Set - Out - English - 2014

102 / 111

Coimbra S et al.
490 Rev Assoc Med Bras 2014; 60(5):490-499
Review article
Waldenström’s macroglobulinemia – a review
Susana Coimbra1,2*, Rafael Neves3, Margarida Lima4, Luís Belo1,3, Alice Santos-Silva1,3
1Institute of Molecular and Cellular Biology (IBMC), University of Porto, Porto, Portugal.
2CESPU, Institute for Research and Advanced Training in Health Sciences and Technologies, Gandra-PRD, Portugal.
3Department of Biological Sciences, Biochemistry Laboratory, Faculty of Pharmacy, University of Porto, Porto, Portugal.
4Santo António General Hospital, Centro Hospitalar of Porto, Porto, Portugal.
Summary
Study conducted at the University of Porto,
Porto, Portugal
Article received: 6/4/2013
Accepted for publication: 1/30/2014
*Correspondence:
Instituto de Biologia Molecular e Celular
(IBMC), Universidade do Porto
Address: Rua do Campo Alegre, 823
Postal Code: 4150
Porto – Portugal
Phone: 351 962677495
Conflict of interest: none
Waldenström’s macroglobulinemia (WM) is a lymphoproliferative disease of B
lymphocytes, characterized by a lymphoplasmocytic lymphoma in the bone mar-
row and by IgMmonoclonal hypergammaglobulinemia. It was first described in
1944 by Jan Gösta Waldenström, reporting two patients with oronasal bleeding,
lymphadenopathy, anemia, thrombocytopenia, high erythrocyte sedimentation
rate and serum viscosity, normal radiography and bone marrow infiltrated by
lymphoid cells.
The WM is a rare disease with a typically indolent clinical course, affecting mainly
individuals aged between 63 and 68 years. Most patients have clinical signs and
symptoms related to hyperviscosity resulting from IgMmonoclonal gammopa-
thy, and/or cytopenias resulting from bone marrow infiltration by lymphoma.
The differential diagnosis with other lymphomas is essential for the assessment
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, puri-
ne analogs and anti-CD20 monoclonal antibodies are used. However, the disea-
se 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 refrac-
tory to therapy, autologous transplantation may be indicated.
The aim of this paper is to describe in detail the current knowledge on the pa-
thophysiology of WM, main clinical manifestations, diagnosis, prognosis and
treatment.
Keywords: Waldenström’s macroglobulinemia, hypergammaglobulinemia, IgM,
lymphocytes B. prognosis.
Introduction
Waldenström’s macroglobulinemia (WM), described in
1944 by Jan Gösta Waldenström, is a lymphoplasmacytic
lymphoma (LPL) characterized by IgM monoclonal hy-
pergammaglobulinemia and bone marrow infiltration.1
LPLs are rare and indolent cancers of mature B-lym-
phocytes, which predominantly involve the bone marrow
and, less commonly, the spleen, lymph nodes, peripheral
blood and other organs.1
Epidemiology
WM has an estimated incidence of 3 cases/million/year,
accounting for about 2% of all hematological cancers.2
There is a higher incidence in individuals aged between
63 and 68 years.3 Approximately 60% of patients are men,
and it is more common in caucasian indivuals.3 The ave-
rage survival is 5 years,3 however, approximately 10% of
patients survive up to 15 years. 4 As the disease is mainly
diagnosed in old age, about 50% of patients die due to
comorbidities not related directly to WM.3
Etiology
Its etiology is unknown, but several studies suggest a pos-
sible causal relationship with autoimmune diseases, ex-
posure to environmental factors and chronic antigenic