Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:2.1.1.69 (BMT)
2,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the literature 63 patients with myelodysplastic syndromes (MDS) are reported who were treated with syngeneic or allogeneic bone marrow transplantation. 62 patients were prepared for BMT with TBI containing regimens or Busulfan/Cyclophosphamide. GvHD prophylaxis was heterogenous. 33/63 patients are alive and well between 4 and 132 months after BMT. 23/63 patients died due to GvHD (n = 6), interstitial pneumonitis (n = 6), other infections (n = 4), toxicity of the preparative regimen (n = 5) or graft failure (n = 2). 9/63 patients relapsed between 2 and 98 (in median 6) months after BMT. There seems to be a correlation between the subtype of the MDS and the relapse rate: only 1/18 patients with RA/RARS relapsed compared to 7/42 patients with RAEB/RAEB-T. These results indicate that marrow transplantation can induce long term survival and may result in cure of patients with MDS.
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PMID:Allogeneic bone marrow transplantation for myelodysplastic syndromes (MDS). 269 17

Myelodysplastic syndromes (MDS) represent an acquired group of clonal disorders of the pleuripotent stem cells, resulting in progressive life-threatening cytopenias or transformation into acute leukemias. A major issue of using alloBMT in MDS is the criteria used for patient selection. Therapeutic trials of lesser intensity such as differentiating agents, and cytokines could be the preferable choice for patients with good prognostic features. On the other hand, patients with poor prognostic features may urgently need to establish a normal hematopoiesis through allogeneic bone marrow transplantation (alloBMT). Important prognostic indicators in MDS include FAB classification, presence of abnormal localization of immature precursors, degree of cytopenias and cytogenetic abnormalities. We used a novel preparative regimen--"BAC" consisting of the consecutive administration of 1 mg/kg of busulfan every 6 hours for 16 doses followed by 2 g/M2 of cytosine arabinoside (ara-C) given every 12 hours for four doses, and finally 60 mg/kg of cyclophosphamide daily for 2 days. Thirty two patients transplanted had a median age of 33 years. Nine of the patients had either RA or RARS, 21 had RAEB or RAEB-T and 2 were unclassified MDS. Twenty two of our patients had chromosomal abnormalities while 10 had a normal karyotype analysis. Nine of the 32 patients had documented leukemic transformation and received induction therapy prior to BMT. The median time from diagnosis to BMT was 5.6 months, ranging from 1.3 to 30.2 months. Nineteen out of 32 patients are alive without disease, with a median follow up of 24 months. The actuarial event-free survival for the entire group is 52%. Two patients have relapsed with an actuarial relapse rate of 12%. Only significant favorable prognostic indicator for the event-free survival was in the recipient of a genotypically matched graft (76%) compared to recipients of a non-genotypic graft (23%) (p = 0.02). "BAC" is a unique preparative regimen for alloBMT in MDS with excellent results.
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PMID:Allogeneic bone marrow transplantation in patients with myelodysplastic syndromes. 781 96

The Chronic Leukemia Working Party of the EBMT has collected data on 118 patients of median age 24 years (range 0.3 to 53 years) who underwent an allogeneic bone marrow transplantation from unrelated donors for treatment of MDS or secondary AML (RA/RARS, n = 24; RAEB, n = 26; RAEB-t, n = 34; CMML, n = 12; sAML, n = 22) between 1986 and 1996. The data were reported by 49 EBMT centers. Thirty-four of 118 patients are alive, relapse was the cause of death in 19 of 84 patients and the remaining patients died of transplant-related mortality. For the whole group the actuarial probability of survival at 2 years is 28%, disease-free survival 28%, relapse risk 35% and transplant-related mortality is 58%. The transplant-related mortality is significantly influenced by the age of the recipient (<18 years 40%, 18-35 years 61%, >35 years 81%). The relapse rate after BMT is influenced by FAB classification of the disease at BMT. Patients with a low blast count (RA, RAEB) have a lower probability of relapse (13%, 15%) compared to patients with RAEB-t or sAML (29%, 45%). Furthermore, we found evidence of a graft-versus-leukemia effect in MDS/sAML. Patients with acute GVHD, grade II-IV, had a probability of relapse of 26% vs 42% in patients with no acute GVHD or grade I only. Allogeneic transplantation with an HLA-matched, unrelated donor may be offered to younger patients (age <35 years) with poor risk myelodysplasia or secondary AML.
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PMID:Unrelated bone marrow transplantation in patients with myelodysplastic syndromes and secondary acute myeloid leukemia: an EBMT survey. European Blood and Marrow Transplantation Group. 967 54