Brazilian Thalassemia Association protocol for iron chelation therapy in patients under regular transfusion.
In the absence of an iron chelating agent, patients with beta-thalassemia on regular transfusions present complications of transfusion-related iron overload. Without iron chelation therapy, heart disease is the major cause of death; however, hepatic and endocrine complications also occur. Currently there are three iron chelating agents available for continuous use in patients with thalassemia on regular transfusions (desferrioxamine, deferiprone, and deferasirox) providing good results in reducing cardiac, hepatic and endocrine toxicity. These practice guidelines, prepared by the Scientific Committee of Associação Brasileira de Thalassemia (ABRASTA), presents a review of the literature regarding iron overload assessment (by imaging and laboratory exams) and the role of T2* magnetic resonance imaging (MRI) to control iron overload and iron chelation therapy, with evidence-based recommendations for each clinical situation. Based on this review, the authors propose an iron chelation protocol for patients with thalassemia under regular transfusions.
Centro Infantil de Investigações Hematológicas Dr Domingos A Boldrini, Campinas, SP, Brazil.
Monica Pinheiro de Almeida
MP
Veríssimo
Centro de Hematologia de São Paulo - CHSP, São Paulo, SP, Brazil.
Sandra Regina
SR
Loggetto
Faculdade de Medicina de Marilia - FAMEMA, Marilia, SP, Brazil.
Antonio
A
Fabron Junior
Centro de Hematologia e Hemoterapia do Paraná - HEMEPAR, Curitiba, PR, Brazil.
Giorgio Roberto
GR
Baldanzi
Hospital Israelita Albert Einstein, São Paulo, SP, Brazil.
Nelson
N
Hamerschlak
Instituto de Ensino e Pesquisa Jose Michel Kalaf, Campinas, SP, Brazil.
Juliano Lara
JL
Fernandes
Fundação de Hematologia e Hemoterapia de Pernambuco-HEMOPE, Recife, PE, Brazil.
Aderson da Silva
Ada S
Araujo
Instituto Estadual de Hematologia Arthur de Siqueira Cavalcanti - HEMORIO, Rio de Janeiro, RJ, Brazil.
Clarisse Lopes de Castro
CL
Lobo
Universidade de Campinas - UNICAMP, Campinas, SP, Brazil.
Kleber Yotsumoto
KY
Fertrin
University of Southern California, Los Angeles, AC, United States.
Vasilios Antonios
VA
Berdoukas
University of Cagliari, Cagliari, AC, Italy.
Renzo
R
Galanello
Blood transfusion; Brazil; Chelation therapy; Deferasirox; Deferiprone; Iron chelating agents; Iron overload; Iron/metabolism; Magnetic resonance imaging; Practice guidelines as topic; Protocols; beta-Thalassemia