Document 0874
 DOCN  M9460874
 TI    Bone marrow transplantation (BMT) in Europe for primary
       immunodeficiencies other than severe combined immunodeficiency: a report
       from the European Group for BMT and the European Group for
       Immunodeficiency.
 DT    9404
 AU    Fischer A; Landais P; Friedrich W; Gerritsen B; Fasth A; Porta F;
       Vellodi A; Benkerrou M; Jais JP; Cavazzana-Calvo M; et al; Departement
       de Pediatrie et INSERM U 132, Hopital des; Enfants-Malades, Paris,
       France.
 SO    Blood. 1994 Feb 15;83(4):1149-54. Unique Identifier : AIDSLINE
       MED/94154279
 AB    Bone marrow (BM) transplantations performed between 1977 and 1991 at 13
       European centers in 149 patients with 11 different primary
       immunodeficiency (ID) diseases (excluding severe combined
       immunodeficiency) were analyzed retrospectively. Overall survival among
       recipients of HLA genetically identical BM (n = 56) was 66%. Since
       October 1985, the date of a previous survey, a significant improvement
       in survival has been achieved in most ID diseases (overall survival,
       81.5% v 51.7%; P < .01), primarily because of a decrease in the
       frequency of infectious complications. In long-term survivors, disease
       correction is excellent, with minimal sequelae in most patients. In 22
       patients who received closely matched BM (ie, from phenotypically
       identical related donors, matched unrelated donors, or one
       HLA-ag-mismatched related donors), the survival rate (45.5%) was not
       significantly better than among 71 recipients of BM with 2 or 3
       mismatched HLA antigens (38%). In the latter group, favorable outcome
       was associated with younger age, with transplantation since October 1985
       (47% v 25%; P < .0001), and with a diagnosis of leukocyte adhesion
       deficiency. The improvement in outcome was mainly because of a higher
       engraftment rate and a decrease in the frequency of infections, although
       Epstein-Barr virus-induced B-lymphocyte proliferative disorders occurred
       in 16 patients (mainly those with Wiskott-Aldrich syndrome), 10 of whom
       died. The improvement in engraftment corresponded to the introduction of
       treatment in vivo with anti-LFA-1 antibody to prevent rejection of
       T-cell-depleted grafts (74% engraftment and 45% survival in 38 treated
       patients versus 37.5% and 21%, respectively, in 24 untreated patients.
 DE    Adult  Aged  *Bone Marrow Transplantation/IMMUNOLOGY  Cause of Death
       Comparative Study  Europe  Follow-Up Studies  Histocompatibility Testing
       Human  Immunologic Deficiency Syndromes/IMMUNOLOGY/MORTALITY/*THERAPY
       Major Histocompatibility Complex  *Severe Combined Immunodeficiency
       Support, Non-U.S. Gov't  Survival Analysis  JOURNAL ARTICLE  MULTICENTER
       STUDY

       SOURCE: National Library of Medicine.  NOTICE: This material may be
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