Allogenic bone narrow transplantation in sickle-cell diseases.

• Autor(a) principal: Simões,Belinda Pinto
• Data de Publicação: 2016
• Outros Autores: Pieroni,Fabiano, Costa,Thalita, Barros,George Navarro, Darrigo Jr.,Guilherme, Grecco,Carlos Settani, Bernardes,Juliana Elias, Moraes,Daniela, Stracieri,Ana Beatriz Lima, Cunha,Renato, Rodrigues,Maria Carolina
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Revista da Associação Médica Brasileira (Online)
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0104-42302016001200016
• topic: Sickle Cell Disease; Stem Cell Transplantation; Hemoglobinopathies

SUMMARY 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.