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2014; 60(5):490-499

hiperviscosidade, resultante da gamopatia monoclonal IgM

e/ou com as citopenias, resultantes da infiltração medular

pelo linfoma. O diagnóstico diferencial com outros linfo-

mas é essencial para a avaliação do prognóstico e a aborda-

gem terapêutica.

O tratamento dos doentes com MW assintomática não

melhora a qualidade de vida do doente nem aumenta a

sua sobrevivência, recomendando-se o acompanhamen-

to clínico. Para o tratamento dos doentes sintomáticos,

são usados agentes alquilantes, análogos das purinas e

anticorpos monoclonais anti-CD20. No entanto, a doen-

ça é incurável e a resposta à terapêutica nem sempre é fa-

vorável. Estudos relativamente recentes mostram resul-

tados promissores com o bortezomibe, um inibidor dos

proteossomas, e alguns doentes respondem à talidomi-

da. Nos doentes com recidivas ou refratários à terapêuti-

ca, pode-se indicar o transplante autólogo.

O objetivo deste trabalho é descrever, de forma detalha-

da, o conhecimento atual sobre a fisiopatologia da MW,

as principais manifestações clínicas, o diagnóstico, o prog-

nóstico e o tratamento.

Palavras-chave:

macroglobulinemia de Waldenström;

hipergamaglobulinemia; IgM; linfócitos B; prognóstico.

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