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2015 ; 5
(3
): e394
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Waldenstrom macroglobulinemia: prognosis and management
#MMPMID25815903
Oza A
; Rajkumar SV
Blood Cancer J
2015[Mar]; 5
(3
): e394
PMID25815903
show ga
Waldenstrom macroglobulinemia (WM) is a B-cell lymphoplasmacytic lymphoma
characterized by monoclonal immunoglobulin M protein in the serum and
infiltration of bone marrow with lymphoplasmacytic cells. Asymptomatic patients
can be observed without therapy. First-line therapy should consist of the
monoclonal anti-CD20 antibody, rituximab, given typically in combination with
other agents. We prefer dexamethasone, rituximab, cyclophosphamide (DRC) as
initial therapy for most patients with symptomatic WM. Other reasonable options
are bortezomib, rituximab, dexamethasone (BoRD) or bendamustine plus rituximab
(BR). All of these regimens are associated with excellent response and
tolerability. Initial therapy is usually administered for 6 months, followed by
observation. Response to therapy is assessed using the standard response criteria
developed by the International Working Group on Waldenstrom macroglobulinemia.
Relapse is almost inevitable in WM but may occur years after initial therapy. In
symptomatic patients relapsing more than 1-2 years after initial therapy, the
original treatment can be repeated. For relapse occurring sooner, an alternative
regimen is used. In select patients, high-dose chemotherapy followed by
autologous hematopoietic cell transplantation may be an option at relapse.
Options for therapy of relapsed WM besides regimens used in the front-line
setting include ibrutinib, purine nucleoside analogs (cladribine, fludarabine),
carfilzomib and immunomodulatory agents (thalidomide, lenalidomide).
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