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Lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia
Umberto Vitolo1, Andrés J M Ferreri, Silvia Montoto
1Hematology Unit, Azienda Ospedaliera S. Giovanni Battista Molinette, Turin, Italy.
Critical Reviews in Oncology/Hematology
|May 24, 2008
Summary
Waldenstrom's macroglobulinemia (WM) is a low-grade lymphoma associated with IgM paraproteinemia. This indolent malignancy has a median survival of 50-60 months, with treatment guided by prognostic factors and symptoms.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Waldenstrom's macroglobulinemia (WM) is a clinical syndrome characterized by IgM paraproteinemia, associated with lymphoplasmacytic lymphoma (LPL).
- LPL, a rare non-Hodgkin lymphoma (NHL), involves monoclonal B-cell expansion and is linked to Hepatitis C Virus in 26% of cases.
- LPL/WM cells exhibit specific immunophenotypes (IgM+, pan-B-cell markers) and genetic alterations like t(9;14) and 6q21 deletion.
Purpose of the Study:
- To provide a comprehensive overview of lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia (LPL/WM).
- To detail the clinicopathologic features, diagnosis, and prognosis of LPL/WM.
- To discuss current and emerging therapeutic strategies for LPL/WM.
Main Methods:
- Review of existing literature and WHO classification for LPL/WM.
- Analysis of immunophenotypic and cytogenetic characteristics of LPL/WM cells.
- Evaluation of clinical presentation, prognostic factors, and treatment outcomes.
Main Results:
- LPL/WM is an indolent B-cell lymphoma primarily affecting older adults, often presenting with disseminated disease.
- Hyperviscosity syndrome, due to IgM paraprotein, occurs in over 20% of WM patients, manifesting with bleeding, visual disturbances, and neurological symptoms.
- Median survival is 50-60 months; adverse prognostic factors include older age, B symptoms, anemia, and elevated SGOT/beta-2 microglobulin.
Conclusions:
- LPL/WM is an indolent malignancy with a variable clinical course, often not curable with conventional therapies.
- Treatment decisions are based on prognostic factors and symptom development, with ongoing research into novel therapeutic agents.
- Updated response criteria and therapeutic recommendations were established at the Third International Workshop on WM.
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