Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

For young patients with SAA, allogeneic bone marrow transplantation is the best curative approach. Major obstacles to the success of allogeneic bone marrow transplantation include GVHD, infections, and graft rejection. A careful management approach to patients prior to transplant may decrease some of these complications. Immunosuppressive therapy should be utilized in older patients with SAA. Underlying clonal disorders, eg, acute leukemia and recurrent aplasia, are late complications.
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PMID:Hypoplastic/aplastic anemia. Role of bone marrow transplantation. 157 64

Children with hematological malignancies (n = 33), severe aplastic anemia (SAA, n = 7) and other non-malignant diseases (n = 4) were followed for cataract development after bone marrow transplantation (BMT). The children with hematological malignancies were subjected to total body irradiation (TBI), 10 Gy, in one session with no shielding of the eyes as part of their conditioning regimen before BMT. The children with SAA or other non-malignant diseases received either no irradiation before BMT or a reduced dose, 8 Gy, with shielding of their eyes. After 3 years all patients who had undergone BMT for hematological malignancies had developed lens opacification. No patients in the other groups, without leukemia, showed any sign of cataract development. There was no relationship between steroid treatment for graft-versus-host disease and cataract development. No relation to age of onset of treatment or to the sex of the patient and cataract formation was seen. It seems evident from the present study that TBI given in one session was the main cause of cataract development after BMT.
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PMID:The development of cataract in children as a late side-effect of bone marrow transplantation. 187 89

4 patients (3 female, 1 male) bone marrow transplanted for severe aplastic anemia (3 allogeneic, 1 syngeneic) became pregnant or parented children 19, 37, 81, and 34, 52 months, respectively, after BMT. Conditioning regimen consisted of 200 mg/kg Cyclophosphamide. Donor buffy coat cells were used for rejection prophylaxis, and methotrexate was used as GVHD prophylaxis. 1 female patient developed a mild chronic graft versus host disease with vaginal sclerosis which made a cesarean section necessary. 3 out of 5 pregnancies were uneventful. 1 patient had mumps in the 14th week, another patient had a late abortion in the 25th week of unknown cause. A high incidence of offspring complications was noticed including 1) persistence of fetal circulation syndrome, 2) erythroblastosis fetalis, and 3) prolonged newborn-icterus. These observations confirm previous reports on resumed fertility after BMT for SAA. A relation between the unexpected high incidence of pre-, peri-, and postpartal complications and the BMT procedure can neither be proved nor excluded.
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PMID:[Pregnancy and fetal complications after bone marrow transplantation]. 194 50

We studied serum amyloid A levels of 31 bone marrow transplant recipients with and without acute graft-versus-host disease. Before transplantation the mean SAA concentration was 5.1 +/- 0.8 mg/l (mean +/- SEM). It remained low in patients with no signs of aGVHD but increased significantly during aGVHD to 54.0 +/- 8.2 mg/l (p less than 0.001 compared to pre-BMT value). Severe infections also induced high SAA levels.
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PMID:Serum amyloid A levels in acute graft-versus-host disease in bone marrow transplant recipients. 206 9

From a bank of 50,000 HLA typed French bone marrow donors, 125 transplants have been performed since 1986, with HLA AB and DR--identical MLC--negative donors. The median age was 25 years and the diagnosis was CGL in 59 cases, ALL in 22 cases, AML in 17 cases, SAA in 7 cases, inborn errors in 7 cases and others in 13 cases. Most of the patients received a standard conditioning regimen according to their diagnosis. The prophylaxis of GVHD was methotrexate and cyclosporine A in 77 cases; in addition to this combination 44 patients received an anti-IL2 receptor monoclonal antibody from day +1 to day +28. There was no difference between the two groups as regards the incidence and severity of GVH or survival. The actuarial survival was 36% with a median follow up of 300 days. Unlike matched sibling grafts, the usual prognostic factors such as stage of disease or age were not found to significantly modify the incidence of GVHD, which was 75%. The results of matched unrelated donor transplants are reasonably good, but must be improved by a better selection of donors and better prevention of GVHD.
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PMID:Matched unrelated bone marrow transplants. Results from the French group (GEGMO). 209 99

We report a single center experience of 222 patients (pts) less than 18 years old transplanted from 1973 to 1987. The median age was 11 years (1-18). The donor was a monozygotic twin (9 pts), an HLA-id sibling (193 pts), an HLA-id, parent (9 pts), a mismatched related donor (9 pts) and a matched unrelated donor (1 pt). Ninety-six pts were transplanted for SAA. Conditioning varied with time but the majority (59 pts) received CY 150 mg/kg and 6 Gy TAI. The long term actuarial survival is 66% with a median follow-up of 3 years. The group who received CY 200 mg/kg and MTX had a 33% long term survival (LTS). GVH was the main complication with 40% acute and 37% chronic GVHD. Chronic GVHD tended to improve with time after 2 to 4 years of evolution. Ninety pts were transplanted for leukemia (35 AML, 45 ALL and 11 CGL), 20 pts were in relapse. Pts in CR had a LTS of 40%, in pts in relapse, it was 12%. The main causes of death were: interstitial pneumonitis (30%), relapse (27%), GVH (15%). Thirty-five pts were transplanted for constitutional disease: Fanconi anemia (FA) (26 pts), Dyskeratosis congenita (2 pts), Blackfan-Diamond erythroblastopenia (2 pts), Glanzmann thrombasthenia (1 pt), osteopetrosis (1 pt) and Gaucher's disease (1 pt). In FA, the LTS is 70% with a CY 20 mg/kg, 5 Gy TAI regimen. In all disease categories, we did not find any influence of donor's sex on GVH and survival.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pediatric bone marrow transplantation for leukemia and aplastic anemia. Report of 222 cases transplanted in a single center. 267 24

The SAA Registry of the EBMT now contains data on 171 children younger than 15 years of age with acquired SAA and undergoing BMT between 1970 and 1988. The overall actuarial survival is 63% at 10 years. In a multivariate Cox analysis, the year of transplant was the most important prognostic factor with a significant advantage for children grafted in 1984-88 (81%) vs 1981-83 (67%) and 1970-80 (41%) (p = 0.02). Cyclosporine A given for GVHD prophylaxis, no treatment before transplant and an interval less than 90 days from diagnosis to BMT were all favourable variables in univariate analysis. As regard to transplant procedures, the better results were obtained using Cyclophosphamide and Cyclosporine A (78%) followed by Cyclophosphamide plus irradiation plus Cyclosporine A (77%). Sex, etiology and the severity of the aplasia had no impact on survival in both uni and multivariate analysis.
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PMID:Allogeneic bone marrow transplantation (BMT) for acquired severe aplastic anaemia (SAA) in children. 269 23

5 patients underwent bone marrow transplantation for severe aplastic anemia (2) and acute leukemia (ALL) in first remission (3). Graft versus host disease prophylaxis was performed by depleting T lymphocytes in the donor bone marrow with the rat monoclonal Campath-1 and autologous complement. In addition, patients received cyclosporin A. Engraftment occurred normally in all 5 patients but 1 patient (SAA) had a late graft failure. Two patients suffered mild degrees of GvHD. All patients are currently in complete remission, one having undergone a second transplantation.
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PMID:[Transplantation of allogeneic bone marrow treated in vitro with Campath-1 monoclonal antibody]. 390 87

Allogeneic BMT is the treatment of choice for patients with SAA who have an HLA-identical sibling donor. The results, however, have been relatively poor for transplants from partially matched family donors or unrelated donors because of the high incidence of graft rejection and/or GVHD. Six multiply transfused patients received a novel conditioning regimen of CY 200 mg/kg and TBI 800 cGy prior to receiving marrow from their HLA-haploidentical family donors. Three recipient-donor pairs were mismatched for one HLA locus, one for two loci and two for three loci. A combination of MTX and CsA was used for GVHD prophylaxis. Engraftment was noted in all six patients. Acute GVHD occurred in four patients, two each for grade I and II, respectively. One patient, who was ABO-compatible with her donor had delayed onset of pure red cell aplasia (PRCA) which completely recovered 6 months after additional immunotherapy with prednisolone. There were two deaths; both occurred while patients were on treatment for GVHD. One was from systemic fungemia and the other probably from cytomegalovirus interstitial pneumonitis (CMV-IP). Four patients (66.7%) have been alive and disease-free for more than 8.2, 27.3, 38.4 and 47.2 months after BMT, respectively. The results suggest that CY/TBI-800 may be a simple and effective conditioning regimen for SAA patients receiving BMT from family members other than HLA-identical siblings.
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PMID:CY/TBI-800 as a pretransplant regimen for allogeneic bone marrow transplantation for severe aplastic anemia using HLA-haploidentical family donors. 886 34

The clinical course of 59 children, who underwent BMT during 1988-1998 with a matched unrelated donor (MUD), was compared with 59 case controls receiving a sibling donor marrow. Thirty-eight patients had haematological malignancies while 21 had a nonmalignant disorder. The cumulative incidence of acute GVHD grade II-IV was 28% for MUD recipients vs 11% (P = 0.014) for sibling recipients. Extensive chronic GVHD was rare in both groups. The 5-year probability of survival was 52% for MUD vs 77% for sibling recipients (P= 0.014). For children with malignancies the 4-year probability of survival was 52% for MUD vs 67% for sibling recipients with a RFS of 49% vs 62%. In the ALL patients the survival of the MUD recipients was 77% and equalled that of the sibling group. For SAA survival was 43% vs 86% (P = 0.09) and for metabolic disorders 63% vs 89% (P = 0.025). The transplant-related mortality was higher in the MUD group, while death due to relapse was equally distributed. These results of MUD BMT in children compare favourably with most previous reports, and support the use of alternative donors in cases who lack an HLA-identical siblings. Bone Marrow Transplantation (2000).
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PMID:Unrelated bone marrow transplantation in children: outcome and a comparison with sibling donor grafting. 1082 66


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