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First report of successful stem cell transplantation in a patient with sickle cell hemoglobin D disease.

Authors :
Hamidieh AA
Jalili M
Khojasteh O
Ghavamzadeh A
Source :
Journal of pediatric hematology/oncology [J Pediatr Hematol Oncol] 2010 Jul; Vol. 32 (5), pp. 397-9.
Publication Year :
2010

Abstract

Sickle cell hemoglobin D disease is a rare variant of sickle cell disease. Affected patients suffer from episodes of acute exacerbation of clinical course with a wide range of manifestations such as acute chest syndrome, stroke, painful vaso-occlusive crises, acute sequestration crises, joint necrosis, organ failure, infections, and temporary aplastic crises, collectively called sickling crises. Conventional treatments for patients with sickle cell disease include hydroxyurea therapy and prophylactic red blood cell transfusion. However, morbidity and mortality rates remain high with these remedies. In this article, we report hematopoietic stem cell transplantation as an alternative treatment in children with high-risk factors. According to our knowledge and an extensive review of the literature, stem cell transplantation in sickle cell hemoglobin D disease previously has not been reported in any published study and our patient is the first case.

Details

Language :
English
ISSN :
1536-3678
Volume :
32
Issue :
5
Database :
MEDLINE
Journal :
Journal of pediatric hematology/oncology
Publication Type :
Academic Journal
Accession number :
20502357
Full Text :
https://doi.org/10.1097/MPH.0b013e3181df614b