An indolent B-cell lymphoma; most common in older white men.
The pathophysiologic hallmark is monoclonal IgM production by a malignant lymphoplasmacytic clone that can cause damage to multiple organs.
Presents with IgM secretion, and, in less than 15% of cases, also features lymphadenopathy and splenomegaly. By contrast, multiple myeloma usually presents with IgG or IgA secretion and lytic bone lesions.
The symptoms and signs of hyperviscosity (such as skin and mucosal bleeding, retinopathy with visual disturbances, and neurologic symptoms such as headache, dizziness, and vertigo) are important clinical manifestations and should be treated with urgency by plasmapheresis and chemotherapy.
Although the tumor is responsive to treatment with a number of agents (including alkylating agents, nucleoside analogs, and monoclonal antibodies), it remains incurable by conventional chemotherapy.
There is no standard of care. Treatment is individualized and ranges from observation for asymptomatic patients, to monotherapy or combination chemotherapy, to high-dose therapy with stem cell rescue. Participation in clinical trials is recommended.
Waldenström macroglobulinemia (WM) is an indolent lymphoma in which malignant monoclonal plasmacytoid lymphocytes infiltrate bone marrow and visceral organs and hypersecrete monoclonal IgM, causing damage to multiple organs. The clinical manifestations are related to organ infiltration by malignant cells (splenomegaly, hepatomegaly, lymphadenopathy), bone marrow infiltration leading to cytopenias (anemia, thrombocytopenia, pancytopenia), and abnormal IgM properties and/or organ deposition (hyperviscosity syndrome, polyneuropathy, cryoglobulinemia, cold agglutinin hemolytic anemia, bleeding from the nose and gums, purpura, thrombosis, and kidney disease). In contrast with multiple myeloma, bone lesions are uncommon.
History and exam
- hx of IgM monoclonal gammopathy of undetermined significance (MGUS)
- FHx of B-cell lymphoma or myeloma
- FHx of WM with or without monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma
- peripheral neuropathy
- weight loss
- Raynaud phenomenon
NYU School of Medicine
BK declares that he has no competing interests.
Associate Professor of Medicine
NYU Langone Medical Center
KH declares that he has no competing interests.
Department of Hematology
SK declares that he has no competing interests.
Professor of Medicine
Chief, Section of Hematology
Department of Internal Medicine
Yale University School of Medicine
MD declares that he has no competing interests.
Instructor in Hematology
Department of Hematology
Hopital Huriez CHRU
XL has received lecture fees and research funding from Janssen-Cilag, Celgene, Chugai, Amgen, Novartis, Mundipharma, and Roche. XL is an author of a number of references cited in this monograph.
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