This protocol applies to patients with Waldenstrom's macroglobulinemia / lymphoplasmacytic lymphoma (WM/LPL) who have undergone primary systemic therapy and now require a next-step treatment approach due to insufficient response or progressive disease.
Primary systemic therapy for WM/LPL — which may include regimens such as bendamustine/rituximab, bortezomib/dexamethasone/rituximab, ibrutinib with or without rituximab, or zanubrutinib, among others — targets meaningful reduction in serum IgM protein and symptom relief. When these goals are not achieved, a subsequent line of treatment is indicated.
For previously treated WM/LPL, the structured protocol defines a hierarchy of preferred regimens, other recommended options, and regimens useful in specific circumstances — spanning targeted agents, anti-CD20-based combinations, and, in selected patients, more intensive approaches. The choice among these depends on prior therapy received and individual clinical factors. The complete algorithm is available via the link below.
Response is assessed by reduction in serum IgM protein: a minor response requires at least a 25% reduction; a partial response, at least 50%; and a very good partial response, at least 90%. Symptom relief is a co-equal goal of treatment.
DOI: 10.6004/jnccn.2024.0001
All regimens listed under primary treatment options are effective options for consideration in patients with previously treated WM.
Response to therapy in WM is defined by reduction in the IgM protein. The goal of treatment is symptom relief and reducing the risk of organ damage.
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