8 research outputs found

    Early hepatic complication in first year after bone marrow transplantation in major βthalassemia patients

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    Bone marrow transplantation in a child with type C of niemann-pick disease (second case in the world)

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    Abstract Background: Thalassemia major and its treatment by stem cell transplantation can have deleterious effects on bone integrity. This study assesses the adverse effects of transplantation on growing bones of pediatric thalassemic patients. Methods: Bone mineral density (BMD) of 20 patients from three thalassemia classes whose mean (SD) age was 7.4 (3.8) years were tested with a Norland XR-46 device at baseline (before transplantation), 6 and 12 months after transplantation. Results: At 6 and 12 months after transplantation we observed no signi cant changes in mean BMD. There were no Z-scores less than -2 among patients. Class 3 thalassemia did not negatively impact BMD. Calcium (Ca), phosphorous (P) and ferritin levels were not signi cantly related to patients' BMD scores. Transfusion duration and chelation therapy showed positive signi cant relationships to BMD (g/ cm 2 ), but no signi cant relation with the BMD Z-score. The deleterious relation between corticosteroid use and changes in BMD was not signi cant. In contrast, patients who developed acute graft versus host disease (aGVHD) after transplantation showed signi cant adverse effects on BMD of their femur (P = 0.020) and spine (P = 0.027). Conclusion: Stem cell transplantation in pediatric thalassemic patients who do not develop aGVHD does not appear to have any signicant positive or negative effects on BMD

    RESULTS OF HEMATOPOIETIC CELL TRANSPLANTATION IN PEDIATRIC LEUKEMIA

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    Hematopoietic cell transplantation (HCT) is an accepted treatment for acute myeloid leukemia (AML) in first remission, the treatment of choice for chronic myeloid leukemia (CML) and high risk groups of ALL who relapse with conventional chemotherapy. We assessed results of HCT for pediatric leukemia in our center. A total of 92 children, 63 with diagnose of AML, 23 with ALL and 6 with CML received allogeneic transplantation from HLA full matched siblings (57.6%) and autologous transplantation (42.4%). Source of hematopoietic cells were peripheral blood 83.7%, bone marrow 15.2% and cord blood 1.6%. The median transplanted nucleated cells were 6.4 ± 4.7 ×108 /Kg (body weight of patients) and mononuclear cells were 5.5 ± 2.9×108/Kg. The most common conditioning regimens were cyclophosphamide + busulfan. Prophylaxis regimen for GVHD was cyclosporin ± methotrexate. GVHD occurred in 50 (54.3%) patients. Eighty five of children had engraftment, 26 (28.6%) relapsed and 57 (62%) are alive. The most common cause of death was relapse (68.6%). Five years overall survival of patients with AML and ALL were 49% and 44% respectively and disease free survival of them were 52% and 49%. One year overall survival and disease free survival of CML was 57%. Overall survival increased with increasing age of patients at transplantation time (P = 0.06). Longer survival significantly related to earlier WBC and platelet recovery (P < 0.0001 and P = 0.006 respectively). Considering acceptable overall and disease free survival of patients after HCT, we concluded that is a good modality in treatment of leukemia of children
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