Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria
Hematol., Transfus. Cell Ther. (Impr.)
;
43(3): 341-348, July-Sept. 2021. tab, ilus
Artículo
en Inglés
| LILACS
| ID: biblio-1346248
ABSTRACT
Paroxysmal nocturnal hemoglobinuria is a chronic, multi-systemic, progressive and lifethreatening 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.
Texto completo:
Disponible
Índice:
LILACS (Américas)
Asunto principal:
Hemoglobinuria Paroxística
Tipo de estudio:
Estudio diagnóstico
/
Guía de Práctica Clínica
/
Estudio pronóstico
/
Estudio de tamizaje
Límite:
Humanos
Idioma:
Inglés
Revista:
Hematol., Transfus. Cell Ther. (Impr.)
Asunto de la revista:
Hematologia
/
TransfusÆo de Sangue
Año:
2021
Tipo del documento:
Artículo
País de afiliación:
Brasil
Institución/País de afiliación:
Faculdade de Ciências Médicas da Santa Casa de São Paulo - FCMSCSP/BR
/
Fleury Medicina e Saúde/BR
/
Hospital de Hematologia e Hemoterapia de Pernambuco - HEMOPE/BR
/
Instituto de Hematologia Arthur de Siqueira Cavalcanti - HEMORIO/BR
/
Universidade Estadual de Campinas - UNICAMP/BR
/
Universidade Federal de São Paulo - UNIFESP/BR
/
Universidade de São Paulo - USP/BR
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