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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A review of the present results of bone-marrow transplantation (B.M.T.) in severe aplastic anaemia is presented. Nowadays, there is little doubt that for patients with severe aplastic anaemia the treatment of choice is the B.M.T. provided always that a suitable donor exists. If the patient is fortunate enough to have a normal identical twin, the syngeneic B.M.T. without immunosuppresive conditioning must be performed. This is usually successful, though in some patients failures can be observed due to probable immunological interference. This can be overcome by a new syngeneic B.M.T. preceded by immunosuppression. The usual type of suitable donors is an HLA-identical (including locus D) sibling (allogeneic B.M.T.). Approximately 50% of patients treated in this way can become long-term survivors. The chief complications causing mortality from the allogeneic B.M.T. are graft rejection and graft-versus-host-disease (G.V.H.D.). In order to reduce the graft rejection rate, transfusions from marrow-donor and relatives prior to the transplantation should be avoided. Other probable factor influencing the final outcome of the allogeneic B.M.T. are the interval diagnosis-transplantation, age of the patient, marrow cell dose, the difference of sex between the donor and the recipients and others. Semi-incompatible and incompatible allogeneic B.M.T. are briefly considered.
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PMID:[Results of bone marrow transplantation in severe aplastic anemia. Bibliographic review]. 4 20

Much progress has been made in allogeneic bone marrow transplantation for severe aplastic anemia (SAA) and acute leukemia (AL). In SAA it was shown that hemopoietic chimerism and apparently permanent cures can be achieved in the majority of patients by conditioning with cyclophosphamide followed by bone marrow transplantation (BMT) from an HLA-identical sibling. The previous transfusion history is crucial for failure or success: untransfused patients do very well while graft rejection is an enormous problem in most polytransfused ones. We have shown that most patients without HLA-identical sibling donors can be adequately helped as well. After conditioning with ALG followed by transfusion of haploidentical marrow and low dose androgens there is partial to complete autologous hemopoietic reconstitution in virtually all patients. This points to the fact that most of these patients have pluripotent hemopoietic stem cells that are intact, but apparently unable to differentiate to mature cells, because they are inhibited by autoimmune mechanisms. The results of BMT in patients with endstage leukemia are modest. New pilotstudies with early marrow grafts, i.e. for ANLL in first remission and for ALL in second remission indicate that with this type of approach potentially over 50% of all patients with HLA-identical siblings can be cured. We recommend that HLA-typing should be performed early in families with SAA and AL and that the possibility of a marrow graft should be seriously considered before the patients have endstage disease. Marrow grafts are technically simple but they may pose enormous problems such as graft versus host reaction (GvH), interstitial pneumonia, graft rejection and leukemic recurrence. Therefore, the procedure should only be performed in highly specialized centers with much knowledge and experience in the immunobiology of bone marrow transplantation.
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PMID:[Bone marrow transplantation in severe aplastic anemia and acute leukemia]. 4 65

9 patients with severe aplastic anemia (SAA) were treated with bone marrow transplantation (BMT). 5 were conditioned with cyclophosphamide and received and HLA-identical graft (4 patients) or a mismatched graft (1 patient): 1 rejected the graft on day 30 and died on day 34 during conditioning for a second transplant; 1 died on day 15 with acute and severe graft versus host disease (GvHD) in the absence of haemopoietic engraftment; 3 are alive and complete chimeras at 1,069, 490 and 332 days after transplantation. GvHD developed in 4 patients and was treated successfully in 3 with high dose methylprednisolone and/or antilymphocytic globulin (ALG). 4 patients were conditioned with ALG and received bone marrow from a haploidentical sibling or parent: 1 patient was refractory; 3 patients showed evidence of hematologic reconstitution, but 2 of these required a second course of ALG. 3 patients in this group are alive between 60 and 490 days; 1 patient died on day 121 of HBSAg-negative acute hepatitis.
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PMID:Bone marrow transplantation for severe aplastic anemia. A report of 9 cases. 4 62

Experience with 17 allogeneic bone marrow transplantation (BMT) patients with refractory acute leukemia is reported. The authors show that it is extremely difficult to cure patients with end stage disease by this procedure. Based on experience that, if performed early, BMT can bring about over 50% potential cures, it is postulated that marrow grafts should be performed much earlier than is done at present. From the results in 45 patients with severe aplastic anemia it is concluded that the majority do not have a defect at the pluripotent stem cell itself. There is disturbed maturation to functional end cells which can be corrected in most instances by ALG, infusion of allogeneic marrow and low dose androgens. BMT between HLA-identical siblings need not cause major problems, but it involves a considerable risk of fatal complications such as irreversible marrow rejection and GvH.
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PMID:[Progress in bone marrow transplantation in acute leukemia and aplastic anemia]. 4 94

Using the Seattle protocol with minor modifications, 23 patients with severe aplastic anaemia received allogeneic bone marrow transplants from HLA/mixed leucocyte culture matched sibs in three London centres between 1973 and 1977. Ten patients (43.5%) are alive 6 months to 5 years after transplantation, and are well with full haemopoietic reconstitution, two with autologous bone marrow recovery following the graft procedure. A failure of the marrow graft to take, or take followed by rejection occurred in 12 patients (52%). Failure of marrow recovery was associated with a high early mortality from bacterial or fungal infection. The only survivors amongst those who rejected the first graft were four patients in whom a subsequent graft from the same donor was successful, and two in whom autologous recovery occurred. Graft versus host disease (GVHD) occurred in seven patients, and was fatal in one case. The most frequent complication after successful engraftment was varicella-zoster infection which occurred in five patients and was fatal in one patient. The overall results compare favourably with those from other transplant centres, but the high rate of graft rejection and low incidence of GVHD differ from other series. The results should encourage further referral of patients with severe AA for bone marrow transplantation.
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PMID:Allogeneic bone marrow transplantation for severe aplastic anaemia--the London experience. 4 92

Twenty-one patients weth severe aplastic anaemia were evaluated at a single hospital between 1972 and 1975. Patients without histocompatible donors were treated conventionally with androgens, corticosteroids, and HLA-matched platelet and granulocyte transfusions. Bone-marrow transplantation was performed in patients with HLA-identical siblings. The two groups had comparable clinical and haematological prognostic indicators and received similar supportive therapy. All nine patients who were not transplanted died. Median survival in these patients was 82 days (range 21-545). Seven of the twelve (58%) transplant recipients were alive at 120 days--greater than 930 days (p less than 0.04). The one-year actuarial survival of the transplant group was 53%. This study shows that bone-marrow transplantation is a rational alternative to conventional therapy in selected patients with aplastic anaemia.
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PMID:Bone-marrow transplantation in severe aplastic anaemia. 6 59

29 patients with severe aplastic anaemia were treated with either antilymphocyte globulin (A.L.G.) alone (15 patients) or A.L.G. followed by infusion of allogeneic bone-marrow (14 patients). The overall response to both forms of treatment in terms of 1-year survival was 55%; 12 of the 29 patients showed a sustained haematological improvement, during a period of observation of up to 4 1/2 years. No potentially fatal complications were observed. None of the bone-marrow infusions led to a permanent "take" or graft-versus-host disease. How A.L.G. acts is unknown, but our findings accord with the hypothesis that, in a substantial proportion of cases of aplastic anaemia, unspecified autoimmune reactions block the development of residual stem cells A.L.G. seems to offer a good chance of survival, especially for those patients who do not have HLA-matched siblings. Its value should be further established.
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PMID:Treatment of aplastic anaemia by antilymphocyte globulin with and without allogeneic bone-marrow infusions. 7 59

A female patient with severe idiopathic aplastic anaemia received a successful bone-marrow transplant from her HLA-identical, mixed-lymphocyte-culture-compatible, brother. 8 months after transplantation she had localised cutaneous measles. Chronic sclerodermatous changes developed which were indistinguishable from chronic graft-versus-host disease and were limited to the areas of the original exanthem. Interaction between viral infection and minor histocompatibility differences probably resulted in graft-versus-host disease in this patient.
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PMID:Sclerodermatous graft-versus-host disease limited to an area of measles exanthem. 7 22

One-way mixed leukocyte cultures were used to study the ability of cells from 53 patients with aplastic anemia and 65 of their HLA-identical siblings to stimulate and to respond to cells from unrelated individuals. The capacity of leukocytes from 28 of 53 patients to stimulate was diminished or absent, wheras their ability to respond remained comparable to that of cells from their HLA-identical siblings. Leukocytes from the group of patients with the lowest peripheral lymphocyte count were least stimulatory. The results indicate that in acquired aplastic anemia there exists a dissociation between stimulation and response in mixed culture.
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PMID:Aplastic anemia: failure of patient leukocytes to stimulate allogeneic cells in mixed leukocyte culture. 13 Sep 46

Thirty-four patients with severe aplastic anemia were treated with cyclophosphamide or total body irradiation and an infusion of marrow from a genotypically HLA-identical sibling donor. Engraftment occurred in all patients but rejection was noted in 12. Mixed leukocyte culture (MLC) tests undertaken before grafting to determine donor-recipient histocompatibility were analyzed retrospectively. The response of patient cells to sibling cells in mixed culture was compared to the response of patient cells to cells of an unrelated individual and expressed as a relative response index (RRI). Of the 12 patients who showed positive RRI to donor leukocytes in pregraft MLCs, 9 rejected their grafts, wheras of 23 patients with negative RRI only three rejected their grafts (P LESS THAN 0.005). The results suggest that the MLC test can detect individuals who are most likely to reject their HLA identical sibling graft.
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PMID:Correlation of the relative response index with marrow graft rejection in patients with aplastic anemia. 13 86


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