, Remissions induced in hereditary angioneurotic edema with an attenuated androgen (Danazol). Correlation between concentrations of Cl inhibitor and the fourth and second components of complement, J. Lab. Clin. Med. 92: 501.
Polmar, S. H., Wetzler, E. M., Stern, R. C., and Hirschhorn, R., 1975, Restoration of in vitro lymphocyte responses with exogenous adenosine deaminase in a patient with severe combined immunodeficiency, Lancet 2: 743.
Prasad, A. S., and Koza, D. W., 1954, Agammaglobulinemia, Ann. Intern. Med. 41: 629.
Preud’homme, J. L., Griscelli, C., and Seligmann, M., 1973, Immunoglobulins on the surface of lymphocytes in fifty patients with primary immunodeficiency disease, Clin. Immunol. Immunopathol. 1: 241.
Rams0e, K., Skinhe P., Andersen, V., Ernst, P., Faber, V., Platz, P., Thomsen, M., Svejgaard, A., Eriksen, K. R., Plesner, T., Morling, N., Philip, J., Killmann, S. A., Koch, C., Müller-Berat, N., Henningsen, K., and Axelsen, N. H., 1978, Successful nonsibling bone marrow transplantation in severe combined immunodeficiency, Transplantation 26: 369.
Reed, W. B., Epstein, W. L., Boder, E., and Sedgwick, R., 1966, Cutaneous manifestations of ataxia-telangiectasia, J. Am. Med. Assoc. 195: 746.
Reisner, Y., Itzicovitch, L., Meshorer, A., and Sharon, N., 1978, Hematopoietic stem cell transplant using mouse bone marrow and spleen cells fractionated by lectins, Proc. Natl. Acad. Sci. USA 75: 2933.
Rieger, C. H. L., Lustig, J. V., Hirschhorn, R., and Rothberg, R., 1977, Reconstitution of T cell function in severe combined immunodeficiency disease following transplantation of early embryonic liver cells, J. Pediatr. 90: 707.
Rittner, C.H., Hauptmann, G., Grosse-Wilde, H., Grosshans, E., Tongio, M. M., and Mayr, S., 1975, Linkage between HL-A (major histocompatibility complex) and genes controlling the synthesis of the fourth component of complement, in: Histocompatibility Testing 1975, p. 945, Munksgaard, Copenhagen.
Root, R. K., Rosenthal, A. S., and Balestra, D. J., 1972, Abnormal bactericidal, metabolic and lysosomal functions of Chediak-Higashi syndrome leukocytes, J. Clin. Invest. 51: 649.
Rosen, F. S., and Janeway, C. A., 1966, The gammaglobulins. III. The antibody deficiency syndromes, N. Engl. J. Med. 275: 709.
Rosen, F. S., Charache, P., Pensky, J., and Donaldson, V., 1965, Hereditary angioneurotic edema: Two genetic variants, Science 148: 957.
Rötstein, J., and Good, R. A., 1962, Significance of the simultaneous occurrence of connective tissue disease and agammaglobulinemia, Ann. Rheum. Dis. 21: 202.
Rubinstein, A., Hirschhorn, R., Sicklick, M., and Murphy, R. A., 1979, In vivo and in vitro effects of thymosin and adenosine deaminase on adenosine-deaminasedeficient lymphocytes, N. Engl. J. Med. 300: 387.
Safai, B., Gupta, S., and Good, R. A., 1978, Pemphigus vulgaris associated with a syndrome of immunodeficiency and thymoma: A case report, Clin. Exp. Dermatol. 3: 129.
Sanford, J. P., Favour, C. B., and Tribeman, M. S., 1954, Absence of serum gamma globulins in an adult, N. Engl. J. Med. 250: 1027.
Schwartz, S. A., 1980, Heavy chain-specific suppression of immunoglobulin synthesis and secretion by lymphocytes from patients with selective IgA deficiency, J. Immunol. 124: 2034.
Schwartz, S. A., Choi, Y. S., Shou, L., and Good, R. A., 1977, Modulatory effects on immunoglobulin synthesis and secretion by lymphocytes from immunodeficient patients, J. Clin. Invest. 59: 1176.
Segal, A. W., and Peters, T. J., 1976, Characterization of the enzyme defect in chronic granulomatous disease, Lancet 1: 1364.
Sharpe, M. R., and Peterson, R. D. A., 1978, Improvement of cellular immunodeficiency with thymosin, Clin. Res. 26: 818A (abstr.).
Shoeck, V. V., Peterson, R. D. A., and Good, R. A., 1963, Familial occurence of Leterer-Siwe disease, Pediatrics 32 (6): 1055.
Siegal, F. P., Pernis, B., and Kunkel, H. G., 1971, Lymphocytes in human immunodeficiency states: A study of membrane-associated immunoglobulins, Eur. J. Immunol. 1: 482.
Siegal, F. P., Wernet, P., Dickler, H. B., Fu, S. M., and Kunkel, H. G., 1975, B lymphocytes lacking surface Ig in patients with immune deficiency: Initiation of Ig synthesis in culture in cells of a patient with thymoma, Birth Defects 11 (1): 40.
Siegal, F. P., Siegal, M., and Good, R. A., 1976, Suppression of B cell differentiation by leukocytes from hypogammaglobulinemic patients, J. Clin. Invest. 58: 109.
Siegal, F. P., Siegal, M., and Good, R. A., 1978, Role of helper, suppressor and B-cell defects in the pathogenesis of the hypogammaglobulinemias, N. Engl. J. Med. 299: 172.
Sineby, B., 1966, Ataxia-telangiectasia, Acta Paediatr, Scand. 55: 239.
Slavin, S., Strober, S., Fuks, Z., and Kaplan, H. S., 1977, Induction of specific tissue transplantation tolerance using fractionated total lymphoid irradiation in adult mice: Long-term survival of allogeneic bone marrow and skin grafts, J. Exp. Med. 146: 34.
Solitare, G. B., 1968, Louis-Bar’s syndrome (ataxia-telangiectasia), Neurology 18: 1180.
Soothill, J. F., 1975, Interactions in immunodeficiency, Birth Defects 11 (1): 50.
Sorell, M., Kirkpatrick, D., Kapoor, N., Kapadia, A., Pahwa, R., Chaganti, R. S. K., Dupont, B., Good, R. A., and O’Reilly, R. J., 1979a, Bone marrow transplant for combined immune deficiency (CID) and agranulocytosis associated with cartilage-hair hypoplasia (CHH), Pediatr. Res. 13: 455 (abstr.).
Sorell, M., Rosen, J. F., Kapoor, N., Kirkpatrick, D. Chaganti, R. S. K., Pollack, M. S., Dupont, B., Goossen, C., Good, R. A. and O’Reilly, R. J., 1979b, Bone marrow transplant for osteopetrosis in a 10 year old boy, Pediatr. Res. 13: 481 (abstr.)
Sorell, M., Kapoor, N., Pahwa, R., Halac, E., Cooper, D., Kirkpatrick, D., Changanti, R. S. K., Dupont, B., Good, R. A. and O’Reilly, R. J., 1980a, Correction of combined immunodeficiency and agranulocytosis in a patient with cartilage hair hypoplasia by marrow transplantation, (submitted).
Sorell, M., Kapoor, N., Kirkpatrick, D., Rosen, J. F., Chaganti, R. S. K., Lopez, C., Dupont, B., Pollack, M. S., Terrin, B. N., Harris, M. B., Vine, D., Rose, J. S., Goossen, C., Lane, J., Good, R. A., and O’Reilly, R. J., Marrow transplantation for juvenile osteopetrosis, Am. J. Med. (in press).
Steele, R. W., Limas, C., Thurman, G. B., Schuelein, M., Bauer, H., and Bellanti, J. A., 1976, Familial thymic aplasia. Attempted reconstitution with fetal thymus in a millipore diffusion chamber, N. Engl. J. Med. 287: 787.
Stern, R., Fu, S. M., Fotino, M., Agnello, V., and Kunkel, H. G., 1976, Hereditary C2 deficiency: Association with skin lesions resembling the discoid lesion of SLE, Arthritis Rheum. 19: 45.
Stiehm, E. R., 1975, Plasma therapy: An alternative to gammaglobulin injections in immunodeficiency, Birth Defects 11 (1): 343.
Stillman, M. A., and Baer, R. L., 1973, Mucocutaneous candidiasis and thymoma, J. Am. Med. Assoc. 224 (5)
Sussman, M., Jones, J. S., and Almeida, J. D., 1973, Deficiency of the second component of complement associated with anaphylactoid purpura and presence of mycoplasma in the serum, Clin. Exp. Immunol. 14: 531.
Touraine, J. L., Touraine, F., Dutruge, J., Gilly, J., Colen, S., and Gilly, R., 1975, 1. T-lymphocyte precursors and T-lymphocyte differentiation in partial Di-George syndrome, Clin. Exp. Immunol. 21: 39.
Trotta, P. P., Smithwick, E. M., and Balis, M. E., 1976a, A normal level of adenosine deaminase activity in the red cell lysates of carriers of patients with severe combined immunodeficiency disease, Proc. Natl. Acad. Sci. USA 73: 104.
Trotta, P. P., Smithwick, E. M., Good, R. A., and Balis, M. E., 1976b, Characterization of adenosine deaminase from red cell lysates of carriers and patients with severe combined immunodeficiency, Fed. Proc. 35: 1733.
Tulunay, O., Good, R. A., and Yunis, E. J., 1975, Protection of lethally irradiated mice with allogeneic fetal liver cells. Influence of irradiation dose on immunologic reconstitution, Proc. Natl. Acad. Sci. USA 72: 4100.
Twomey, J. J., Jordan, P. H., Jarrold, T., Trubowitz, S., Ritz, N. D., and Conn, H. O., 1969, The syndrome of immunoglobulin deficiency and pernicious anemia, Am. J. Med. 47: 340.
Twomey, J. J., Jordan, P. H., Jr., Laughter, A. H., Meuwissen, H. J., and Good, R. A., 1970, The gastric disorder in immunoglobulin-deficient patients, Ann. Intern. Med. 72: 499.
Valdimarsson, H., Higgs, J. M., Wells, R. S., Yamamura, M., Hobbs, J. R., and Holt, P. J. L., 1973, Immune abnormalities associated with chronic mucocutaneous candidiasis. Cell. Immunol. 6: 348.
Vesin, P., and Cattan, R., 1963, Antibody deficiencies and intestinal disorders, in: Plasma Proteins and Gastrointestinal Tract in Health and Disease: Proceedings of the International Symposium, Paris, August 1961 ( M. Schartz, and P. Vesin, eds.), p. 214, Williams and Wilkins, Baltimore.
Vogler, L. B., Pearl, E. R., Gathings, W. E., Lawton, A. R., and Cooper, M. D., 1976, B lymphocyte precursors in bone marrow in immunoglobulin deficiency diseases, Lancet 2:376 (Letters to the Editor).
Vossen, J. M., de Koning, J., Van Bekkum, D. W., Dicke, K. A., Eijsvoogel, P. P., Hijams, W., Van Logen, E., Radl, J., Van Rood, J. J., Van Der Waay, D., and Dooren, L. J., 1973, Successful treatment of an infant with severe combined immunodeficiency by transplantation of bone marrow cells from an uncle, Clin. Exp. Immunol. 13: 30.
Waldmann, T. A., and Laster, L., 1964, Abnormalities of albumin metabolism in patients with hypogammaglobulinemia, J. Clin. Invest. 43: 1025.
Waldmann, T. A., and McIntire, K. B., 1972, Serum alpha-fetoprotein levels in patients with ataxia-telangiectasia, Lancet 2: 1112.
Waldmann, T. A., Broder, S., Blaese, R. M., Durm, M., Blackman, M., and Strober, W., 1974, Role of suppressor T cells in pathogenesis of common variable hypogammaglobulinemia, Lancet 2: 609.
Waldmann, T. A., Broder, S., Krakauer, R., MacDermott, R. P., Durm, M., Goldman, C., and Meade, B., 1976, The role of suppressor cells in the pathogenesis of common variable hypogammaglobulinemia and the immunodeficiency associated with myeloma, Fed. Proc. 35: 2067.
Wells, R. S., Higgs, J. M., MacDonald, A., Valdimarsson, H., and Holt, P. J. L., 1972, Familial chronic mucocutaneous candidiasis, J. Med. Genet. 9: 302.
West, C. D., Hong, R., and Holland, N. H., 1962, Immunoglobulin levels from the newborn period to adulthood and in immunoglobulin deficiency states, J. Clin. Invest. 41: 2054.
Windhorst, D. B., Page, A. R., Holmes, B., Quie, P. G., and Good, R. A., 1968, The pattern of genetic transmission of the leukocyte defect in fatal granulomatous disease of childhood, J. Clin. Invest. 47: 1026.
Wolff, S. M. Dale, D. C., Clark, R. A., Root, R. K., and Kimball, H. R., 1972, The Chediak-Higashi syndrome: Studies of host defense, Ann. Intern. Med. 76: 293.
Wollheim, F. A., 1968, Primary “acquired” hypogammaglobulinemia: Genetic defect or acquired disease? Birth Defects 4 (1): 311.
Wu, L. Y., F., Lawton, A. R., and Cooper, M. D., 1973a, Differentiation of capacity of cultured B lymphocytes from immunodeficient patients, J. Clin. Invest. 52: 3180.
Wu, L. Y. F., Lawton, A. R., Greaves, M. F., and Cooper, M. D., 1973b, Evaluation of human B lymphocyte differentiation using pokeweed mitogen (PWM) stimulation: In vitro studies in various antibody syndromes, in: Proceedings of the Seventh Leucocyte Culture Conference ( F. Daguillard, ed.), p. 485, Academic Press, New York.
Yount, J., Nichols, P., Ochs, H. D., Hammar, S., Scott, C. R., Chen, S., Giblett, E. R., and Wedgwood, R. J., 1974, Absence of erythrocyte adenosine deaminase associated with severe combined immunodeficiency, J. Pediatr. 84: 173.
Zinneman, H. H., Hall, W. H., and Heller, B. I., 1954, Acquired agammaglobulinemia: Report of three cases, J. Am. Med. Assoc. 156: 1390.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1981 Plenum Publishing Corporation
About this chapter
Cite this chapter
Good, R.A., Pahwa, R.N., West, A. (1981). Primary Immunodeficiency Diseases of Man. In: Safai, B., Good, R.A. (eds) Immunodermatology. Comprehensive Immunology, vol 7. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-7228-2_28
Download citation
DOI: https://doi.org/10.1007/978-1-4615-7228-2_28
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4615-7230-5
Online ISBN: 978-1-4615-7228-2
eBook Packages: Springer Book Archive