WALDENSTRÖM MACROGLOBULINEMIA - A STATE-OF-THE-ART REVIEW: PART 1: EPIDEMIOLOGY, PATHOGENESIS, CLINICOPATHOLOGIC CHARACTERISTICS, DIFFERENTIAL DIAGNOSIS, RISK STRATIFICATION, AND CLINICAL PROBLEMS

Main Article Content

Michele Bibas
Shayna Sarosiek
Jorge J. Castillo

Keywords

Amyloidosis, Anemia, B cells, BCL2, Bruton tyrosine kinase, Bing-Neel syndrome, Bruton Tyrosine Kinase Inhibitors, Cryoglobulinemia, CXCR4, flow cytometry, hyperviscosity, lymphoplasmacytic lymphoma, MYD88, Waldenström Macroglobulinemia, Peripheral neuropathy.

Abstract

Waldenström macroglobulinemia (WM) is an infrequent variant of lymphoma, classified as a B-cell malignancy identified by the presence of IgM paraprotein, infiltration of clonal, small lymphoplasmacytic B cells in the bone marrow, and the MYD88 L265P mutation, which is observed in over 90% of cases. The direct invasion of the malignant cells into tissues like lymph nodes and spleen, along with the immune response related to IgM, can also lead to various health complications, such as cytopenias, hyperviscosity, peripheral neuropathy, amyloidosis, and Bing-Neel syndrome. Chemoimmunotherapy has historically been considered the preferred treatment for WM, wherein the combination of rituximab and nucleoside analogues, alkylating drugs, or proteasome inhibitors has exhibited notable efficacy in inhibiting tumour growth. Recent studies have provided evidence that Bruton Tyrosine Kinase inhibitors (BTKI), either used independently or in conjunction with other drugs, have been shown to be effective and safe in the treatment of WM. The disease is considered to be non-curable, with a median life expectancy of 10 to 12 years.

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