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)

Ninety-one patients with de novo acute myeloid leukemia (AML) in first complete remission (CR) undergoing an HLA-identical sibling BMT and with a minimum follow-up of 12 months were analyzed for disease-related and transplant-related variables predicting survival and relapse. The overall actuarial 5 year survival is 53% and the relapse rate 29%, with a medium follow-up for surviving patients of 1552 days (range 365-4094 days). In univariate analysis the following variables were found to be associated with an increased risk of failure: high-dose cyclosporin (CsA), M4-M6 FAB subtype and a long interval (> or = 180 days) between diagnosis and BMT. Other disease-related variables at presentation were not significant, including WBC count > 50 x 10(9)/l, marrow blasts < 70%, time to enter remission > 40 days and > 2 courses to enter remission. Survival was 58% vs 43% for M1-M3 vs M4-M6 FAB subtypes (p = 0.03) and 71% vs 42% for low-dose vs high-dose CsA (p = 0.01). A multivariate analysis was then run separately on survival, relapse and transplant related mortality (TRM). Survival was negatively influenced by M4-M6 FAB subtypes (p = 0.009), high-dose CsA (p = 0.03) and a long interval between diagnosis and BMT (p = 0.04). Leukemia relapse was higher in patients receiving high-dose CsA (p = 0.003) and in females (p = 0.04). Transplant-related mortality was higher in FAB M4-M6 patients (p = 0.01) and patients grafted late after diagnosis (p = 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Allogeneic bone marrow transplantation for acute myeloid leukemia in first complete remission: the effect of FAB classification and GVHD prophylaxis. 819 67

Hematopoetic stem cell transplantation (SCT) often represents a unique opportunity for curing children with leukemia. Nevertheless, selecting the patient who could really benefit from this procedure remains a controversial issue. The current consensus is as follows: About 20% of children with ALL can be defined as high-risk patients by criteria such as t(9;22), t(4;11), no complete remission at day 42, poor prednisone response, and T-immunophenotype or pre-pre B-ALL, myeloid markers or more than 100,000 white blood cells/microliter. This high-risk group is eligible for alloBMT in first remission, provided a family-matched donor is available. At relapse the majority of patients will benefit from alloBMT, and alternative donor sources can be considered in high-risk patients. Only early alloBMT relapses (up to 6 months after end of initial therapy) are sure candidates, whereas late relapses, especially extramedullary sites, may equally benefit from an intensive conventional relapse treatment. However, any alloBMT relapse beyond second remission should be transplanted with allogeneic stem cells (bone marrow or peripheral stem cells). In particular, family mismatched donors or matched unrelated donors may be acceptable in high-risk cases beyond first remission. In contrast, ASCR in ALL seems not to be superior to conventional therapy. In AML the standard-risk patient, defined by criteria such as FAB M1/M2-Auer rods positive, all FAB M3, and FAB M4, is not a candidate for SCT in first remission. Patients presenting other criteria or more than 5% of blasts in the bone marrow at day 15 are at high risk in first remission and should be considered for allo BMT if a family matched donor is available. ASCR in first remission AML remains a controversial issue. In contrast, in second remission alloBMT as well as ASCR are superior to conventional chemotherapy.
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PMID:The present role of bone marrow and stem cell transplantation in the therapy of children with acute leukemia. 938 54

A 52-year-old female underwent autologous BMT because of acute myeloid leukaemia FAB M4 in second remission. Since the patient had no HLA-identical sibling she received a purged autologous BM transplant. On day +5 she developed signs of a sepsis syndrome with fluid retention and was treated with broad-spectrum antibiotic therapy. However, her body weight remained high, ascites and an increase of total serum bilirubin and alkaline phosphatase developed. The icterus worsened to a total bilirubin level of 25 mg/100 ml. Sonographic and endoscopic imaging showed a dilated gall bladder but disclosed a post-hepatic cause for the icterus. A transjugular liver biopsy on day +71 revealed severe cholestasis and siderosis. The patient remained aplastic with constantly increased bilirubin levels. On day +73 septic shock syndrome occurred and the patient died of multiorgan failure 3 days later. At autopsy, a highly differentiated bile duct adenocarcinoma at the porta hepatis, so-called Klatskin tumour, was found, explaining the fatal course with intractable cholestasis.
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PMID:Bile duct adenocarcinoma mimicking veno-occlusive disease after autologous bone marrow transplantation for acute leukaemia. 967 64