Skip to main content
PMC home page
Clinical and Experimental Immunology logoLink to Clinical and Experimental Immunology
. 1973 Jan;13(1):9–20.

Successful treatment of an infant with severe combined immunodeficiency by transplantation of bone marrow cells from an uncle

J M Vossen, J de Koning, D W van Bekkum, K A Dicke, V P Eysvoogel, W Hijmans, Erna van Loghem, J Rádl, J J van Rood, D van der Waay, L J Dooren
PMCID: PMC1553761  PMID: 4587502

Abstract

A 4½-month-old boy suffering from congenital severe combined immunodeficiency was successfully treated by transplantation of bone marrow-derived cells. His parents were cousins in the first degree. The donor was a 32-year-old maternal uncle, who was HL-A genotypically identical with the patient as was shown by serological typing and MLC. A stem cell rich fraction of the donor's bone marrow was prepared by albumin gradient centrifugation. The infant showed a full immunological reconstitution without any sign of GVH disease. Remarkably the only allotypic marker of his IgG which was different from the donor allotype remained in the serum after transplantation, and even showed an increase of its level.

The infant was nursed in strict reverse isolation and his unfavourable endogenous microflora of high potential pathogenicity was eliminated by antibiotic decontamination.

Full text

PDF
11

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adler F. L., Fishman M., Dray S. Antibody formation initiated in vitro. 3. Antibody formation and allotypic specificity directed by ribonucleic acid from peritoneal exudate cells. J Immunol. 1966 Oct;97(4):554–558. [PubMed] [Google Scholar]
  2. Bell C., Dray S. Conversion of non-immune rabbit spleen cells by ribonucleic acid of lymphoid cells from an immunized rabbit to produce IgM and IgG antibody of foreign heavy-chain allotype. J Immunol. 1971 Jul;107(1):83–95. [PubMed] [Google Scholar]
  3. Bensch J., Berg T., Hagberg B., Johansson S. G., Skoog W. Congenital immunological defect with early progressive brain disorder. Lancet. 1971 May 22;1(7708):1070–1070. doi: 10.1016/s0140-6736(71)91632-1. [DOI] [PubMed] [Google Scholar]
  4. Cleveland W. W., Fogel B. J., Brown W. T., Kay H. E. Foetal thymic transplant in a case of Digeorge's syndrome. Lancet. 1968 Dec 7;2(7580):1211–1214. doi: 10.1016/s0140-6736(68)91694-2. [DOI] [PubMed] [Google Scholar]
  5. Dayan A. D. Combined immunodeficiency and chronic encephalitis. Lancet. 1971 Jun 5;1(7710):1178–1179. doi: 10.1016/s0140-6736(71)91681-3. [DOI] [PubMed] [Google Scholar]
  6. De Koning J., Van Bekkum D. W., Dicke K. A., Dooren L. J., Rádl J., Van Rood J. J. Transplantation of bone-marrow cells and fetal thymus in an infant with lymphopenic immunological deficiency. Lancet. 1969 Jun 21;1(7608):1223–1227. doi: 10.1016/s0140-6736(69)92112-6. [DOI] [PubMed] [Google Scholar]
  7. Dicke K. A., Lina P. H., van Bekkum D. W. Adaptation of albumin density gradient centrifugation to human bone marrow fractionation. Rev Eur Etud Clin Biol. 1970 Mar;15(3):305–309. [PubMed] [Google Scholar]
  8. GURNER B. W., COOMBS R. R. Examination of human leucocytes for the ABO, MN, Rh, Tja, Lutheran and Lewis systems of antigens by means of mixed erythrocyte-leucocyte agglutination. Vox Sang. 1958 Jan;3(1):13–22. doi: 10.1111/j.1423-0410.1958.tb03554.x. [DOI] [PubMed] [Google Scholar]
  9. Harboe M., Pande H., Brandtzaeg P., Tveter K. J., Hjort P. F. Synthesis of donor type gamma-G-globulin following thymus transplantation in hypo-gamma-globulinaemia with severe lymphocytopenia. Scand J Haematol. 1966;3(5):351–374. doi: 10.1111/j.1600-0609.1966.tb02379.x. [DOI] [PubMed] [Google Scholar]
  10. Hitzig W. H., Landolt R., Müller G., Bodmer P. Heterogeneity of phenotypic expression in a family with Swiss-type agammaglobulinemia: observations on the acquisition of agammaglobulinemia. J Pediatr. 1971 Jun;78(6):968–980. doi: 10.1016/s0022-3476(71)80426-2. [DOI] [PubMed] [Google Scholar]
  11. Levey R. H., Gelfand E. W., Klemperer M. R., Sanderson A. R., Bachelor J. R., Berkel A. I., Rosen F. S. Bone-marrow transplantation in severe combined immunodeficiency syndrome. Lancet. 1971 Sep 11;2(7724):571–575. doi: 10.1016/s0140-6736(71)92151-9. [DOI] [PubMed] [Google Scholar]
  12. Richter M., Abdou N. I. Cells involved in the immune response. VII. The demonstration, using allotypic markers, of antibody formation by irradiation-resistant cells of irradiated rabbits injected with normal allogeneic bone marrow cells and sheep erythrocytes. J Exp Med. 1969 Jun 1;129(6):1261–1273. doi: 10.1084/jem.129.6.1261. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Rubinstein A., Speck B., Jeannet M. Successful bone-marrow transplantation in a lymphopenic immunologic deficiency syndrome. N Engl J Med. 1971 Dec 16;285(25):1399–1402. doi: 10.1056/NEJM197112162852503. [DOI] [PubMed] [Google Scholar]
  14. Rádl J., Dooren L. J., Eijsvoogel V. P., van Went J. J., Hijmans W. An immunological study during post-transplantation follow-up of a case of severe combined immunodeficiency. Clin Exp Immunol. 1972 Feb;10(2):367–382. [PMC free article] [PubMed] [Google Scholar]
  15. Vossen J. M., van der Waay D. Reverse isolation in bone marrow transplantation: ultra-clean room compared with laminar flow technique. I. Isolation systems. Rev Eur Etud Clin Biol. 1972 May;17(5):457–461. [PubMed] [Google Scholar]
  16. Walls R. S., Basten A., Leuchars E., Davies A. J. Mechanisms for eosinophilic and neutrophilic leucocytoses. Br Med J. 1971 Jul 17;3(5767):157–159. doi: 10.1136/bmj.3.5767.157. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. de Koning J., van der Waay D., Vossen J. M., Versprille A., Dooren L. J. Barrier nursing of an infant in a laminar cross-flow bench. Maandschr Kindergeneeskd. 1970 Apr;38(1):1–13. [PubMed] [Google Scholar]
  18. van Bekkum D. W. Use and abuse of hemopoietic cell grafts in immune deficiency diseases. Transplant Rev. 1972;9:3–53. doi: 10.1111/j.1600-065x.1972.tb01560.x. [DOI] [PubMed] [Google Scholar]
  19. van Boven C. P., Verhoef J., Winkler K. C. Bacteriophage typing of coagulase-negative staphylococci. Antonie Van Leeuwenhoek. 1969;35(2):232–232. doi: 10.1007/BF02219134. [DOI] [PubMed] [Google Scholar]

Articles from Clinical and Experimental Immunology are provided here courtesy of British Society for Immunology

RESOURCES