Pediatric Hematologist Oncologist and BMT Flowship Non-Communicable Pediatrics Diseases Research Center, Babol University of Medical Sciences, Babol, Iran.
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.
Mahmoodi Nesheli, H. (2014). Bone Marrow Transplant (BMT) is the Main Cureavailable for Thalassamia. Journal of Pediatric Perspectives, 2(2.3), 24-24. doi: 10.22038/ijp.2014.2673
MLA
Mahmoodi Nesheli, H. . "Bone Marrow Transplant (BMT) is the Main Cureavailable for Thalassamia", Journal of Pediatric Perspectives, 2, 2.3, 2014, 24-24. doi: 10.22038/ijp.2014.2673
HARVARD
Mahmoodi Nesheli, H. (2014). 'Bone Marrow Transplant (BMT) is the Main Cureavailable for Thalassamia', Journal of Pediatric Perspectives, 2(2.3), pp. 24-24. doi: 10.22038/ijp.2014.2673
CHICAGO
H. Mahmoodi Nesheli, "Bone Marrow Transplant (BMT) is the Main Cureavailable for Thalassamia," Journal of Pediatric Perspectives, 2 2.3 (2014): 24-24, doi: 10.22038/ijp.2014.2673
VANCOUVER
Mahmoodi Nesheli, H. Bone Marrow Transplant (BMT) is the Main Cureavailable for Thalassamia. Journal of Pediatric Perspectives, 2014; 2(2.3): 24-24. doi: 10.22038/ijp.2014.2673