Waldenström macroglobulinaemia

Abstract

Summary Over time, Waldenström macroglobulinaemia (WM) has evolved conceptually from a clinical syndrome to a distinct clinicopathological entity. Progress is being made in standardization of the disease definition and treatment response criteria, although nosologic controversies persist. According to the Second International Workshop on WM, the disease is defined as a B‐cell neoplasm characterized by a lymphoplasmacytic infiltrate in the bone marrow, with an associated immunoglobulin (Ig) M paraprotein. Disease symptoms are often divided into those related to tumour infiltration and those related to the rheological effects of the monoclonal IgM. As with other low‐grade lymphomas, asymptomatic patients are observed only, with treatment reserved for symptomatic patients. There is no standard treatment for WM and choices include rituximab, alkylating agents, purine nucleoside analogues, alone or in combination, as well as autologous peripheral blood stem cell transplant in eligible patients. Novel treatments, such as bortezomib, oblimersen sodium, perifosine and others are being evaluated.

Hyperviscosity Syndrome Provoking Heart Failure in a Patient with Waldenström macroglobinemia

“…The diagnosis is based on the fulfillment of the following criteria presence of IgM monoclonal gammopathy (of any size) in the serum; greater than 10% infiltration by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation (lymphoplasmacytic features or lymphoplasmacytic lymphoma) with an intertrabecular pattern, on bone marrow biopsy; expression of typical immunophenotypes on bone marrow biopsy (e.g., surface IgM+, CD5-, CD10-, CD11c-, CD19+, CD20+, CD22+, CD23-, CD25+, CD27+, FMC7+, CD103-, CD138-). The plasmacytic component will be CD138+, CD38+ and CD45-or dim [14,15].…”

CDDO-imidazolide mediated inhibition of malignant cell growth in Waldenström macroglobulinemia

“…Treatment of WM patients is determined by the presence of disease related symptoms. The most recent recommendations for therapy include the use of combination therapy with nucleoside analogues and alkylating agents, rituximab with nucleoside analogues or combination therapy such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) [2,3,6]. However, even though patients may initially respond to treatment, they invariably relapse and identification of novel therapeutic agents is clearly needed.…”

“…WM is a B-cell disorder with a highly variable clinical outcome. Clinical symptoms vary and include the infiltration of lymphoplasmacytic cells into the bone marrow, the production of a monoclonal IgM protein, and associated symptoms such as anemia, lymphadenopathy and serum hyperviscosity [1][2][3][4][5]. Treatment of WM patients is determined by the presence of disease related symptoms.…”

Stem cell transplant for Waldenström macroglobulinemia: an underutilized technique

“…The BM pattern is predominantly intertrabecular. 2 The median age at diagnosis is 64 years (range, 30--88 years), and 53% of patients are aged 65 years or younger. 3 The median survival from the time of diagnosis is 6.4 years, and the median disease-specific survival is 11.2 years.…”

“…6 Waldenströ m pathophysiology is divided into morbidities caused by the tumor mass and those caused by the monoclonal protein (Table 1). 2 The disease is incurable with current therapies. This review is designed to summarize the data in support of autologous and allogeneic stem cell transplants in the management of this lymphoma.…”