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)

Eleven of twenty-four patients with severe aplastic anemia given marrow grafts from HLA-identical siblings between October 1970 and March 1973 are alive with normal marrow function and continued evidence of engraftment 3-5 yr later. Ten have been leading normal lives with no immunosuppressive or other drug therapy since day 100 postgrafting. One has had chronic graft-versus-host disease of the skin which is now slowly improving with no therapy. He returned to full-time employment in the summer of 1975. The long-term well-being of almost half of our initial patients emphasizes the importance of marrow transplantation for the treatment of severe aplastic anemia.
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PMID:Allogeneic marrow grafting for treatment of aplastic anemia: a follow-up on long-term survivors. 78 10

Examples of patients are used as a basis for discussing the treatment of severe aplastic anaemia with clonal chromosomal aberrations in haematopoietic cells, the aplastic form of paroxysmal nocturnal haemoglobinuria and myelodysplasia, with allogeneic bone marrow transplantation. Transplantation with marrow from an HLA-identical sibling donor has curative potential in selected patients with these disorders. The procedure should preferably be carried out before the patients have received massive treatment for the complications of marrow failure. The use of matched unrelated bone marrow donors is still at the investigation stage.
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PMID:[New therapeutic possibilities for acquired clonal blood diseases and myelodysplasia]. 141 33

Use of allogeneic bone marrow transplants continues to increase. During the 36-year period between 1955 and 1990, more than 33,000 patients received allogeneic bone marrow transplants; more than 45% of these were performed during the 3 years 1988-1990. Transplants are effective therapy for leukemia and other hematologic diseases. It is widely considered that transplants are the treatment of choice for aplastic anemia and chronic myelogenous leukemia, those who fail conventional therapy for acute leukemia and a variety of genetic, metabolic and immune deficiency disorders. Successful application of bone marrow transplantation is limited by complications such as graft failure, graft versus host disease GVHD and interstitial pneumonia and, until recently, the requirement for an HLA-identical sibling donor. In the past few years, an increasing number of transplants were performed using unrelated or HLA-partially matched related donors with some success. Development of post-transplant complications can often be predicted by risk factor assessment. In this report, current data from the IBMTR are summarized and several risk factors affecting outcome identified.
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PMID:Current status of allogeneic bone marrow transplantation. 142 Oct 39

Over the last 20 years allogeneic bone marrow transplantation from an HLA-identical sibling donor has become the treatment of choice for a number of human haematological malignancies, severe aplastic anaemia, some congenital diseases of the immune and haemopoietic systems, and some inborn errors of metabolism. Recently, the successful introduction of HLA-matched unrelated donor transplants, convenient T cell depletion technology, combination immunosuppressive therapy to minimise graft-versus-host disease, blood products that are seronegative for cytomegalovirus, effective antiviral agents, and cloned haemopoietic and immune system growth factors have markedly increased the scope of bone marrow transplantation. Additionally, autologous transplantation appears to have promise especially in lymphoma and breast cancer.
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PMID:Bone marrow transplantation. 144 94

In order to determine the incidence and causes of death during the first 100 days after BMT (early deaths) in a pediatric population we have examined data reported in the AIEOP BMT Registry. Up to July 1990, data on 486 children who underwent allogeneic (180) or autologous (306) BMT were evaluable. The children had acute lymphoblastic leukemia (148 cases), acute non-lymphoblastic leukemia (127 cases), neuroblastoma (82 cases), chronic myelogenous leukemia (15 cases), aplastic anemia (nine cases), solid tumors, lymphoma, immunodeficiency or storage diseases. The overall survival is 55% for allogeneic HLA matched and 38% for autologous transplants at 5 years, 24% for HLA mismatched graft at 2 years. Out of the 486 children, 70 (14%) died during the first 100 days after BMT: 33/306 (11%) after autologous BMT, 24/150 (16%) after allogeneic matched BMT and 13/30 (43%) after mismatched BMT. Causes of early death were as follows: disease progression: 12 children (10/306 after autologous and 2/180 after allogeneic BMT); infection: 12 children (five after autologous and seven after allogeneic BMT); interstitial pneumonitis: 21 children (seven after autologous and 14 after allogeneic BMT); cardiac failure: five children (four after autologous BMT); veno-occlusive disease: eight children (three after autologous, five after allogeneic BMT); acute renal failure: three children (one after autologous and two after allogeneic BMT); multiple organ failure: two cases (one after autologous BMT); cerebral hemorrhage: three children (one after autologous BMT); hypertension: one child; acute GVHD: three children (12% of early deaths after allogeneic BMT).
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PMID:Early deaths in children after BMT. Bone Marrow Transplantation Group of the Italian Association for Pediatric Hematology and Oncology (AIEOP) and Gruppo Italiano Trapianto di Midollo Osseo (GITMO). 146 3

Conditioning regimens for transplantation are important in determining transplant outcome. This review focuses on transplantation in aplastic anemia and leukemia using marrow from HLA-identical siblings. Results of conditioning with newer regimens such as busulfan plus cyclophosphamide and etoposide plus total body irradiation are reviewed and compared to results achieved with cyclophosphamide and total body irradiation. The potential for improved results using recent innovations such as dose adjustment of busulfan, agents which may decrease transplant-related toxicity, and directed radiation are discussed.
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PMID:Conditioning regimens for allogeneic bone marrow transplantation. 148 92

Bone marrow transplantation from an HLA-identical sibling is increasingly used as a curative therapy for patients with hemopoietic stem cell disorders including acute leukemia, chronic myelogenous leukemia and severe aplastic anemia. Between March 1983 and March 1991, we performed 86 cases of allogeneic bone marrow transplantation (BMT) for the patients with hemopoietic stem cell disorders: 25 acute myelogenous leukemia (AML); 15 acute lymphoblastic leukemia (ALL); 20 chronic myelogenous leukemia (CML); and 26 severe aplastic anemia (SAA). Ten out of 25 AML are in disease free survival (DFS). The causes of death were recurrence of leukemia (12), acute GVHD (3), sepsis (1) and veno-occlusive disease (1). Nine of 15 ALL are in unmaintained remission. Thirteen out of 20 CML are in DFS. Among 26 SAA, 21 are enjoying DFS, but 1 died of engraftment failure, 3 of graft rejection followed by cytomegalovirus (1) and aspergillus pneumonia (1). Comparing the survival between standard [less than or equal to CR1: 9/14 (64%)] and high risk [greater than or equal to CR1: 1/11 (9%)] AML, our data suggest that preparative regimen for high risk AML was not potent enough to eradicate the minimal residual disease in advanced AML. Although our cases are limited and the follow-up period is short, our result of ALL [overall: CCR (60%), standard risk (adult less than or equal to CR1, children less than or equal to CR2; 8/11 (73%) and high risk; 1/4 (25%)] and CML [overall: 65%, CP; 9/10 (90%), AP; 4/6 (67%), BP; 0/4 (0%)] are optimistic. It is of our interest that the incidence of death related with IP (1/33: 3%) and with AGVHD 94/33: 12%) were much less than that of other's observation but the explanation for this still remains to be clear.
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PMID:Allogeneic bone marrow transplantation for the patients with hemopoietic stem cell disorders: CUMC experience. 151 32

Transfusion associated graft versus host disease is a rare disorder usually confined to patients who are immunosuppressed. A case is described in a 77 year old woman who was presumed immunocompetent. She was transfused with one unit of blood from an individual who was homozygous for the same HLA haplotype as her. The diagnosis of transfusion associated graft versus host disease should be suspected in a patient who develops aplastic anaemia within 30 days of a transfusion of blood products. It is suggested that blood donations from first degree relatives should not be permitted, unless the donation is irradiated to prevent lymphocyte proliferation.
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PMID:Transfusion associated graft versus host disease in an immunocompetent patient. 151 64

We previously demonstrated that multitransfused patients with severe aplastic anaemia (SAA) exhibit high numbers of alloreactive cytotoxic T lymphocyte precursors directed against their HLA identical siblings. In this study a group of patients who had received multiple blood transfusions for SAA, other haematological diseases or acute blood loss were tested for autocytotoxicity and the results compared with those of untransfused controls. These controls consisted of normal individuals, patients with chronic myeloid leukaemia (CML) or untransfused patients with SAA. There was a significantly higher degree of autocytotoxicity in multitransfused patients, than in the untransfused controls, including untransfused patients with SAA (P = 0.0001). These results suggest that blood transfusion is responsible for inducing autoreactivity. In one patient, in whom both alloreactive anti-non-MHC and autoreactive cytotoxic T lymphocytes (CTL) had been detected, it was demonstrated that there was no crossreactivity between the alloreactive and autoreactive CTL responses. Inhibition studies using monoclonal antibodies revealed the effector cells to be T lymphocytes and the restricting determinants to be both HLA class I and II molecules.
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PMID:Lymphocytes from multi-transfused patients exhibit cytotoxicity against autologous cells. 152 Jun 20

This study was undertaken to ascertain the frequency of compatible sibling donors for individuals requiring bone marrow transplantation (BMT) in Ireland. During the study period 1984-89, a total of 392 patients were HLA typed. Of these, 218 (55.6%) had a compatible sibling donor. Among the latter there were 4 degrees of compatibility: 168 (42.9%) were HLA-A,B,DR identical MLC unreactive: three (0.8%) were HLA-A,B,DR identical MLC reactive: 12 (3.0%) were HLA-A,B,DR identical (no MLC performed) and 35 (8.9%) were HLA-A, B identical (no DR or MLC performed). The leukaemias and aplastic anaemia comprised 82.9% of all requests. The majority of patients with acute myeloid leukaemia (64.4%), acute lymphoblastic leukaemia (51.2%), chronic myeloid leukaemia (73.9%) and aplastic anaemia (77.3%) had a potential sibling donor. Subsequently 144 of these patients had an allogeneic BMT, 79.9% of which were for patients with leukaemia (acute and chronic). This study found that there was a higher probability of finding a donor within the family than reported in most series. A clear relationship was demonstrated between family size and the likelihood of obtaining a HLA-identical sibling donor.
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PMID:Probability of finding a compatible sibling donor for bone marrow transplantation in Ireland. 153 37


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