Waldenström's macroglobulinemia (WM) is characterized by infiltration of lymphoplasmocytic cells in bone marrow and presence of IgM monoclonal gammopathy.
A consensus report (2006) indicates that chlorambucil, nucleoside analogs (fludarabin, cladribin), rituximab, and combination regimens are equal.
This disease responds less effectively to chlorambucil than chronic lymphatic leukemia, and has a prolonged disease course.
Patients should be observed untreated unless they have one of the followins symptoms or findings:
- Disease related anemia (Hb < 10 g/dl), thrombocytes < 100 x 109/L
- Enlarged lymph nodes or splenomegaly, symptomatic hyperviscosity
- Serious neuropathy, amyloidosis
- Cold agglutinin disease
- Disease transformation to aggressive B-cell lymphoma (often diffuse large cell B-cell lymphoma)
Use of combination regimens such as fludarabin-cyclophosphamide, fludarabin-rituximab, and CHOP-based chemotherapy) are considered equal to monotherapy, but data from randomized studies are lacking (Treon SP et al., Blood 2006). Bendamustin in combination with rituximab is a promising alternative (Treon SP et al Clin Lymphoma Myeloma Leuk 2011).
The same regimens are also given for recurrence.
Local radiation therapy (30 Gy) should be considered for symptomatic nodal tumors.