Guidelines on the management of Waldenström macroglobulinemia (WM) were published on January 12, 2022 by the British Society for Haematology (BSH), in the British Journal of Haematology.
Sequential monitoring of IgM/monoclonal protein should be performed in a single laboratory using a single methodology.
Tests for neuropathy, cryoglobulinemia, amyloidosis, cold agglutinins, bleeding diathesis, and central nervous system disease should be tailored to the clinical scenario.
Screening for hepatitis B and C and human immunodeficiency virus (HIV) is required prior to the introduction of treatment.
Bone marrow aspirate and trephine biopsy is needed to make a definitive diagnosis of WM and is recommended in all patients with suspected symptomatic WM or other IgM disorder.
Flow cytometry is the preferred method for establishing B-cell immunophenotype.
In all patients undergoing bone marrow assessment, MYD88 L265P should be assessed using an assay of established sensitivity.
Assessment of CXCR4 and TP53 should be considered and should be performed prospectively in all clinical trials.
CT scan of the neck, chest, abdomen, and pelvis is recommended in all patients prior to the commencement of each line of therapy.
Dexamethasone, rituximab, and cyclophosphamide (DRC), bendamustine and rituximab (BR), bortezomib-based regimens (bortezomib, cyclophosphamide, and rituximab [BCR] and bortezomib, dexamethasone, and rituximab [BDR]) and Bruton tyrosine kinase (BTK) inhibitors are all acceptable first-line treatments.
Given the risk of IgM flare, careful monitoring of all patients receiving rituximab is required with monitoring of sequential IgM levels, clinical assessment for hyperviscosity syndrome, and monitoring of plasma viscosity if available. The introduction of rituximab should be deferred (or prophylactic plasmapheresis performed) in patients considered at a higher risk of hyperviscosity, as defined by an IgM/M-protein >40 g/L.
For more information, please go to Waldenstrom Macroglobulinemia.
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Cite this: Waldenstrom Macroglobulinemia Clinical Practice Guidelines (BSH, 2022) - Medscape - Feb 02, 2022.