Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

73 consecutive patients with severe aplastic anemia were treated by marrow transplantation from hematologically normal HLA identical siblings. 68 patients lived long enough to document marrow engraftment. 21 rejected the graft and 19 of these died. 47 sustained engraftment and 18 of these died. In 16 patients, death was associated with graft versus host disease. 29 patients with sustained engraftment are alive with complete hematologic restoration between 8 mo and 5 yr. This analysis, by using a proportional hazards regression model, was directed at identifying factors that predicted survival (and absence of graft versus host disease). Of the 24 factors entered into the analysis only two strongly correlated with survival: (a) sex match of donor and recipient (P less than 0.01), and (b) absence of refractoriness to random donor platelets at the time of transplantation (P less than 0.05). Refractoriness adversely influenced the survival of the sex mismatched patients, These data suggest that X and Y-associated transplantation antigen systems are important determinants of the outcome of marrow grafts between HLA identical siblings for the treatment of aplastic anemia. The machanism by which refractoriness to random donor platelets influences survival is currently unclear.
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PMID:Treatment of aplastic anemia by marrow transplantation from HLA identical siblings. Prognostic factors associated with graft versus host disease and survival. 1 72

Using the cell-mediated lympholysis (CML) technique, we studied lymphocytes of six individuals with discrepancies between the karyotypic and phenotypic sex. Two sets of cytotoxic T cells (CTLs) obtained from two multitransfused female aplastic anemia patients were used as typing reagents. These cells were previously shown to kill allogeneic target cells from HLA-A2- or B7-positive male donors. An antiserum obtained from one of the patients likewise killed HLA-A2 male lymphocytes. The six patients studied were selected for the required antigens. Positive reactions were obtained with lymphocytes from a 46,XY woman with pure gonadal dysgenesis and a 45,XO male. Target cells of the mother of the latter patient were also lysed. One individual with a 45,XO/46,X,del(Y)? karyotype was weakly positive, while three 46,XX males were completely negative. The reactivity of the HLA-A2-restricted H-Y-specific antibody showed the same discriminatory patterns. The results obtained by the HLA-restricted CTLs as well as by the antiserum did not correlate with the presence of testes as is the case in a different test system for the serologically detectable male (SDM) antigen in man. On the other hand, there was a correlation with the presence of cytologically detectable Y-chromosome material in five of the six individuals studied. The HLA-restricted CTLs and the antibody might recognize the classical transplantation antigen H-Y.
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PMID:The recognition of abnormal sex chromosome constitution by HLA-restricted anti-H-Y cytotoxic T cells and antibody. 660 15