title: Allogenic bone narrow transplantation in sickle-cell diseases.
abstract: 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.
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Belinda Pinto
authors.initials: BP
authors.lastname: Simões
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Fabiano
authors.initials: F
authors.lastname: Pieroni
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Thalita
authors.initials: T
authors.lastname: Costa
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: George Navarro
authors.initials: GN
authors.lastname: Barros
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Guilherme
authors.initials: G
authors.lastname: Darrigo
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Carlos Settani
authors.initials: CS
authors.lastname: Grecco
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Juliana Elias
authors.initials: JE
authors.lastname: Bernardes
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Daniela
authors.initials: D
authors.lastname: Moraes
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Ana Beatriz Lima
authors.initials: AB
authors.lastname: Stracieri
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Renato
authors.initials: R
authors.lastname: Cunha
authors.affiliation: Transplantation Unit of Hospital das Clínicas, FMRP, USP, Brasil.
authors.forename: Maria Carolina
authors.initials: MC
authors.lastname: Rodrigues
mesh_terms: D000755:Anemia, Sickle Cell / Q000601:surgery*; D016026:Bone Marrow Transplantation / Q000379:methods*; D001938:Brazil; D036101:Cord Blood Stem Cell Transplantation / Q000379:methods; D018572:Disease-Free Survival; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D019653:Myeloablative Agonists / Q000627:therapeutic use; D009313:National Health Programs; D012720:Severity of Illness Index; D019172:Transplantation Conditioning / Q000379:methods; D014184:Transplantation, Homologous / Q000379:methods
chemical_list: D019653:Myeloablative Agonists