Bone Marrow Transplant (BMT) is the Main Cureavailable for Thalassamia

Author

Pediatric Hematologist Oncologist and BMT Flowship Non-Communicable Pediatrics Diseases Research Center, Babol University of Medical Sciences, Babol, Iran.

Abstract

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.
 

Keywords