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Current global scenario of guidelines on the management of paroxysmal nocturnal hemoglobinuria: a systematic literature review

de Almeida Soares, Rodolfo Daniel ; Belintani Blum Fonseca, Patricia ; Yotsumoto Fertrin, Kleber ; Marinato, André ; Lima Melo, Andresa ; Kneese Nascimento, Ana Clara ; Bunn Moreno, Adrienne ; Vecina, Antonianna ; Vieira Dias, Bruno ; Loureiro Lima, Carlos Bernardo ; Vieira Pericole de Souza, Fernando ; Oliveira Cantadori, Lucas ; Pontes Reis, Marcos Laercio ; Roberto Ganacini, Paulo ; Helman, Ricardo

Jornal Brasileiro de economia da saúde, 2020-12, Vol.12 (3), p.281-290

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  • Título:
    Current global scenario of guidelines on the management of paroxysmal nocturnal hemoglobinuria: a systematic literature review
  • Autor: de Almeida Soares, Rodolfo Daniel ; Belintani Blum Fonseca, Patricia ; Yotsumoto Fertrin, Kleber ; Marinato, André ; Lima Melo, Andresa ; Kneese Nascimento, Ana Clara ; Bunn Moreno, Adrienne ; Vecina, Antonianna ; Vieira Dias, Bruno ; Loureiro Lima, Carlos Bernardo ; Vieira Pericole de Souza, Fernando ; Oliveira Cantadori, Lucas ; Pontes Reis, Marcos Laercio ; Roberto Ganacini, Paulo ; Helman, Ricardo
  • É parte de: Jornal Brasileiro de economia da saúde, 2020-12, Vol.12 (3), p.281-290
  • Descrição: Objective: To report the outcomes of a systematic literature review of guidelines and consensus on the management of paroxysmal nocturnal hemoglobinuria (PNH) and describe the main therapeutic options available worldwide. Methods: A systematic literature review was conducted in April 2018 with no time limit and reported in line with the PRISMA statement. The AGREE II instrument was used to determine the quality of each guideline included in the systematic review. Results: Eight guidelines/consensus were eligible, one developed by an international group, two in Spain, and one each in Turkey, Germany, Argentina, Australia and the United Kingdom. Supportive treatment with erythrocyte transfusion, anticoagulants and steroids is indicated by all guidelines and consensus. The use of erythropoietin is suggested by three of them. Recommendations for the prescription of eculizumab were consistent in all but one guideline, published in 2005. Allogeneic hematopoietic stem cell transplantation is reported as the only potentially curative treatment for PNH, although its association with high mortality and morbidity rates is emphasized, being indicated for a selected group of patients. The AGREE II scores applied for each domain showed in general a low and heterogeneous methodological quality among guidelines. Conclusion: Despite the low and heterogeneous methodological quality, in general the comparison of guidelines and consensus for PNH management showed consistent recommendations regarding supportive care, eculizumab and hematopoietic stem cell transplantation.
  • Idioma: Inglês
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  • Realização: ABCD-USP USP   Logos de Redes Sociais: Freepik

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