Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ DEFINITION ++ Waldenström macroglobulinemia (WM) is a lymphoid neoplasm resulting from the accumulation, predominantly in the marrow, of a clonal population of lymphocytes, lymphoplasmacytic cells, and plasma cells, which secrete a monoclonal immunoglobulin (Ig) M. WM corresponds to lymphoplasmacytic lymphoma (LPL) as defined in the Revised European-American Lymphoma (REAL) and World Health Organization classification systems. Most cases of LPL are WM; less than 5 percent of cases are IgA-secreting, IgG-secreting, or nonsecreting LPL. ++ EPIDEMIOLOGY ++ The age-adjusted incidence rate of WM is 3.4 per 1 million among males and 1.7 per 1 million among females in the United States. The incidence rate is higher among Americans of European descent. Americans of African descent represent approximately 5 percent of all patients. Approximately 20 percent of patients are of Eastern European descent, specifically of Ashkenazi-Jewish ethnic background. Approximately 20 percent of 257 sequential patients with WM presenting to a tertiary referral center had a first-degree relative with either WM or another B-cell disorder. ++ PATHOGENESIS ++ Cytogenetic Findings ++ Loss of all or part of chromosomes 17, 18, 19, 20, 21, 22, X, and Y have been commonly observed, and gains in chromosomes 3, 4, and 12 also occur. Chromosome 6q deletions encompassing 6q21–25 have been observed in up to half of WM patients. ++ Nature of the Tumor Cell ++ The marrow B-cells in WM undergo maturation from small lymphocytes with large focal deposits of surface immunoglobulins, to lymphoplasmacytic cells or to plasma cells that contain intracytoplasmic immunoglobulins. ++ CLINICAL FEATURES ++ Table 70–1 lists clinical and laboratory features at the time of diagnosis for 356 patients in a large study. Presenting symptoms most commonly are fatigue, weakness, weight loss, episodic bleeding, and manifestations of the hyperviscosity syndrome. Physical findings include: — Lymphadenopathy. — Hepatosplenomegaly. — Dependent purpura and mucosal bleeding. — Dilated tortuous retinal veins. — Multiple flesh-colored papules on extensor surfaces (deposits of IgM reacting to epidermal basement membrane antigens). — Peripheral sensory neuropathy. — Raynaud phenomenon, especially upon exposure to cold. — Splenomegaly and lymphadenopathy are uncommon. ++Table Graphic Jump LocationTABLE 70–1CLINICAL AND LABORATORY FINDINGS FOR 356 CONSECUTIVE NEWLY DIAGNOSED PATIENTS WITH WALDENSTRÖM MACROGLOBULINEMIAView Table|Favorite Table|Download (.pdf) TABLE 70–1 CLINICAL AND LABORATORY FINDINGS FOR 356 CONSECUTIVE NEWLY DIAGNOSED PATIENTS WITH WALDENSTRÖM MACROGLOBULINEMIA Median Range Normal Reference Range Age (years) 58 32–91 NA Gender (male/female) 215/141 NA Marrow involvement (% of area on slide) 30 5–95 NA Adenopathy (% of patients) 15 NA Splenomegaly (% of patients) 10 NA IgM (mg/dL) 2620 270–12,400 40–230 igG (mg/dL) 674 80–2770 700–1600 IgA (mg/dL) 58 6–438 70–400 Serum viscosity (cp) 2.0 1.1–7.2 1.4–1.9 Hematocrit (%) 35 17–45 35–44 Platelet count (× 109/L) 275 42–675 155–410 White cell count (× 109/L) 6.4 1.7–22 3.8–9.2 β2M (mg/dL) 2.5 0.9–13.7... GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. What is MyAccess? Create a FREE MyAccess profile to: Use this site remotely Bookmark your favorite content Track your self-assessment progress and more!