Literature DB >> 20502357

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

Amir Ali Hamidieh1, Mahdi Jalili, Omid Khojasteh, Ardeshir Ghavamzadeh.   

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.

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Year:  2010        PMID: 20502357     DOI: 10.1097/MPH.0b013e3181df614b

Source DB:  PubMed          Journal:  J Pediatr Hematol Oncol        ISSN: 1077-4114            Impact factor:   1.289


  2 in total

1.  Pulmonary thromboembolism in a child with sickle cell hemoglobin d disease in the setting of acute chest syndrome.

Authors:  Hazel Villanueva; Sandeepkumar Kuril; Jennifer Krajewski; Aziza Sedrak
Journal:  Case Rep Pediatr       Date:  2013-09-18

2.  Hemoglobin D-Punjab: origin, distribution and laboratory diagnosis.

Authors:  Lidiane de Souza Torres; Jéssika Viviani Okumura; Danilo Grünig Humberto da Silva; Claudia Regina Bonini-Domingos
Journal:  Rev Bras Hematol Hemoter       Date:  2015-02-23
  2 in total

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