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Common treatment regimes

Most patients with symptomatic WM will received a combination of rituximab plus chemotherapy. The exact choice of regimen depends on multiple factors, including the level of bone marrow involvement, whether there is hyperviscosity or neuropathy, the age and fitness of the patient, and if autologous stem cell transplantation is planned.

The most commonly used regimens are:

Bendamustine-rituximab (BR)

This regimen consists of intravenous bendamustine on days 1 and 2 of each cycle, with rituximab also given on day one. The treatment is repeated every four weeks (a ‘cycle’) 4-6 times. The dose of bendamustine is adjusted according to age and kidney function. If the IgM level is high, the rituximab may be delayed until it has come down somewhat. This is because the rituximab can cause the IgM to increase at first (called IgM flare).

DRC

This regimen consists of intravenous dexamethasone on day 1, intravenous rituximab on day 1, and oral cyclophosphamide twice daily for the first five days (days 1-5). This is repeated every 21 days (a ‘cycle’) for a total of six courses.

Single-agent rituximab

This regimen consists of intravenous rituximab every week, usually for four doses. This may be repeated after three months.