Síndrome hemolítico urémico / Púrpura trombocitopénica trombótica
Palavras-chave:
Disfunção endotelial, factor de von Willebrand, factor H, plasmaférese, púrpura trombocitopénica trombótica, síndrome hemolítica urémicaResumo
A síndrome hemolítica urémica e a púrpura trombocitopénica
trombótica caracterizam-se pela presença de anemia hemolítica microangiopática e trombocitopenia, estando a disfunção
endotelial e a libertação de multímeros de factor von Willebrand
de alto peso molecular implicados na sequência de eventos que
levam à trombose microvascular. O aumento do nível sérico da
desidrogenase láctica constitui o marcador mais sensível de hemólise microangiopática.
Na síndroma hemolítica urémica os microtrombos localizam-se,
sobretudo, na vasculatura renal, constituindo a insuficiência renal
aguda a apresentação dominante. A púrpura trombocitopénica
trombótica envolve sobretudo o cérebro, com formação intermitente de microtrombos e sinais neurológicos flutuantes.
Abordam-se as principais características, quadro clínicos
prognóstico e tratamento das formas primárias e secundárias
de síndrome hemolítica urémica/ púrpura trombocitopénica
trombótica, bem como as formas agudas, crónicas, recorrentes
e resistentes à plasmaferese
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