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Type: Artigo
Title: Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria
Author: Cançado, Rodolfo D.
Araújo, Aderson da Silva
Sandes, Alex Freire
Arrais, Celso
Lobo, Clarisse Lopes de Castro
Figueiredo, Maria Stella
Gualandro, Sandra Fátima Menosi
Saad, Sara Teresinha Olalla
Costa, Fernando Ferreira
Abstract: Paroxysmal nocturnal hemoglobinuria is a chronic, multi-systemic, progressive and life-threatening disease characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. Paroxysmal nocturnal hemoglobinuria results from the expansion of a clone of hematopoietic cells that due to an inactivating mutation of the X-linked gene PIG-A are deficient in glycosylphosphatidylinositol-linked proteins. Early diagnosis, using flow cytometry performed on peripheral blood, the gold standard test to confirm the diagnosis of paroxysmal nocturnal hemoglobinuria, is essential for improved patient management and prognosis. The traditional therapy for paroxysmal nocturnal hemoglobinuria includes blood transfusion, anti-thrombosis prophylaxis or allogeneic bone marrow transplantation. The treatment that has recently become available is the complement blockade by the anti-C5 monoclonal antibody eculizumab. In this consensus, we are aiming to review the diagnosis and treatment of the paroxysmal nocturnal hemoglobinuria patients, as well as the early recognition of its systemic complications. These procedures express the opinions of experts and have been based on the best available evidence and international guidelines, with the purpose of increasing benefits and reducing harm to patients
Subject: Hemoglobinúria
Country: Brasil
Editor: Elsevier
Rights: Fechado
Identifier DOI: 10.1016/j.htct.2020.06.006
Date Issue: 2020
Appears in Collections:FCM - Artigos e Outros Documentos

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