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Hematopoietic SCT for the Black African and non-Black African variants of sickle cell anemia.

Abstract：

:Sickle cell anemia (SCA) remains associated with high risks of morbidity and early death. Allogeneic hematopoietic SCT (HSCT) is the only curative treatment for SCA. We report our experience with transplantation in a group of patients with the non-Black African variant and the Black African variant of SCA. This study included 40 consecutive SCA patients (13 patients with the non-Black African variant and 27 with the Black African variant) who underwent BM transplantation from HLA-identical sibling donors between June 2004 and May 2013, following a myeloablative-conditioning regimen. All patients obtained sustained engraftment. One patient (non-Black African variant) became a stable mixed chimera with 25% donor cells more than 6 years after transplantation. The probabilities of survival, SCA-free survival and TRM at 5 years after transplant were 91%, 91% and 9%, respectively. All surviving patients remained free of any SCA-related events after transplantation. Our results confirm that it is possible to offer a greater than 90% chance of cure to children with SCA. HSCT should be considered the standard of care for who have an HLA-identical donor, before complications result from the sickling of RBC.

journal_name

Bone Marrow Transplant

journal_title

Bone marrow transplantation

authors

Lucarelli G,Isgrò A,Sodani P,Marziali M,Gaziev J,Paciaroni K,Gallucci C,Cardarelli L,Ribersani M,Alfieri C,De Angelis G,Armiento D,Andreani M,Testi M,Amato A,Akinyanju OO,Wakama TT

doi

10.1038/bmt.2014.167

subject

Has Abstract

pub_date

2014-11-01 00:00:00

pages

1376-81

issue

11

eissn

0268-3369

issn

1476-5365

pii

bmt2014167

journal_volume

49

pub_type

杂志文章

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