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)

An IgM antibody, present in the serum of a female patient with aplastic anemia, is described that reacted in a modified complement-dependent cytotoxicity test with a subset of the B cells from HLA-A2-positive, but not HLA-A2-negative males. With the exception of two HLA-A2 positive females, the antibody did not react with other cells from either HLA-A2-positive or HLA-2-negative females. The cells of one of these and from HLA-A2-positive males were able to absorb the antibody from the serum. Cells from other donors were unable to absorb the antibodies. The mononuclear cells of the same patient were cytolytic in cell-mediated lympholysis (CML) for phytohemagglutinin blasts from all HLA-A2-positive males and one of the females reacting with antibody, but not with blasts from HLA-A2-negative males and all other females. Thus, the results obtained with the antibody in the complement-dependent cytotoxicity test showed an almost perfect correlation with cytolysis in CML tests. These results suggest that the IgM antibody may be the first example with major histocompatibility complex restriction. Because the antibody reacted with the cells from two female donors, the restricting determinant is not, in all probability, the H-Y determinant.
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PMID:Major histocompatibility complex-restricted antibody reactivity mainly, but not exclusively, directed against cells from male donors. 50 Dec 84

In 68 idiopathic aplastic anemia patients (65 HLA genotypes), HLA-A2 is slightly increased (p corrected less than 0.03) leading to a relative risk of 2.30. A slight excess of homozygotes for HLA-A was observed (22.2 vs. 14.09%), involving mostly the A2 antigen. In the siblings the patients were more frequently homozygote A2 than by chance (p less than 0.005). The same trends are observed in Fanconi's anemia = out of 18 patients, 7 are HLA-A homozygote (of whom 4 are A2). The hypothesis of the existence of one or several recessive genes involved in hematopoiesis situated close to HLA-A is advanced.
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PMID:[Excess of HLA-A2 and HLA-A2 homozygotes in patients with aplastic and Fanconi's anemias]. 55 39

A male patient with severe aplastic anemia who had rejected a first T cell depleted graft from his HLA-identical sister was retransplanted from the same donor without T cell depletion and using an intensified conditioning regimen. After 8 weeks acute graft versus host disease (GVHD) developed and peripheral blood mononuclear cells (PBMC) were obtained. After in vitro restimulation and following limiting dilution cloning, 15 proliferative (PLT) and 25 cytolytic (CTL) T cell lines specific for host PBMC but unreactive to donor PBMC were isolated. Only 1 of 10 clones which could be expanded sufficiently for testing recognized a target cell (haploidentical sister), and in only 4 instances could a restriction element be found in a panel study (3 times HLA-A2; once HLA-BW49) of 13 unrelated stimulators. Of the 8 CTL clones testable after expansion, none showed a clear restriction and none recognized any of the family cells. Our data demonstrate that anti-patient reactive PLT and CTL lines can be found in PBMC. In no instance was segregation with HLA found. Only HLA-class I restriction was detectable.
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PMID:[In vivo detection of alloreactive T cells of the donor in graft versus host disease]. 354 Nov 72

Previous studies have shown that influenza virus-immune cytotoxic T lymphocytes can recognize virus in conjunction with self HLA-A2 antigens. Nevertheless, the virus-infected target cells from one HLA-A2-positive male donor (designated M7) could not be lysed by the virus-immune cytotoxic lymphocytes from any HLA-A2-matched unrelated donors. Although extensive serological analyses showed no difference between the HLA-A2 antigens of donor M7 and other HLA-A2-positive donors, isoelectric focusing of the HLA-A2 molecule from donor M7 revealed a clear difference in the heavy polypeptide chains when compared with the HLA-A2 molecules of other donors. The present study demonstrates that the HLA-A2-restricted anti-H-Y cytotoxic T lymphocytes obtained from a female aplastic anaemia patient fail to lyse the male M7 target cells, whereas the HLA-A2-restricted anti-H-Y antibodies from the same patient react with the cells of donor M7. These results suggest that: (a) HLA-A2-restricted anti-H-Y antibodies can recognize self determinants on the HLA-A2 molecule that are distinct from those that are recognized by HLA-A2-restricted anti-H-Y cytotoxic T cells; and (b) HLA-restricted T and B cells may use different receptor repertoires for the recognition of foreign antigens such as H-Y.
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PMID:Major histocompatibility complex-restricted H-Y-specific antibodies and cytotoxic T lymphocytes may recognize different self determinants. 617 22

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

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

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

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

The objective of this study was to analyse human leukocyte antigen (HLA) and disease association in common blood diseases [chronic myelogenous leukemia (CML), acute nonlymphocytic leukemia (ANLL), thalassemia and severe aplastic anemia] in Thais. The subjects were patients from the Hematological Clinic, Departments of Medicine and Pediatrics, Ramathibodi Hospital who were referred for HLA typing for bone marrow transplantation (BMT) at the Histocompatibility Laboratory from March 1988 to September 1997. A total of 129 patients had complete HLA-ABC typing. The patients included 45 CML, 40 ANLL, 26 thalassemia (Thal) and 18 severe aplastic anemia (SAA). Of these, 88 patients were typed for HLA class II. The HLA class I (ABC) and II (DR, DQ) typings were performed by microlymphocytotoxicity test. It was found that HLA class I was associated with CML, ANLL and Thal, whereas, HLA class II was associated with SAA. HLA-B8 and HLA-B18 were increased in CML with R.R. values of 12.2 and 3.9, respectively, whereas, HLA-B18 was increased in ANLL with R.R. value of 4.5. In addition, HLA-DR2 and DR3 were increased in SAA with R.R. values of 3.8 and 4.8, respectively. For Thal, HLA-A2 and B46 were increased in Thal in Central Thais with R.R. values of 3.3 and 6.1, respectively, whereas, HLA-B13 was increased in Thal in Northern Thais with R.R. value of 8.5. On the other hand, HLA-B7 was absent in CML. HLA-Cw7 was decreased in CML and SAA, whereas, HLA-DR6 was decreased in ANLL and SAA. Furthermore, HLA-Cw6 was also decreased in CML, whereas, HLA-A33 and Bw4 were decreased in SAA. Although the sample size of each disease was small, the increase of HLA-DR2 was observed in SAA in Thais which was similar to other studies in different ethnic groups. These preliminary data may be useful for further study in HLA and blood disease association.
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PMID:Preliminary study of HLA-ABCDR antigens in CML, ANLL, thalassemia and severe aplastic anemia in Thais. 1086 19