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2014; 60(5):490-499

A concentração do sCD27 e a avaliação do valor de

cadeias leves livres monoclonais apresentam-se como po-

tenciais biomarcadores para a monitoração laboratorial

do tratamento.

75,76

A investigação de biomarcadores al-

ternativos é essencial para uma avaliação clínica mais con-

fiável e menos invasiva.

ummary

Waldenström’s macroglobulinemia: a review

Waldenström’s macroglobulinemia (WM) is a lympho-

proliferative disease of B lymphocytes, characterized by

a lymphoplasmocytic lymphoma in the bone marrow and

by IgM monoclonal hypergammaglobulinemia. It was

first described in 1944 by Jan Gösta Waldenström, repor-

ting two patients with oronasal bleeding, lymphadeno-

pathy, anemia, thrombocytopenia, high erythrocyte sedi-

mentation rate and serum viscosity, normal radiography

and bone marrow infiltrated by lymphoid cells.

The WM is a rare disease with a typically indolent clini-

cal course, affecting mainly individuals aged between 63

and 68 years. Most patients have clinical signs and symp-

toms related to hyperviscosity resulting from IgMmono-

clonal gammopathy, and/or cytopenias resulting from

bone marrow infiltration by lymphoma. The differential

diagnosis with other lymphomas is essential for the as-

sessment of prognosis and therapeutic approach.

Treatment of patients with asymptomatic WM does not

improve the quality of life of patients, or increase their

survival, being recommended, therefore, their follow-up.

For the treatment of symptomatic patients, alkylating

agents, purine analogs and anti-CD20 monoclonal anti-

bodies are used. However, the disease is incurable and the

response to therapy is not always favorable. Recent stu-

dies have shown promising results with bortezomib, an

inhibitor of proteasomes, and some patients respond to

thalidomide. In patients with relapse or refractory to the-

rapy, autologous transplantation may be indicated.

The aim of this paper is to describe in detail the current

knowledge on the pathophysiology of WM, main clinical

manifestations, diagnosis, prognosis and treatment.

Keywords:

Waldenström’s macroglobulinemia, hyper-

gammaglobulinemia, IgM, lymphocytes B. prognosis.

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