TY - CHAP M1 - Book, Section TI - Macroglobulinemia A1 - Castillo, Jorge J. A1 - Treon, Steven P. A2 - Kaushansky, Kenneth A2 - Prchal, Josef T. A2 - Burns, Linda J. A2 - Lichtman, Marshall A. A2 - Levi, Marcel A2 - Linch, David C. PY - 2021 T2 - Williams Hematology, 10e AB - SUMMARYWaldenström macroglobulinemia (WM) is an indolent B-cell neoplasm manifested by the accumulation of clonal immunoglobulin (Ig) M secreting lymphoplasmacytic cells. MYD88 and CXCR4 somatic mutations are present in more than 90% and 30% to 35% of WM patients, respectively, and affect disease presentation, treatment outcome, and/or overall survival. Familial predisposition is common in WM. Asymptomatic patients should be observed. Patients with disease-related hemoglobin of less than 100 g/L, platelets less than 100 × 109/L, bulky adenopathy and/or organomegaly, symptomatic hyperviscosity, peripheral neuropathy, amyloidosis, cryoglobulinemia, cold-agglutinin disease, or transformed disease should be considered for therapy. Plasmapheresis should be used for patients with symptomatic hyperviscosity and before administration of rituximab in those with high serum IgM levels to preempt a symptomatic IgM flare. The treatment choice should take into account specific goals of therapy, necessity for rapid disease control, risk of treatment-related neuropathy, immunosuppression and secondary malignancies, and planning for future autologous stem cell transplantation. Initial treatments include rituximab alone or combined with alkylating agents (bendamustine, cyclophosphamide); proteasome inhibitors (bortezomib, carfilzomib, ixazomib); nucleoside analogues (fludarabine, cladribine); and Bruton tyrosine kinase (BTK) inhibitors (ibrutinib, acalabrutinib, zanubrutinib). In patients with relapsed or treatment-resistant disease, an alternative frontline regimen, a BTK inhibitor (if not used previously) or an autologous stem cell transplantation can be considered. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - hemonc.mhmedical.com/content.aspx?aid=1178752658 ER -