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Rev Assoc Med Bras (1992) ; 62 Suppl 1: 16-22, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27982320

RESUMO

Sickle-cell diseases are the most common inherited hemoglobinopathies worldwide. Improvement in survival has been seen in the last decades with the introduction of careful screening and prevention of complications and the introduction of hydroxyurea. Stem-cell transplantation is currently the only curative option for these patients and has been indicated for patients with neurological events, repeated vaso-occlusive crisis, any organ damage or presence of red blood cell antibodies. Related bone-marrow or cord-blood transplant has shown an overall survival of more than 90% with a disease-free survival of 90% in 1,000 patients transplanted in the last decades. The use of unrelated donors unfortunately has not shown the same good results, but better typing methods and improved support may improve the outcome with this source of stem cells in the future. In Brazil, only recently stem cell transplant from related donors has been included in the procedures performed in the public health system. The use of related bone marrow or cord blood and a myeloablative conditioning regimen are considered standard of care for patients with sickle-cell diseases. Transplants with non-myeloablative regimens, unrelated donors or haploidentical donors should be performed only in controlled clinical trials.


Assuntos
Anemia Falciforme/cirurgia , Transplante de Medula Óssea/métodos , Brasil , Transplante de Células-Tronco de Sangue do Cordão Umbilical/métodos , Intervalo Livre de Doença , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Agonistas Mieloablativos/uso terapêutico , Programas Nacionais de Saúde , Índice de Gravidade de Doença , Condicionamento Pré-Transplante/métodos , Transplante Homólogo/métodos
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