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)

"Bare lymphocytes," which cannot be typed for the HLA-A,B, and C antigens were observed in two siblings, nine and six years of age. The elder child presented with aplastic anemia and was being considered for bone marrow transplantation. The younger child was healthy. The inability to phenotype both children for these three gene products persisted throughout the 21-month period of observation. The DR antigens were demonstrable which rendered it possible to deduce their A, B, and C genotype from the typing of the other four family members. Although alloantisera failed to detect the antigens on peripheral blood lymphocytes, monoclonal antibodies demonstrated reduced amounts of the HLA-A,B, and C antigens on the cells. The reduced level was confirmed following EBV transformation of the cells. After prolonged culture, HLA antigens immunoprecipitated from the cell extracts were normal in amount, molecular weight, and polypeptide composition. Southern blot analysis did not reveal gross genomic rearrangements. A regulatory defect leading to the expression of these Class I antigens is postulated.
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PMID:"Bare lymphocytes" without immunodeficiency. 657 6

A reassessment of HLA-A and B antigen associations in patients with severe aplastic anaemia confirmed the significantly elevated frequency of HLA-A2 in Caucasoid patients reported by Albert et al. (1976). In addition, a highly significant increase in the frequency of HLA-B14 in Italian patients was observed in an initial sample of 25 patients and confirmed in a second sample of 16 patients. This latter finding was cancelled out when the Italian Caucasoid patients were pooled with the non-Italian Caucasoid patients. These results suggest that the classification of populations as "Caucasoids", without examining their ethnic origins, may introduce a serious confounding error into HLA and disease studies.
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PMID:HLA associations in Italian and non-Italian Caucasoid aplastic anaemia patients. 657 13

A newborn with graft-vs-host (GVH) disease following an exchange transfusion was treated by attempting to eradicate the incompatible graft and to reconstitute the child hematologically and immunologically with a bone marrow transplant. The patient was a female term infant (blood group B, Rh+ Coombs test positive) who received a one-unit group O, Rh- exchange transfusion from an unrelated female donor for hyperbilirubinemia due to ABO incompatibility on day 2. Signs of acute GVH disease began on day 8 and the clinical diagnosis was supported by skin biopsy. With antithymocyte globulin and high dose dexamethasone, the GVH reaction improved somewhat. Cyclophosphamide, 200 mg/kg total dose, was given over four days followed by a marrow graft from a brother who was HLA-A, B identical, and probably also D locus compatible in mixed lymphocyte culture. All signs of GVH resolved with cyclophosphamide treatment and hematologic reconstitution was evident by 14 days after transplant. Two weeks later the GVH reaction and aplastic anemia recurred and Y chromatin was detected in only 6% of marrow cells. The infant died on day 80. Autopsy showed disseminated candidiasis, disseminated cytomegalovirus infection, thymic dysplasia, hypoplastic marrow, and other histopathologic changes consistent with GVH disease. The persistence of female cells in blood and bone marrow and the destruction of the reconstituted marrow suggest that the original incompatible transfusion-derived graft was not eliminated and that it ultimately rejected the histocompatible marrow graft.
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PMID:Probable graft-vs-graft reaction in an infant after exchange transfusion and marrow transplantation. 680 70

A cytotoxic T cell (CT) lines grown as a population (CT line) was initiated from the peripheral blood lympocytes (PBL) of a female aplastic anemia patient who was known to express CT that were able to lyse HLA-A2-positive male cells. The anti-H-Y HLA-A2-restricted cytotoxic activity could be maintained over prolonged periods of time. The CT lines could be expanded and maintained in culture for >65 d by the use of mitogens and irradiated feeder cells. Out of 68 cultures obtained after cloning of the CT lines, 43 showed varying, but always specific, anti-H-Y HLA-A2-restricted lytic capacity on a per-cell basis. We could show that the cloned cultures were composed of >80% T cells that carry the HLA-A, -B, -C, and also the HLA-DR antigens identical to the original PBL.
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PMID:Production, expansion, and clonal analysis of T cells with specific HLA-restricted male lysis. 696 35

Treatment of acquired aplastic anemia with androgens has been occasionally associated with the development of hepatic tumors. We have studied a 13-year-old boy with idiopathic aplastic anemia in whom oxymetholone treatment was associated with a partial hematological remission. Thirty-four months later, however, the patient developed multiple hepatic tumors. When oxymetholone therapy was discontinued, the aplastic anemia relapsed. He then underwent bone marrow transplantation from his HLA-A, B, and D-compatible sibling. This was followed by hematological and immunological reconstitution. The hepatic tumors underwent progressive regression after bone marrow transplantation. The patient is now 3 years post-bone marrow transplantation and is in complete remission of his aplastic anemia with no evidence of detectable liver tumors.
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PMID:Regression on oxymetholone-induced hepatic tumors after bone marrow transplantation in aplastic anemia. 701 Jul 13

Clinical histocompatibility testing has now developed to a stage where it is possible to select related bone marrow donors for some patients, when HLA genotypically identical siblings are not available. The most common type of such donors are the HLA phenotypically or HLA-D phenotypically identical related donors. The HLA-D homozygous recipient offers special options, since these patients can potentially receive bone marrow transplants from any of the parents or from HLA-haploidentical siblings. The studies in SCID have demonstrated that HLA-D compatibility in spite of HLA-A or B incompatibilities can be tolerated and there is now accumulating evidence that even patients with aplastic anemia or acute leukemia can be successfully treated with such bone marrow grafts.
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PMID:Histocompatibility testing for clinical bone marrow transplantation and prospects for identification of donors other than HLA genotypically identical siblings. 702 35

As of 31 December 1979, 39 patients in Seattle have received marrow grafts from donors other than HLA genotypically identical siblings. Sixteen transplants were between siblings, 21 from a parent to a child, one from a paternal uncle, and one from an unrelated donor. Ten patients had aplastic anemia and 29 had a hematological malignancy. As of 1 February 1980, only one of the ten patients transplanted for aplastic anemia is currently alive (greater than 1048 days) with a normal marrow and without graft-versus-host disease. This surviving patient was untransfused and received marrow from an HLA phenotypically identical mother. There were five episodes of graft rejection among the ten aplastic patients. Among the 29 patients transplanted for hematological malignancy, 12 (42%) are surviving from greater than 64 to greater than 995 days. Twelve of 29 patients were transplanted while in remission and eight (75%) are alive from greater than 148 to greater than 790 days. The two most frequent causes of death were relapse of leukemia and interstitial pneumonia. Only two patients died from complications clearly related to graft-versus-host disease. Five of the surviving patients were phenotypically identical with their donor for HLA, while seven were incompatible for some HLA determinants. One patient--donor pair was incompatible for HLA-D and DR as a result of HLA-B/D recombination, and six pairs were incompatible for HLA-A and/or B.
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PMID:Marrow transplantation from donors other than HLA identical siblings. 702 18

The substitution of thrombocytes is on principle indicated only when a disturbance of the formation is present. The prophylactic application, i.e. before the occurrence of a haemorrhage, which is sometimes life-limiting, is to be preferred, however, on account of the danger of sensitization it is bound to particular conditions: temporarily limited need of substitution, decreased immune reagibility of the recipient (basic disease, therapy), medico-therapeutically induced thrombocytopenia (cytostatic drugs); example: haemoblastoses. Prerequisites are subtile control of clinical effectiveness and formation of antibodies. In planned bone marrow transplantation only preparation of individual donors (not related!) with HLA-A/B match are to be used. The same is applied to already alloimmunized patients, in which case a negative lymphocytotox cross test must still be present. Patients without option to transplantation or without HLA-antibodies may receive also random preparations of individual donors or mixed preparations. AB0-minor-incompatibilities are without significance, AB0-major-incompatibilities are not permissible. The introduction of thrombocyte-specific testings is urgently to be aspired to.--Exclusively therapeutic substitution of thrombocytes, i.e. in manifest haemorrhage, is performed in a primarily not limited period of substitution, in a completely immune competent recipient with regularly low values of thrombocytes without cytoreductive therapy (example: aplastic anaemia). In these cases on account of high danger of sensitization only preparations of individual donors with HLA-A/B-match should be used. In cases of exception in thrombocytopenic, life-threatening haemorrhages on account of metabolic disturbances (immune thrombocytopenia, massive transfusions) the substitution with thrombocytic mixed concentrates is satisfied.--The substitution of thrombocytes is a common task of clinic and blood donation service, which opens new possibilities of therapy (e.g. transplantation of bone marrow).
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PMID:[Indication for prophylactic and therapeutic thrombocyte substitution]. 713 92

135 patients, suffering from aplastic anaemia (AA) and their families were genotyped for HLA. The antigen and haplotype frequencies were compared to an HLA genotyped control panel composed of 209 normal couples and their healthy offsprings, and to another series of 2286 normal individuals. An excess of HLA-A2 was observed in the patients: 61% versus 42% (pc less than 0.001) (relative risk: 2) and versus 48.5% (p less than 0.01) in two control series, respectively. When considering the HLA-A, B antigens shared in common by the parents of the AA patients, an excess of HLA-A2 was observed: 32% as compared to 17% shared by normal couples (p less than 0.001). An excess of homozygous HLA-A2 was noted in the AA patients (14%) in comparison to the normal controls (4%) (p less than 0.001). The mechanism of this association is discussed as well as the hypothesis of a gene involved in haematopoiesis which might interact within the HLA-A region.
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PMID:HLA markers in patients suffering from aplastic anaemia. 727 83

Among 42 consecutive recipients of unrelated marrow were 39 HLA-A, -B, -DR identical, matched unrelated donors (MUD) and three with one HLA antigen mismatch. The majority were genomically typed for DRB, DQA, DQB and DPB. The recipients of MUD marrow were compared with 39 recipients of marrow from HLA-identical siblings with similar diagnoses, disease status and age. Each group included 24 patients with hematological malignancies, 6 with severe aplastic anemia and 9 inherited disorders. Immunosuppression consisted of anti-thymocyte globulin (ATG; pre-BMT mainly to recipients of unrelated marrow), CsA and four doses of MTX. Grade I acute GVHD was treated with prednisolone 2 mg/kg. In a comparison of MUD marrow recipients and HLA-identical siblings 34 of 39 and 36 of 39 of the patients engrafted, respectively. Recipients of MUD marrow and HLA-identical siblings achieved 0.2 x 10(9) WBC/l on day 16 (median) and 14, respectively (P = 0.03). Furthermore, the recipients of MUD marrow needed more platelet transfusions (P = 0.04). The incidence of acute GVHD grade II-III was 15% in the MUD marrow recipients compared with 11% among the HLA-identical siblings. The 2-4 year cumulative incidence of chronic GVHD was 29% and 22% in the two groups, respectively. The overall 2-year survival was 59 and 78%, respectively. Among patients with CML in chronic phase or accelerated phase (n = 26), 2-year relapse-free survival was 79% in both groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Similar incidence of graft-versus-host disease using HLA-A, -B and -DR identical unrelated bone marrow donors as with HLA-identical siblings. 765 90


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