Hemofilia A Adquirida - Caso Clínico
Palavras-chave:
Hemofilia A adquirida, factor VII activado recombinante, discrasia hemorrágica adquiridaResumo
A hemofilia A adquirida (HA) é uma doença rara mas potencialmente grave que resulta da presença em circulação de anticorpos
dirigidos para diversos domínios da molécula de factor VIII. O
quadro clínico difere significativamente dos com hemofilia A
congénita, sendo caracterizado por hematomas e hemorragias
dos tecidos moles, hemorragia gastrintestinal e hematúria e só raramente hemartroses.1,2
Apresentamos o caso clínico de um homem de 66 anos,
anteriormente saudável, internado por discrasia hemorrágica.
Detectaram-se anticorpos anti-factor VIII. O doente foi submetido
a terapêutica de substituição com factor VII recombinante activado
(rFVIIa) e terapêutica de imunosupressão/modulação com corticóides e gamaglobulina com erradicação bem sucedida do anticorpo
circulante em cerca de quatro semanas. Revemos ainda os dados
actuais relativos à etologia e abordagem terapêutica da HA.
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Referências
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