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15 - Waldenstrom’s macroglobulinemia/lymphoplasmacytic lymphoma
- from Section 3 - Myeloma: clinical entities
- Edited by Stephen A. Schey, Kwee L. Yong, Robert Marcus, Kenneth C. Anderson
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- Book:
- Myeloma
- Published online:
- 18 December 2013
- Print publication:
- 05 December 2013, pp 190-215
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Summary
Introduction
Waldenstrom’s macroglobulinemia (WM) is a distinct clinicopathological entity resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphocytes, lymphoplasmacytic cells and plasma cells which secrete a monoclonal IgM protein (Figure 15.1)[1]. This condition is considered to correspond to the lymphoplasmacytic lymphoma (LPL) as defined by the World Health Organization classification system[2]. Most cases of LPL are WM, with less than 5% of cases made up of IgA, IgG and non-secreting LPL.
Epidemiology and etiology
WM is an uncommon disease, with a reported age-adjusted incidence rate of 3.4 per million among males and 1.7 per million among females in the USA, and a geometrical increase with age[3]. The incidence rate for WM is higher among Caucasians, with African descendants representing only 5% of all patients. Genetic factors appear to be important to the pathogenesis of WM. A common predisposition for WM with other malignancies has been raised[4,5], and there have been numerous reports of familiar predisposition, including clustering of family members with WM and other B cell lymphoproliferative diseases[6–10]. In a recent study, 28% of 924 serial WM patients presenting to a tertiary referral had a first or second degree relative with either WM or another B - cell disorder[5]. Frequent familiar association with other immunological disorders in healthy relatives, including hypogammaglobulinemia and hypergammaglobulinemia (particularly polyclonal IgM), autoantibody (particularly to thyroid) production, and manifestation of hyper-responsive B cells have also been reported[10,11].
9 - Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma
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- By Steven P. Treon, Northwestern University Feinberg School of Medicine, Giampaolo Merlini, University of Pavia
- Edited by Robert Marcus, King's College London, John W. Sweetenham, University of Utah, Michael E. Williams
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- Book:
- Lymphoma
- Published online:
- 18 December 2013
- Print publication:
- 05 December 2013, pp 138-154
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Summary
Introduction
Waldenstrom's macroglobulinemia (WM) is a distinct clinicopathological entity resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphocytes, lymphoplasmacytic cells, and plasma cells that secrete a monoclonal IgM protein (Figure 9.1). This condition is considered to correspond to lymphoplasmacytic lymphoma (LPL) as defined by the World Health Organization classification system. Most cases of LPL are WM, with less than 5% of cases made up of IgA, IgG, and non-secreting LPL.
Epidemiology and etiology
WM is an uncommon disease, with a geometrical increase with age. The incidence rate for WM is higher among Caucasians, with African descendants representing only 5% of all patients. Genetic factors appear to be important to the pathogenesis of WM. A common predisposition for WM with other malignancies has been raised, and there have been numerous reports of familial predisposition, including clustering of family members with WM and other B-cell lymphoproliferative diseases. In a recent study, 28% of 924 serial WM patients presenting to a tertiary referral had a first or second degree relative with either WM or another B-cell disorder. Frequent familial associations with other immunological disorders in healthy relatives have also been reported. The role of environmental factors in WM remains to be clarified, but chronic antigenic stimulation from infections, certain drug and agent orange exposures remain suspect.
12 - Waldenstrom's macroglobulinemia/lymphoplasmacytic lymphoma
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- By Steven P. Treon, Bing Center for Waldenstrom's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA, Giampaolo Merlini, Department of Biochemistry at the University of Pavia and Biotechnology Research Laboratories, University Hospital Policlinico San Matteo, Pavia, Italy
- Edited by Susan O'Brien, Julie M. Vose, Hagop M. Kantarjian
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- Book:
- Management of Hematologic Malignancies
- Published online:
- 10 January 2011
- Print publication:
- 18 November 2010, pp 207-227
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Summary
Introduction
Waldenstrom's macroglobulinemia (WM) is a distinct clinicopathologic entity resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphocytes, lymphoplasmacytic cells, and plasma cells which secrete a monoclonal IgM protein (Figure 12.1). This condition is considered to correspond to the lymphoplasmacytic lymphoma (LPL) as defined by the Revised European–American Lymphoma (REAL) and World Health Organization (WHO) classification systems. Most cases of LPL are WM, with less than 5% of cases made up of IgA, IgG, and non-secreting LPL.
Epidemiology and etiology
WM is an uncommon disease, with a reported age-adjusted incidence rate of 3.4 per million among males and 1.7 per million among females in the USA, and a geometrical increase with age. The incidence rate for WM is higher among Caucasians, with African descendants representing only 5% of all patients. Genetic factors appear to be an important factor to the pathogenesis of WM. Approximately 20% of WM patients have an Ashkenazi (Eastern European) Jewish ethnic background, and there have been numerous reports of familiar disease, including multigenerational clustering of WM and other B-cell lymphoproliferative diseases. In a recent study, approximately 20% of 257 serial WM patients presenting to a tertiary referral had a first-degree relative with either WM or another B-cell disorder. Frequent familiar association with other immunologic disorders in healthy relatives, including hypogammaglobulinemia and hypergammaglobulinemia (particularly polyclonal IgM), autoantibody (particularly to thyroid) production, and manifestation of hyperactive B cells have also been reported.
11 - WALDENSTRÖM MACROGLOBULINEMIA/LYMPHOPLASMACYTIC LYMPHOMA
- Edited by S. Vincent Rajkumar, Robert A. Kyle
-
- Book:
- Treatment of Multiple Myeloma and Related Disorders
- Published online:
- 11 July 2009
- Print publication:
- 27 October 2008, pp 129-149
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Summary
INTRODUCTION
Waldenström macroglobulinemia (WM) is a distinct clinicopathological entity resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphocytes, lymphoplasmacytic cells, and plasma cells that secrete a monoclonal immunoglobulin M (IgM) protein (Figure 11.1). This condition is considered to correspond to the lymphoplasmacytic lymphoma (LPL) as defined by the Revised European American Lymphoma (REAL) and World Health Organization classification systems. Most cases of LPL are WM, with less than 5% of cases made up of IgA, IgG, and nonsecreting LPL.
EPIDEMIOLOGY AND ETIOLOGY
WM is an uncommon disease, with a reported age-adjusted incidence rate of 3.4 per million among males and 1.7 per million among females in the United States, and a geometrical increase with age. The incidence rate for WM is higher among Caucasians, with African descendants representing only 5% of all patients. Genetic factors appear to be an important factor to the pathogenesis of WM. Approximately 20% of WM patients have an Ashkenazi (Eastern European) Jewish ethnic background, and there have been numerous reports of familiar disease, including multigenerational clustering of WM and other B-cell lymphoproliferative diseases. In a recent study, approximately 20% of 257 serial WM patients presenting to a tertiary referral had a first degree relative with either WM or another B-cell disorder. Frequent familiar association with other immunological disorders in healthy relatives, including hypogammaglobulinemia and hypergammaglobulinemia (particularly polyclonal IgM), autoantibody (particularly to thyroid) production, and manifestation of hyperactive B cells have also been reported.