Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seventeen patients with aplastic anemia or acute leukemia received transplants from donors who had major ABO incompatibilities. Antibody titers were decreased by plasma and whole blood exchanges prior to marrow infusion. All 17 patients were successfully engrafted, and there was one possible rejection in the patient with the highest pretransplant anti-A IgG titer. Nine of 17 patients are currently alive. A review was carried out of transplants performed in Seattle between HLA-matched siblings with aplastic anemia and leukemia. Two hundred forty-six evaluable patients with ABO-compatible donors were compared with 46 with minor ABO-incompatible donors. There was no effect of minor ABO incompatibility on graft rejection, incidence and severity of graft-versus-host disease, or survival.
 ...
PMID:ABO-incompatible marrow transplants. 3 Jan 94

A patient with aplastic anemia who was found to be homozygous for an HLA-D determinant shared by her unrelated parents achieved sustained engraftment and full restoration of hematopoietic and lymphoid function following a transplant from an HLA-A and -B nonidentical, ABO incompatible sibling who was heterozygous for the shared HLA-D specificity. Transplantation was complicated by transient graft-versus-host disease of moderate severity, which resolved completely following treatment with antithymocyte globulin and prednisone. The case indicates that patients found to be HLA-D-homozygous may be successfully transplanted from HLA-D-heterozygous sibling donors despite HLA-A and HLA-B incompatibilities, and thus further demonstrates the importance of the HLA-D region as a marker of donor-host histocompatibility.
 ...
PMID:Successful transplantation of marrow from an HLA-A, -B, -D mismatched heterozygous sibling donor into an HLA-D-homozygous patient with aplastic anemia. 3 52

By use of the continuous-flow blood-cell separator 137 bags of granulocyte-rich plasma were obtained from normal donors (59 bags) and patients with chronic granulocytic leukaemia (C.G.L.) (78 bags). Eighty-nine courses of granulocyte transfusion therapy consisting of 1 or more such bags were administered to forty-one ABO-compatible patients with acute leukaemia or aplastic anaemia, who had definite or probable infections that had failed to respond to antibiotics. The fever resolved after 67% of courses of transfusions of two or more bags but after only 24% of transfusions of single bags of granulocytes (p less than 0-01), and this result suggests that this form of treatment is in general effective. Granulocytes from C.G.L. and normal donors were equally effective, although transfusion reactions were commoner after C.G.L. cells (33% versus 12%, respectively, p less than 0-05). C.G.L. grafts, and probable graft-versus-host disease, occurred in three recipients of unirradiated C.G.L. cells. Recipients of normal cells whose fevers resolved received on average four times as many granulocytes per sq.m. as those fevers did not respond. No such difference was found when C.G.L. cells were used. The fever was more likely to resolve in recipients with established or clinically probable bacterial or fungal infections than in those with fever of uncertain cause. Fever was less likely to resolve in recipients with peripheral blood granulocyte counts before transfusion of greater than 1000 per mul. It is concluded that granulocyte transfusion therapy is a valuable advance in the management of infections in neutropenic patients.
 ...
PMID:Granulocyte transfusions in treatment of infections in patients with acute leukaemia and aplastic anaemia. 4 10

A patient suffering from aplastic anaemia was treated by bone-marrow transplantation from an ABO- and HLA-identical, MLC- and CML-negative, unrelated donor. MLC and CML became positive after transplantation indicating that a cellular immune response had developed against lymphocyte determinants not recognized prior to sensitization in vivo. Whether these determinants are governed by genes of the HLA region is unknown at present.
 ...
PMID:Education of lymphocytes in vivo following a transient take of a matched unrelated bone-marrow graft in man. 14 56

Bone marrow transplantation is a major therapeutic approach for treatment of patients with severe aplastic anemia or acute leukemia refractory to chemotherapy. It is possible only if an HLA matched sibing is found. ABO compatibility is not necessary. The conditionning regimen includes high doses of cyclophosphamide associated or not with a 1 000 rads total body irradiation. In acute leukemia, the two year percentage survival is 17%, in aplastic anemia it is 40%. Complications are immunologic : rejection, graft versus host disease and persistent severe immune deficiency.
 ...
PMID:[Allogenic bone marrow grafts (author's transl)]. 32 42

The role of the ABO blood group system in determining the outcome of bone marrow transplantation was investigated in 53 patients with aplastic anemia and acute leukemia grafted from HLA-identical siblings. There was no correlation between ABO compatibility and marrow engraftment, graft rejection, or graft-versus-host disease. In 5 recipients with antibodies prior to transplantation to antigens of the ABH system present on the cells of their donors, plasma exchange and antibody absorption in vivo were effective in permitting engraftment of ABO-incompatible bone marrow. These findings indicate that the ABO system is not a clinically significant barrier to successful bone marrow transplantation in otherwise histocompatible individuals.
 ...
PMID:ABO blood group system and bone marrow transplantation. 32 17

Transfusion of about 60 ml of ABO incompatible plasma in 4 units of pooled platelets precipitated severe haemolysis, unmasking the emergence of paroxysmal nocturnal haemoglobinuria (PNH), in a patient with aplastic anaemia. In vitro tests showed that her red cells were lysed by both ABO compatible and incompatible plasma from normal donors. The behaviour of this case and the in vitro results suggest that it might be hazardous to relax the longstanding recommendation on transfusing patients with PNH by restricting the washing of blood components to those containing ABO incompatible plasma.
 ...
PMID:Severe haemolysis and renal failure in a patient with paroxysmal nocturnal haemoglobinuria. 154 4

A 26-year-old ABO-O positive patient with aplastic anemia received a bone marrow transplant from his genotypically HLA identical, but ABO-A positive, brother. Engraftment of myeloid and megakaryocytic lineages occurred within 4 weeks but pure red cell aplasia and transfusion dependent anemia persisted for 160 days. The authors postulated that the failure of erythropoiesis was due to a high titer of anti-A isohemagglutinins. They tested this hypothesis with clonal cell cultures and flow cytometric analysis of ABO antigen expression by colony forming cells in vitro. During the period of prolonged red cell aplasia, the patient had normal numbers (85 +/- 12 per 10(6) cells) of circulating donor derived, burst forming units-erythroid (BFU-E). Immunophenotypic analysis of erythroid burst colonies derived from culture of the patient's bone marrow cells showed that 91 +/- 5% of 274 nucleated red cells were A-antigen positive, confirming full donor engraftment. Autologous plasma and complement added on day 1 of culture did not affect the colony growth (82.5 +/- 15 per 10(6) cells). However, when the addition of complement was delayed until day 7 of culture, there was 90% inhibition of BFU-E (7.5 +/- 5 per 10(6) cells) compared to controls (p less than 0.0004). Based on this, the authors propose a model for expression of ABO antigens during erythropoiesis, in which BFU-E do not express ABO antigens but their progeny do. The data support the hypothesis that the mechanism of prolonged pure red cell aplasia after ABO-incompatible bone marrow transplantation is complement mediated immune destruction of erythroid progenitors past the stage of BFU-E in differentiation.
 ...
PMID:Case report: isoimmune inhibition of erythropoiesis following ABO-incompatible bone marrow transplantation. 177 22

Haptoglobin was administered i.v. in 2 cases of ABO incompatible bone marrow transplantation for the prevention of hemoglobinuria due to acute hemolysis. The first case was a 25-year-old woman with severe aplastic anemia. The transplantation was both major and minor mismatch, where the donor was type A and the recipient was type B. The second case was a 31-year-old man with chronic phase of chronic myelogenous leukemia. The transplantation was major mismatch, where the donor was type B and the recipient was type O. After the administrations of haptoglobin 8,000 units we transfused bone marrow infusates from which incompatible erythrocytes were removed. Though prominent hemoglobinemia was observed in both cases, the serum haptoglobin values were not saturated and hemoglobinuria was not detectable. Haptoglobin appears to be useful for the prevention of acute renal failure associated with acute hemolysis in ABO incompatible bone marrow transplantation. Intravenous haptoglobin supplement therapy makes transplantation safer and may contribute to improve the recovery rate of total nucleated bone marrow cells after the erythrocyte depletion without worrying much about their contamination.
 ...
PMID:[Administration of haptoglobin in ABO incompatible bone marrow transplantation]. 225 63

We have studied long-term engraftment in 24 multiply transfused patients transplanted for severe aplastic anaemia (SAA) 2-7 years previously from HLA identical sibling donors. All 24 patients had engrafted initially; nine (38%) developed grade II-IV a-GVHD, but only 5 (21%) developed chronic GVHD, which was mild, localized and transient. In 22 cases DNA 'fingerprint' analysis using a hypervariable minisatellite DNA probe (33.15) confirmed the donor/recipient origin of patient peripheral blood (PB) nucleated cells. Red cell antigens and PB lymphocyte chromosomes were also analysed in informative cases. In 19 patients (79%) PB cells were of donor origin confirming sustained engraftment, whereas five (21%) had PB cells of recipient origin. In four of these five cases complete autologous reconstitution was demonstrated. In one case DNA fingerprinting revealed mixed haemopoietic chimaerism. In three of the four cases of autologous reconstitution there had been a previous episode of late graft failure. The low incidence of chronic GVHD in the study group was not explained by autologous reconstitution or mixed chimaerism. We conclude that the hypervariable minisatellite probes are valuable in the study of engraftment after BMT, especially when patient and donor are HLA identical, of the same sex, and have the same ABO-Rh blood type. Pre-transplant specimens from the patient are not necessary for interpretation of the results provided that DNA from the donor is available.
 ...
PMID:Use of a hypervariable minisatellite DNA probe (33.15) for evaluating engraftment two or more years after bone marrow transplantation for aplastic anaemia. 284 32


1 2 3 4 Next >>