Waldenstrom Macroglobinemia is a hematologic malignancy marked by:
Pathophysiology
1) type of B-cell lymphoma 2) characterized by monoclonal proliferation of IgM and hyperviscosity syndrome
Signs and Symptoms of Waldenstrom Macroglobulinemia
1) lymphadenopathy 2) hepatosplenomegaly 3) dizziness 4) headache 5) peripheral neuropathy 6) deafness 7) ecchymoses and purpura (especially of the legs) 8) hemorrhagic infarct of fingers and toes 9) recurrent infections 10) visual problems and blindness 11) dilated retinal veins 12) coma
Characteristic Test Findings
Laboratory – 1) increased serum IgM 2) Bence Jones’ proteins in urine (kappa or alpha immunoglobulin light chains) 3) decreased factor 8 4) positive Coombs’ test
Histology/Gross Pathology
1) cells are small lymphocytes with moderate cytoplasm, eccentric nuclei, and plasmacytoid differentiation 2) Dutcher’s bodies (refractile eosinophilic nuclear inclusions 3) plasma cells have Russell’s bodies (cytoplasmic globules)
Associated Conditions
1) Raynaud’s phenomenon 2) occasionally renal damage
Inheritance/Epidemiology
1)Â Waldenstrom macroglobulinemia affects middle-aged to elderly 2) mean survival is 3-4 years if untreated 3) slightly more common in men 4) some think initiating event is viral infection that causes antibody response that then becomes pathologic
Treatment
1) plasmaphereis to treat the Waldenstrom macroglobulinemia hyperviscosity syndrome owing to increased IgM in serum 2) chemotherapy (fludarabine or cladribine)
Tips for USMLE
1) Bence Jones proteins also occur in mulitple myeloma 2) major clinical entity is the hyperviscosity syndrome caused by excessive IgM in plasma 3) to distinguish Waldenstrom’s and multiple myeloma – no hypercalcemia or bone lesions in Waldenstrom’s 4) peripheral neuropathy can present months before neoplastic cells
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