How we manage patients with Waldenström macroglobulinaemia

Area of Science:

• Hematology
• Oncology
• Genetics

Background:

• Waldenström macroglobulinaemia (WM) is an indolent B-cell lymphoproliferative disorder.
• Characterized by bone marrow involvement and monoclonal IgM production.
• Symptoms arise from cytopenias, tumor burden, or IgM-related complications.

Purpose of the Study:

• To review diagnostic procedures for Waldenström macroglobulinaemia.
• To outline current and emerging therapeutic strategies for WM.
• To discuss the role of MYD88 mutations in WM prognosis and treatment.

Main Methods:

• Literature review of diagnostic and therapeutic approaches in WM.
• Analysis of the impact of MYD88 mutations on disease characteristics.
• Evaluation of established and novel treatment regimens.

Main Results:

• Somatic MYD88 mutations are prevalent in WM, associated with gain-of-function and improved prognosis.
• Standard first-line treatment involves anti-CD20 antibodies with chemotherapy (e.g., rituximab, cyclophosphamide, dexamethasone).
• Emerging therapies include proteasome inhibitors and Bruton tyrosine kinase (BTK) inhibitors, showing promising efficacy.

Conclusions:

• WM diagnosis relies on integrated biological and morphological assessments.
• Treatment decisions are individualized based on patient factors and disease urgency.
• Advances in understanding MYD88 mutations and novel drug development are transforming WM management.