Metabolismo do Ferro, Biomarcadores, Sobrecarga de Ferro e Quelação: Recomendações em Síndromes Mielodisplásicas
DOI:
https://doi.org/10.24950/rspmi/Revisao/225/18/3/2019Palavras-chave:
Consenso, Quelantes de Ferro, Síndromes Mielodisplásicas, Sobrecarga de Ferro, Terapia por Quelação, Transfusão de SangueResumo
As síndromes mielodisplásicas (SMD) são um grupo heterogéneo de neoplasias mielóides caracterizadas por citopenias decorrentes de hematopoiese ineficaz e por risco
aumentado de progressão para leucemia mieloide aguda.
Afetam 5/100 000 pessoas por ano em Portugal, dos quais
mais de 60% se tornará dependente de transfusões ao longo do curso de doença. As citopenias podem levar a uma
morbilidade significativa, sendo a sua gestão um dos pontos-chave para a melhoria da qualidade de vida destes
doentes. Este documento compila o resultado do debate
promovido pelo Anemia Working Group Portugal com peritos
reconhecidos sobre a abordagem de suporte dos doentes
com SMD de risco mais baixo, focando os efeitos a médio
e longo prazo das transfusões recorrentes e da sobrecarga
de ferro. Para o diagnóstico das SMD, recomenda-se a aplicação da classificação da Organização Mundial de Saúde
2016. O score International Prognosis Scoring System continua a ser a referência para definição de prognóstico e estratégia terapêutica. A terapêutica de suporte é um pilar fundamental do tratamento dos doentes em qualquer estádio
da sua doença e inclui antibióticos, transfusão de concentrado eritrocitário e plaquetas, fatores de crescimento hematopoiético, agentes antifibrinolíticos e terapêutica quelante.
Em doentes transfundidos com ≥ 20 unidades de concentrado eritrocitário ou com ferritina sérica ≥1 000 ng/mL, deve-se
considerar terapêutica com quelantes de ferro. Os doentes
candidatos a terapêutica quelante devem fazer ressonância
magnética ao fígado e pâncreas com periodicidade semestral ou anual. As opções terapêuticas devem ter em conta as
características da doença e a qualidade de vida do doente,
de acordo com as linhas orientadoras internacionais.
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