bone marrow transplant (bmt) is the main cureavailable for thalassamia

نویسندگان

hassan mahmoodi nesheli pediatric hematologist oncologist and bmt flowship non-communicable pediatrics diseases research center, babol university of medical sciences, babol, iran.

چکیده

introduction: the thalassamias refer to a diverse group of hemoglobin disorders characterized by a reduced synthesis of  one or more of globin chains (α,β, γ, δβ,γδβ, δ and εγδβ).the main cure available today for thalassamia is bone marrow transplantation (bmt)  from compatible donor.on december 3, 1981 a 14-month-old child with β-thalassemia major recieved bmt from his hla-identical sister in seattle.on december 17, 1981 the pesaro team performed a transplant in a 16-year old thalassamia, using marrow from his hla-identical brother.this patient rejected the graft. methods: in our study, twenty twotransfusion dependentthalassemia patients were recruited.when hla matched donor was detected, he or she was checked for asymptomatic infection, electerolytes and endocrinopathy. donors were either heterozygous for β-thalassemia or normal homozygous. results: in our center 22 transfusion dependent hemglobinopathies (10 girls and 12 boys,age3-26y, mean=15.6y) underwent to hsct from september 2010 until may  2014.graft failure happened in 3 patients. retransplantation was done for one patient who was x variant hemoglobinopathy.twenty patients were disease free and didn`t need to transfusion after bmt. although in our bmt center , few thalassemia patients underwent to transplantation, 20 of 22 were independent to blood transfusion after transplantation. one patient who underwent to retransplantation is undependent to transfusion. conclusion: patients and their family were very pleasured and satisfied. we suggest all patients with β-thalassemia who have hla-identical related donors should be transplanted as soon as possible.     keyword: bone marrow transplant, hla-identical, β-thalassemia.

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عنوان ژورنال:
international journal of pediatrics

جلد ۲، شماره ۲.۳، صفحات ۲۴-۲۴

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