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)

A bone marrow transplantation (BMT) was successfully carried out in a seven-year-old boy with aplastic anaemia. An HLA and MLC-identical brother served as donor. During the first 16 days after BMT between 0.55 and 7.11X10(10) granulocytes were transfused 14 times (an average of 4.03X10(10) per transfusion). The maximum increment in the peripheral blood after transfusion was over 4000 granulocytes per microliter. The course of local infections was taken as parameter for determining the clinical effectiveness of the granulocyte transfusion. Under the substitution therapy the necrotic ulcer of a fingertip and the mucous membrane ulcers in the oral cavity subsided. The presence of transfused granulocytes in the oral cavity ("orogranulocytes") could be established. There was a statistically significant relationship between the number of granulocytes in the peripheral blood and the number of orogranulocytes. It could be demonstrated that in children a temporary normalisation of the number of granulocytes in the peripheral blood can be attained by means of granulocyte substitution and that the transfused granulocytes are clinically effective.
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PMID:High dose granulocyte substitution after bone marrow transplantation in a case of aplastic anaemia in childhood. 37 43

The sera of 28 patients with aplastic anaemia were examined for their effect on granulocyte colony growth in soft agar. Normal sera did not affect colony growth, but 13 sera from patients with aplastic anaemia, three from multiparous women, and six from patients polytransfused for various disorders caused colony inhibition. This inhibition was not due to the presence of HLA antibodies in aplasia patients because some sera inhibited HLA compatible bone marrow, and polyspecific HLA antibodies were not found in all inhibitory sera. All patients who failed to show engraftment or who rejected their bone marrow graft within three weeks had serum inhibitory to normal bone marrow cell culture, but inhibition could not be demonstrated against autologous bone marrow cells in these patients with aplastic anaemia. The results show that patients with serum inhibitors have an increased risk of early graft rejection and suggest that this rejection is mediated by antibodies directed against bone marrow stem cells.
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PMID:Marrow graft rejection and inhibition of growth in culture by serum in aplastic anaemia. 37 46

A 26-year-old man with severe aplastic anemia was treated with high-dose Cyclophosphamide followed by the infusion of bone marrow cells from his HLA-identical sister. After initial intake of the graft, rejection ensued by day 46 which was followed by a permanent complete aplasia. After 4 months, bone marrow retransplantation with the same donor was attempted after a more intensive conditioning regimen. This led to permanent engraftment with rapid normalization of the blood counts lasting now for over 12 months. The patient has since remained in excellent clinical condition without signs of graft-versus-host disease.
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PMID:Successful allogeneic bone marrow retransplantation with the same donor after graft rejection: application of a modified conditioning regimen. 38 Jun 89

18 patients with severe aplastic anemia (SAA) but without an HLA-identical sibling were treated by antilymphocyte globulin (ALG) followed by infusion of marrow cells from a semi-compatible family donor. 13 of these received low dose androgens after ALG: 11 (85%) achieved stable remission without transfusion requirement. One patient relapsed after 4 months, one patient with only partial remission died from infection. None of the 4 patients who did not receive androgens after ALG achieved remission. ALG, marrow and low-dose androgens represent a promising therapy for SAA and can be favorably compared with allogeneic bone marrow transplantation.
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PMID:[Immunosuppression, bone marrow infusion and low dose androgens, successful therapy of severe aplastic anemia]. 38 11

Two cases of multitransfused severe aplastic anemia were retransplanted with bone marrow from the same HLA compatible sibling donors after subtotal body irradiation (800 r). Only minor non hematologic toxicity was observed. No permanent take was seen in relation to this procedure. During the survival time of the patients (78-120 days) no signs of interstitial pneumonia were observed.
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PMID:Subtotal body irradiation with linear accelerator as preparation for marrow engraftment in aplastic anemia. 38 12

We proposed the hypothesis that there is an antigen on the hepatitis B virus (HBV) that cross-reacts with a male associated antigen to explain four observations: 1) sex differences in the responses to HBV infection, 2) sex differences in the prevalence of chronic liver diseases associated with hepatitis B, 3) the association of parental responses to HBV with the sex ratio (at birth) of their offspring, and 4) the relation of kidney graft survival with the combination of anti-HBs in the recipients and the sex of the organ donor. Patients with aplastic anemia treated with bone marrow transplantation from HLA identical sibling donors were studied to further test this hypothesis and the results provide additional support. Cross reactivity of HBsAg with H-Y antigen, however, has not been demonstrated.
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PMID:Sex differences in response to hepatitis B virus. III. Responses to HBV and sex of donor and recipient in kidney and bone marrow transplantation. 38 50

Bone marrow (BM) morphology was studied in patients who underwent bone marrow transplantation (BMT) for severe aplastic anaemia (SAA) and acute myeloid leukaemia (AML). Four patients with SAA had marrow transplanted from HLA-identical siblings after conditioning with cyclophosphamide (CY); three cases of AML were treated with allogeneic BMT. The medullary material was generally obtained by aspiration, but treated so as to preserve its architecture; fresh, postvital preparations were particularly useful for this purpose. In SAA, the bone marrow before transplantation showed dense infiltrates composed of macrophages and other inflammatory cells; after BMT, repopulation started at the periphery of the infiltrates, when they were not totally destroyed by CY. Granulocytic repopulation generally preceded erythropoiesis, which always occurred by way of typical erythroblastic reticulocytopoietic islands. Marked but transient dyserythropoiesis was found in two cases; in one of them, it was considered as drug-dependent. Both in SAA and in AML, but more so in the latter, a marked and sometimes imposing macrophagic hyperplasia was found after transplantation. Many macrophages were actively engulfing erythrocytes and nucleated cells. This last type appeared prominent in rejection episodes.
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PMID:Transplantation haemopoiesis. Morphological bone marrow studies after allogeneic marrow transplantation in man for severe aplastic anaemia and acute leukaemia. 39 Apr 94

We studied the effects of peripheral blood lymphocytes from 16 untransfused patients with severe aplastic anemia (AA) of diverse etiologies on the growth of granulocyte-macrophage colonies from normal marrows. Normal lymphocytes in our system increased the number of granulocytic colonies by 31 +/- 6% (mean +/- SEM). Lymphocytes from 3 of 16 untransfused AA patients significantly inhibited growth in HLA-matched sibling marrows (-30%, -40%, and -37%; p less than 0.01). Although these results suggest that the majority of cases of AA are not mediated by a coculture-detectable immunologic mechanism, studies using lymphocytes obtained from AA patients before transfusions may detect the subpopulation whose disease is immune-mediated and who may therefore respond to immunosuppressive therapy.
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PMID:Coculture studies of 16 untransfused patients with aplastic anemia. 44 65

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

Bone marrow transplantation from HLA-matched allogeneic donors was used to treat two series of patients with severe aplastic anemia. No significant differences were detected in all comparisons made between the Registry series (38 patients) and the Seattle series (24 patients), and pooled data from the two series were used in the analyses. Currently, 55% of the patients who received bone marrow transplants within three months of diagnosis are alive, but only 13% of the patients who received bone marrow transplants more than nine months after diagnosis are alive. The difference between the two groups was significant (P less than .02). Patients less than 21 years of age had a significantly higher survival rate than those patients who were 21 years or older at the time of transplantation (P less than .02). Survival rates were significantly higher for patients who had received 15 or fewer pretransplant transfusions than those who received more (P less than .05).
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PMID:Bone marrow transplantation from donors with aplastic anemia. A report from the ACS/NIH bone marrow transplant registry. 78 33


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