HSCT FOR HEMOGLOBINOPATHIES (2022 UPDATE)
نویسندگان
چکیده
In 2021 the Brazilian society of stem cell transplantation and therapy published consensus guidelines regarding hematopoietic (HSCT) for hemoglobinopathies. No changes have been added thalassemia. HSCT with a matched sibling donor (MSD) or related cord blood is treatment choice young patients transfusion dependent Matched unrelated haploidentical HSCT, using bone marrow graft, are clinical option. For sickle disease (SCD), conditioning regimen should be myeloablative < 16 years old. adults, fludarabine, busulfan ATG safe effective regimen. The chemo-free alemtuzumab TBI, pioneered by NIH group, was successfully reproduced other centers good option adults established organ damage. Haploidentical post-transplant cyclophosphamide showed high rejection rates in beginning but improvements were performed, including use pre-transplant immunosuppression, increase TBI dose from 2 cGy to 4 addition thiotepa, which significantly reduced rates. We consider transplant as children significant neurological alteration indications. optimal timing SCD MSD not well established. Previous international reports excellent outcomes children. risk grade 2-4 acute graft-versus-host (GVHD) chronic GVHD higher older than 15 Despite potential complications (GVHD, gonadal dysfunction), at an earlier age may prevent dysfunction, strokes, iron overload improve patients’ quality life. Therefore, early referral strongly recommended. medical dilemma patients, will assessment damage transplantation. course extremely variable no validated genetic score has so far. Most scores phenotypic characteristics together laboratory biomarkers imaging parameters define SCD.
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ژورنال
عنوان ژورنال: Journal of Bone Marrow Transplantation and Cellular Therapy
سال: 2023
ISSN: ['2675-374X']
DOI: https://doi.org/10.46765/2675-374x.2023v4n1p183