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)

Bone-marrow transplantation (BMT) from an unrelated, HL-A-phenotype-identical, MLC-negative donor was performed in a 31 year old woman with severe longlasting aplastic anemia. In vitro assays failed to demonstrate humoral or cellular sensitization of the recipient against donor-type antigens. Following conditioning with cyclophosphamide, prompt but only transient engraftment of the transplant occurred accompanied by signs of mild graft-versus-host-disease (GVHD) of the liver. The results of a second bone marrow transplantation from the same donor cannot be evaluated due to early death of the recipient. It is concluded that bone marrow from unrelated, HL-A and MLC-identical donors may engraft without severe GVHD. Rejection of the graft in our patient may have been related to greater antigenic differences that can be expected to exist between HL-A and MLC-identical unrelated individuals than between HL-A and MLC-identical siblings. However, insufficient preparative immunosuppression with cyclophosphamide due to severe hepatic hemosiderosis appears equally likely as the cause of graft rejection. The possibly increased risk of graft rejection or severe GVHD should not preclude the use of unrelated HL-A and MLC-identical marrow donors, when histocompatible sibling donors are not available; but more potent immunosuppressive regimens than the cyclophosphamide protocol may be necessary to ensure permanent engraftment.
 ...
PMID:Bone marrow transplantation for aplastic anaemia from a HL-A and MLC-identical unrelated donor. 0 Nov 27

Marrow transplantation in aplastic anemia and leukemia has generally been limited to siblings who have been histocompatible at both the serological (A and B) and lymphocyte determined (D or MLC) loci of the HLA system. We studied three male patients, two with aplastic anemia and one with acute myelogenous leukemia, who received transplants from their histoincompatible mothers. MLC studies between donors and recipients showed varying degrees of stimulation. Definite engraftment occurred in one patient and transient engraftment in another. Engraftment in the third patient could not be evaluated. In the patient with sustained engraftment, there was clinical evidence of severe graft versus host disease (GVHD) however, this was not substantiated by histologic findings. This preliminary study suggests that MLC incompatibility may be more of an indicator of the risk of GVHD than of bone marrow rejection. If more effective control of GVHD can be accomplished, marrow transplantation between MLC-reactive individuals may become feasible.
 ...
PMID:Experience with incompatible maternal donors for bone marrow transplantation. 1 47

The mixed lymphocyte culture tests between one patient with acute lymphocytic leukemia and her mother and between another patient with aplastic anemia and his mother were found nonreactive, despite a one-haplotype difference for HL-A. Two crossovers in each family at the MLC locus or a specific defect in lymphocyte responsiveness could be possibly explantations. The mother of each patient could be considered a potential donor for bone marrow transplantation.
 ...
PMID:Atypical mixed lymphocyte culture. Identity between mother and child in leukemia and aplastic anemia. 12 70

Five patients with aplastic anemia were shown to be sensitized in vivo to the transplantation antigens of HL-A identical and MLC nonreactive sibling bone marrow transplantation donors. The sensitization in four cases were detected by antibody-dependent cell-mediated lysis (AICML) techniques, and in one case by antibody-dependent cell-mediated lysis (ADCML). In two cases the inherited nature of the sensitizing antigen was demonstrated, and in one case the inheritance of the antigen was shown by a family study to be independent of the HL-A and MLC loci. Maximal AICML was demonstrated in two cases at the time of bone marrow graft rejection. Successful transplants after immunosuppression with rabbit anti-thymocyte serum, procarbazine, and cyclophosphamide were performed in two patients, in whom sensitization was detected before transplantation.
 ...
PMID:Detection of genetically determined histocompatibility antigen differences between HL-A identical and MLC nonreactive siblings. 12 95

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 using an HLA-MLC-identical sibling is the most valuable treatment of severe aplastic anemia.2,6,7 Between November 1973 and March 1977, 25 consecutive patients have been treated by marrow transplantation in our unit. Nine patients are alive with complete hematologic restoration between 3 months and 3 years. The high mortality can be largely accounted for by marrow graft rejection (14 patients). Despite the small number of patients, we have tried to identify prognostic factors associated with marrow graft rejection. They are mainly the existence of anti-HLA antibodies, the sex difference, and the normal PHA and MLC response before grafting. After the graft, the disappearance of anti-HLA antibodies has a good prognostic value. The appearance of autolymphocytotoxins seems to correlate strongly either with rejection or graft-versus-host disease.
 ...
PMID:Allogeneic bone marrow transplantation in aplastic anemia--report of 25 cases. 34 52

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.
 ...
PMID:High dose granulocyte substitution after bone marrow transplantation in a case of aplastic anaemia in childhood. 37 43

Three consecutive patients considered to have end-stage acquired aplastic anaemia were given 100-160 mg/kg antilymphocyte globulin (ALG) i.v. followed by an infusion of 2-3.8 x 10(8) nucleated marrow cells/kg i.v. from HL-A one haplotype-identical, MLC-positive family donors. All patients showed autologous marrow reconstitutions lasting now 2-3 1/2 years. No clear-cut evidence of marrow engraftment could be established and no graft-versus-host disease was seen. It is assumed that these patients had some normal pluripotent haemopoetic stem cells which proved to be able of endoreduplication and of going into cycle after ALG conditioning and allogeneic marrow transfusion.
 ...
PMID:Autologous marrow reconstitutions in severe aplastic anaemia after ALG pretreatment and HL-A semi-incompatible bone marrow cell transfusion. 81 50

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.
 ...
PMID:Probability of finding a compatible sibling donor for bone marrow transplantation in Ireland. 153 37

Thirty-five patients underwent allogeneic bone marrow transplantation (BMT) from unrelated donors (UD), in a pilot study of the Canadian Bone Marrow Transplant Group with techniques routinely used in BMT from HLA-identical related donors. Thirty-two of the patients had hematologic malignancies and 3 had aplastic anemia. Donors and patients were matched at all HLA loci tested serologically in 29 cases; 19 of these patients had mutually non-reactive mixed leukocyte cultures (MLC's). Six patients had some degree of serologic mismatch. Stable engraftment occurred in all but 3 evaluable patients. Acute graft-versus-host disease (GVHD) developed in greater than 80% and fatal BMT-related deaths occurred in a total of 55% of all patients. Conversely, only two relapses have occurred, and the 1-year actuarial event-free survival for all patients is 40% (95% confidence intervals [CI], 24-55%) with a follow-up of 0.8 to 2.7 years. All survivors are out of the hospital and all save 1 have a normal performance status. Our study has confirmed the utility of unrelated donors for allogeneic BMT. Although more complications are seen than with HLA-matched sibling donors, these patients did not have such donors available and virtually all were incurable without transplants. Further studies, especially those using new methods to prevent transplant-related complications, are needed.
 ...
PMID:The use of unrelated donors (UD) for allogeneic bone marrow transplantation (BMT): a pilot study of the Canadian BMT Group. 204 87


1 2 3 4 Next >>