How I treat Waldenström macroglobulinemia

Abstract

Waldenströ m macroglobulinemia (WM) is a distinct B-cell disorder resulting from the accumulation, predominantly in the bone marrow, of clonally related IgMsecreting lymphoplasmacytic cells. Genetic factors play an important role, with 20% of patients demonstrating a familial predisposition. Asymptomatic patients should be observed. Patients with a disease-related hemoglobin level less than 10 g/L, platelet count less than 100 ؋ 10 9 /L, bulky adenopathy or organomegaly, symptomatic hyperviscosity, peripheral neuropathy, amyloidosis, cryoglobulinemia, cold-agglutinin disease, or evidence of disease transformation should be considered for therapy. Plasmapheresis should be considered for symptomatic hyperviscosity and for prophylaxis in patients in whom rituximab therapy is contemplated. The use of rituximab as monotherapy or in combination with cyclophosphamide, nucleoside analog, bortezomib, or thalidomide-based regimens can be considered for the first-line therapy of WM and should take into account specific treatment goals, future autologous stem cell transplantation eligibility, and long-term risks of secondary malignancies. In the salvage setting, the reuse or use of an alternative frontline regimen can be considered as well as bortezomib, alemtuzumab, and stem cell transplantation. Newer agents, such as bendamustine and everolimus, can also be considered in the treatment of WM. (Blood. 2009;114:2375-2385)

Rituximab–cyclophosphamide‐dexamethasone is highly effective in patients with monoclonal Ig deposit‐related glomerulopathy and indolent non‐Hodgkin lymphomas

“…This suggests that the best level of clonal eradication offers the best renal responses, supporting the use of rituximabchemotherapy rather than an alkylating agent or rituximab monotherapy. Of note, a combination of rituximab plus bendamustine, an alkylating agent, has emerged as a promising regimen in patients with previously untreated or refractory non-Hodgkin's lymphoma, WM or CLL [17,18], This steroid-free treatment may be an attractive alternative for some patients with iNHL-associated glomerulopathy.…”

Phase II trial of weekly bortezomib in combination with rituximab in untreated patients with Waldenström Macroglobulinemia

“…Current recommendations for frontline therapy of patients with WM include the use of alkylating agents (e.g: chlorambucil or cyclophosphamide), nucleoside analogs, (e.g fludarabine), rituximab alone, and combinations of these agents [12,[14][15][16][17][18]. Long-term exposure to chlorambucil or nucleoside analogs in patients who are eligible for stem cell transplant is not recommended [17].…”

“…Long-term exposure to chlorambucil or nucleoside analogs in patients who are eligible for stem cell transplant is not recommended [17]. In addition, recent studies have shown that nucleoside analogs can increase the risk of malignant transformation and myelodysplasia [19].…”

The role of 18F‐FDG PET/CT imaging in Waldenstrom macroglobulinemia

“…Waldenstrom macrogloblulinemia (WM) is a distinct lowgrade B-cell lymphoma characterized by the presence of a serum monoclonal IgM protein and lymphoplasmacytic cells in bone marrow [1][2][3][4]. WM is a rare cancer with 1,500 new cases diagnosed per year in the US.…”