Waldenstrom Macroglobulinemia Clinical Practice Guidelines (2018)

European Society for Medical Oncology (ESMO)

Reviewed and summarized by Medscape editors

November 30, 2018

The guidelines on the diagnosis, treatment, and follow-up of Waldenstrom macroglobulinemia were released on October 1, 2018, by the European Society for Medical Oncology (ESMO).[1]

Diagnosis

Waldenstrom macroglobulinemia (WM) is diagnosed based on histopathologic confirmation of bone marrow (BM) infiltration by monoclonal lymphoplasmacytic cells and serum monoclonal immunoglobulin M (IgM), confirmed with immunofixation.

WM cells are typically positive for the biomarkers CD19, CD20, CD22 and CD79a.

Approximately 90% of patients with WM have the MYD88L265P mutation; this may be useful for differentiating WM from other types of lymphoma and IgM multiple myeloma.

Staging and Risk Assessment

The initial workup for WM includes a complete blood cell (CBC) count, serum chemistry, beta-2 microglobulin (B2M), serum protein electrophoresis, and IgM quantification.

Funduscopic examination is recommended in patients with hyperviscosity symptoms.

Anemia should prompt consideration of Coombs testing, cold agglutinins, cryoglobulins, and iron status.

A neurologist should be consulted for neuropathy, since it may not be associated with WM.

Amyloidosis is uncommon in patients with WM. If it is suspected, a fat aspirate stained with Congo red and cardiac and renal biomarkers should be evaluated.

The initial evaluation should include imaging studies (preferably CT or MRI).

Risk assessment is currently based on the International Prognostic Scoring System for WM (IPSSWM).

Treatment

Asymptomatic WM should be managed with watchful waiting, with monitoring every 3-6 months.

The monoclonal IgM level alone generally does not indicate treatment initiation.

Hyperviscosity syndrome should be treated with plasmapheresis for immediate relief, in combination with appropriate systemic therapy.

Treatment is indicated in patients with constitutional symptoms, cytopenias, hyperviscosity, moderate or severe neuropathy, amyloidosis, symptomatic cryoglobulinemias, and/or cold agglutinin disease.

Primary treatment options include rituximab plus alkylating agents (PO or IV cyclophosphamide or bendamustine) or proteasome inhibitors.

Monotherapy with alkylating agents, nucleoside analogues, or rituximab should be considered only in patients who are not candidates for more effective chemoimmunotherapy combinations.

Rituximab maintenance therapy is not recommended for WM.

The treatment of choice for WM relapse within 12 months of chemoimmunotherapy, including rituximab-refractory disease, is ibrutinib monotherapy.

Ibrutinib monotherapy may be considered in patients who are ineligible for chemoimmunotherapy as first-line therapy.

An alternate chemoimmunotherapy combination or a prior effective regimen or ibrutinib may be considered in patients with late WM relapses after chemoimmunotherapy.

High-dose treatment with autologous stem cell transplantation (ASCT) may be considered in selected young patients with chemosensitive relapse.

All patients with WM should be strongly encouraged to enroll in clinical trials.

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