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12 - Waldenstrom's macroglobulinemia/lymphoplasmacytic lymphoma

Published online by Cambridge University Press:  10 January 2011

By
Steven P. Treon  and
Giampaolo Merlini
Edited by
Susan O'Brien ,
Julie M. Vose  and
Hagop M. Kantarjian
Steven P. Treon
Affiliation:
Bing Center for Waldenstrom's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA
Giampaolo Merlini
Affiliation:
Department of Biochemistry at the University of Pavia and Biotechnology Research Laboratories, University Hospital Policlinico San Matteo, Pavia, Italy
Susan O'Brien
Affiliation:
University of Texas/MD Anderson Cancer Center, Houston
Julie M. Vose
Affiliation:
University of Nebraska Medical Center, Omaha
Hagop M. Kantarjian
Affiliation:
University of Texas/MD Anderson Cancer Center, Houston
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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.

Type
Chapter
Information
Management of Hematologic Malignancies , pp. 207 - 227
Publisher: Cambridge University Press
Print publication year: 2010

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