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.
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.
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.
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:
— 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 70–1CLINICAL AND LABORATORY FINDINGS FOR 356 CONSECUTIVE NEWLY DIAGNOSED PATIENTS WITH WALDENSTRÖM MACROGLOBULINEMIA |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...|
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