Waldenström Macroglobulinemia

Rare hematologic neoplasm (accounts for 1–2% of hematologic malignancies).

In USA, age-adjusted incidence of 5.7 per million among males and 2.7 per million among females. Median age at diagnosis is 73 y.

Racial preponderance: Whites >African Americans (4.1 vs. 1.8 million).

10-y survival rate is 66%.

Factors associated with worse prognosis: age >65 y, hemoglobin <11.5 g/dL, platelet count <100,000, B ₂ -microglobulin >3 mg/L, and monoclonal IgM >7 g/dL.

Consequences of hyperviscosity

Anemia and coagulopathy

Anemia

Coagulopathy

Hyperviscosity

Hypervolemia

Hepatomegaly (20%)

Splenomegaly (15%)

Lymphadenopathy (15%)

Peripheral neuropathy: Most common neurologic complication; may be seen in up to half of all pts.

Primary systemic amyloidosis is a rare complication.

Uncommon lymphoplasmacytic lymphoma associated with monoclonal IgM protein.

Diagnosis: Presence of IgM monoclonal protein is associated with >10% clonal lymphoplasmacytic cells in bone marrow.

Symptoms attributable to tumor infiltration and/or excessive IgM production.

Most common presenting symptom is fatigue related to anemia.

Anemia can be caused by combination of factors: Decrease in red cell survival, impaired erythropoiesis, hemolysis, plasma volume expansion, and blood loss from GI tract.

Potentially severe adverse neurologic, hematologic, and CV problems periop.

Anesthetic concerns similar to those in multiple myeloma, except that hypercalcemia and bone lesions are rare; renal failure and proteinuria less common.