Bone Marrow Transplantation in the Treatment of Congenital T Cell Disorders

  • W. Friedrich
  • C. Knobloch
  • W. Hartmann
Conference paper


Bone marrow transplantation (BMT) plays a central role in the management of congenital immunodeficiencies characterized by profound abnormalities of the T cell system [10, 14, 19]. This heterogeneous group of disorders may be corrected by the establishment of a new, donor-dependent immmune system. BMT in these disorders also represents a unique model to study and to define cellular interactions which are operative in the functional reconstitution of the lymphohemopoietic system in man.


Bone Marrow Transplantation Severe Combine Immunodeficiency Primary Immunodeficiency Disease Adenosine Deaminase Deficiency Congenital Immunodeficiency 
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  1. 1.
    Bortin MM, Rimm RA (1977) Severe combined immunodeficiency: characterization of the disease and results of bone marrow transplantation. JAMA 238: 591–608PubMedCrossRefGoogle Scholar
  2. 2.
    Buckley H, Schiff SE, SampsonHA, Schiff RI et al. (1986) Development of immunity in human severe primary T cell deficiencies following haploidentical bone marrow transplantation. J Immunol 136: 2398–2407PubMedGoogle Scholar
  3. 3.
    Fischer A, Friedrich W, Levinsky R,Vossen J et al. (1986) Bone marrow transplantation for immunodeficiencies and osteopetrosis: European survey 1968–1985. Lancet II: 1080–1083CrossRefGoogle Scholar
  4. 4.
    Fischer A, Landais P, Friedrich W, Morgan G et al. (1990) European experience of bone-marrow transplantation for severe combined immunodeficiency. Lancet II: 850–854CrossRefGoogle Scholar
  5. 5.
    Fischer A, Friedrich W, Fasth A, Blanche S et al. (1991) Reduction of graft failure by a monoclonal antibody (anti-LFA-1 CD11a) after HLA nonidentical bone marrow transplantation in children with immunodeficiencies, osteopetrosis and Fanconi’s anemia: a European Group for Immunodeficiency/European Group for Bone Marrow Transplantation Report. Blood 77: 249–256PubMedGoogle Scholar
  6. 6.
    Friedrich W, Peter HH, Blütters-Sawatzki R, Ebell W (1986) Analysis of the immunoreconstitution in 19 patients with severe combined immunodeficiency after HLA haploidentical bone-marrow transplantation. In: Vossen J, Griscelli C (eds) Progress in immunodeficiency research and therapy II. Experta Medica, Amsterdam, pp 351–359Google Scholar
  7. 7.
    Friedrich W, Goldmann SF, Vetter U, Fliedner TM et al. (1984) Immunological reconstitution in severe combined immunodeficiency after transplantation of HLA haploidentical, T cell depleted bone-marrow. Lancet I: 761–764CrossRefGoogle Scholar
  8. 8.
    Friedrich W, Goldmann SF, Ebell W, Blütters-Sawatzki R et al. (1986) Severe combined immunodeficiency: treatment by bone-marrow transplantation in 15 infants using HLA haploidentical donors. Eur J Pediatr 144: 125–130CrossRefGoogle Scholar
  9. 9.
    Gatti RA, Meeuwissen HJ, Allen HD, Hong R, Good R (1968) Immunologic reconstituion of sex linked lymphopenic immunodeficiency. Lancet II: 1366–1369CrossRefGoogle Scholar
  10. 10.
    Good RA, Kapoor N, Pahwa RN Wert A, O’Reilly RJ (1980) Current approaches to the primary immunodeficiencies In: Fougereau M, Dausset J (eds) Progress in immunology IV. Academic, LondonGoogle Scholar
  11. 11.
    Griscelli C, Durandy A, Virelizier JL,Ballet JJ et al. (1978) Selective defect of precursor T cells associated with apparently normal B lymphocytes in severe combined immunodeficiency. J Pediatr 93: 404–411PubMedCrossRefGoogle Scholar
  12. 12.
    Kapoor N, Kirkpatrick D, Blaese RM, Oleske J et al. (1981) Reconstitution of normal megakaryocytopoiesis and immune functions in Wiskott-Aldrich syndrome by marrow transplantation following myeloablation and immunosuppression with busulfan and cyclophosphamide. Blood 57: 692–699PubMedGoogle Scholar
  13. 13.
    Kenny AB, Hitzig WA (1979) Bone marrow transplantation for severe combined immunodeficiency. Eur J Pediatr 131: 155–177PubMedCrossRefGoogle Scholar
  14. 14.
    O’Reilly RJ, Brochstein J, Dinsmore B, Kirkpatrick (1984) Marrow transplantation for congenital disorders. Semin Hematol 21: 188–213PubMedGoogle Scholar
  15. 15.
    O’Reilly J, Keever CA, Small TN, Brochstein J (1990) The use of HLA-nonidentical T-cell depleted marrow transplants for correction of severe combined immunodeficiency disease. Immunodefic Rev 1: 273–309Google Scholar
  16. 16.
    Parkman R, Rappaport D, Geha R, Cassady R et al. (1978) Complete correction of Wiskott-Aldrich syndrome by allogeneic marrow transplantation. N Engl J Med 298: 921–927PubMedCrossRefGoogle Scholar
  17. 17.
    Pollak MS, Kirkpatrick D, Kapoor N, O’Reilly (1982) Identification by HLA typing of intrauterine-derived maternal T cells four patients with severe combined immunodeficiency. N Engl J Med 307: 662–666CrossRefGoogle Scholar
  18. 18.
    Reisner Y, Kapoor N, Kirpatrick D, Cunningham-Rundels S et al. (1983) Transplantation for severe combined immunodeficiency with HLA A, B, C, DR incompatible paternal marrow fractionated by soybean agglutination and sheep red blood cells. Blood 61: 341–348PubMedGoogle Scholar
  19. 19.
    Rosen FS, Cooper MD, Wegewood RJP (1984) The primary immunodeficiencies. N Engl J Med 311: 235–242; 300–310PubMedCrossRefGoogle Scholar
  20. 20.
    Rumelhart SL, Trigg ME Horowitz SD, Hong R (1990) Monoclonal antibody T-cell-depleted HLA-haploidentical bone marrow transplantation for Wiskott-Aldrich syndrome. Blood 75: 1031–1035PubMedGoogle Scholar
  21. 21.
    World Health Organization (1983) Primary immunodeficiency disease (Report prepared for the WHO by a scientific group on immunodeficiencies). Clin Immunol Immunpathol 28: 447–450Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1993

Authors and Affiliations

  • W. Friedrich
  • C. Knobloch
  • W. Hartmann

There are no affiliations available

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