Year : 2018 | Volume
: 9 | Issue : 4 | Page : 120--125
Hematopoietic stem cell transplantation in children with hemoglobinopathies: A single-center experience
Muhammad Matloob Alam1, Mohamed Salaheldin Bayoumy2, Ibraheem Faisal Abosoudah2, Reem Zabani3, Hassan Ali Al-Trabolsi2
1 Department of Oncology, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
2 King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia
3 Ibn Sina National College, Jeddah, Saudi Arabia
INTRODUCTION: Hematopoietic stem cell transplantation (HSCT) is a curative treatment for children with serious hematological disorders including thalassemia major (TM) and sickle cell disease (SCD). This study aimed to determine the frequencies of transplant-related morbidity and outcome in our tertiary health-care facility.
MATERIALS AND METHODS: We retrospectively analyzed the clinical, laboratory, and outcome data of 32 cases of hemoglobinopathies (TM = 19 and SCD = 13) who underwent bone marrow (BM) transplantation between 2005 and 2016. SPSS was used for statistical analysis, and Kaplan–Meier curve was constructed for overall survival (OS) and event-free survival.
RESULTS: The median age of the study population at the time of transplant was 7.8 (range 2–14 years). Male-to-female ratio was 1:1. All patients were received allogeneic full matched related donor transplantation. Conditioning regimen was Bu/Cy/antithymocyte globulin (ATG) for SCD and Flu/Bu/Cy/ATG for TM. Source of stem cell in all patients was bone marrow and median stem cell dose CD 34+ was 5.26 (range 2.9–9.9) × 106/kg. All patients were received cyclosporin and methotrexate for graft versus host disease (GVHD) prophylaxis. Acute and chronic GVHD were observed in 10 (31.25%) and 2 (6.3%) cases, respectively. The median time to absolute neutrophil count (ANC) engraftment was 22 days (range: 12–27), and the median time to platelet engraftment was 32 days (range: 18–45) in 28 patients and the remaining four patients had delayed platelet engraftment (62–90 days). Cytomegalovirus (CMV) reactivation was observed in 18 (56.3%) cases; majority were asymptomatic (n = 17/18; 94.4%). Three-year event-free survival (EFS) and OS were 90.6% and 93.7%, respectively, with median duration of follow-up 33.72 (± 25.3) months.
CONCLUSION: The results of the pediatric HSCT for hemoglobinopathies at our institution are excellent and are comparable to published reports from international collaborative groups.
Dr. Muhammad Matloob Alam
Department of Oncology, King Faisal Specialist Hospital and Research Centre, P. O. Box 40047, Jeddah 21499
|How to cite this article:|
Alam MM, Bayoumy MS, Abosoudah IF, Zabani R, Al-Trabolsi HA. Hematopoietic stem cell transplantation in children with hemoglobinopathies: A single-center experience.J Appl Hematol 2018;9:120-125
|How to cite this URL:|
Alam MM, Bayoumy MS, Abosoudah IF, Zabani R, Al-Trabolsi HA. Hematopoietic stem cell transplantation in children with hemoglobinopathies: A single-center experience. J Appl Hematol [serial online] 2018 [cited 2019 Apr 25 ];9:120-125
Available from: http://www.jahjournal.org/article.asp?issn=1658-5127;year=2018;volume=9;issue=4;spage=120;epage=125;aulast=Alam;type=0