European experience of bone-marrow transplantation for severe combined immunodeficiency

Lancet. 1990 Oct 6;336(8719):850-4. doi: 10.1016/0140-6736(90)92348-l.

Abstract

The outcome of bone-marrow transplantations (BMT) carried out between 1968 and March 1, 1989, in 183 patients with severe combined immunodeficiency (SCID) was analysed. Recipients of HLA-identical BMTs (70) had a 76% probability of survival (median follow-up 73 months). Of the 32 treated since 1983, 97% have been cured (median follow-up 41 months). This good prognosis was associated with rapid development of T and B cell function. HLA-non-identical, T-cell-depleted, BMT (n = 100) gave significantly lower survival (52%; median follow-up 47 months). Factors associated with poor prognosis were the presence of a lung infection before BMT, absence of a protected environment, and use of female donors for male recipients. Use of a conditioning regimen significantly increased the frequency of sustained engraftment (86% vs 50% for non-conditioned BMT) and resulted in more frequent engraftment of donor B lymphocytes and myeloid cells. Donor B-cell chimerism was strongly associated with the development of normal B-cell function.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Actuarial Analysis
  • B-Lymphocytes / immunology
  • Bone Marrow Transplantation / immunology
  • Bone Marrow Transplantation / mortality*
  • Europe
  • Female
  • Follow-Up Studies
  • Graft Survival / drug effects
  • Graft vs Host Disease / etiology
  • Graft vs Host Disease / immunology
  • Histocompatibility / immunology
  • Humans
  • Immunologic Deficiency Syndromes / classification
  • Immunologic Deficiency Syndromes / immunology
  • Immunologic Deficiency Syndromes / mortality
  • Immunologic Deficiency Syndromes / surgery*
  • Infant
  • Karyotyping
  • Male
  • Premedication
  • Prognosis
  • Retrospective Studies
  • Sex Factors
  • T-Lymphocytes / immunology
  • Time Factors