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Waldenström’s Macroglobulinemia And the Hyperviscosity Syndromes
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Waldenström 1944: Described two patients who developed bleeding from the mouth/nares, anemia, low fibrinogen levels, and hepatosplenomegaly 1944: Described two patients who developed bleeding from the mouth/nares, anemia, low fibrinogen levels, and hepatosplenomegaly 1961: Originated the concept of mono vs. polyclonal gammopathies in 1961 1961: Originated the concept of mono vs. polyclonal gammopathies in 1961
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Rare Disorder, accounts for <1% of blood cancers, ~ 1500 cases per year Median age of onset 60-65 years, affects more men than women Risk factors include chronic inflammation (autoimmunity), hepatitis, rickettsiosis, HIV, MGUS, and age Clinical manifestation % Peripheral Neuropathy Peripheral Neuropathy 10% Splenomegaly<15% Hyperviscosity Syndrome Hyperviscosity Syndrome 20% Anemia80% Cryoglobulins 20% Waldenström’s Macroglobulinemia
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The WHO considers WM a subtype of lymphoplasmacytic lymphoma This is a slow growing, indolent Non-Hodgkin’s Lymphoma arising from plasma cells Patients live several years after diagnosis, although WM is an incurable disease Occurs with much more frequency in Caucasians than in African-Americans Waldenström’s Macroglobulinemia Poor prognostic factors include: Age >65 Age >65 Hemoglobin <11.5 Hemoglobin <11.5 Platelets < 100 Platelets < 100 Beta-2 microglobulin >3 Beta-2 microglobulin >3
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Serum viscosity ranges from 1.4-1.8 centipoise Symptoms include Bleeding from mucosal surfaces Bleeding from mucosal surfaces Vertigo, headache, vision disturbances Vertigo, headache, vision disturbances Seizures, coma Seizures, coma Physical exam findings may include Dilated retinal veins Dilated retinal veins Tortuous, dilated retinal capillaries Tortuous, dilated retinal capillaries Findings consistent with peripheral neuropathy Findings consistent with peripheral neuropathy Hyperviscosity Syndrome
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Workup Workup CBC, chemistries, SPEP/immunofixation, quantitative antibodies, albumin-globulin gap, peripheral smear CBC, chemistries, SPEP/immunofixation, quantitative antibodies, albumin-globulin gap, peripheral smear Bone marrow biopsy Bone marrow biopsy Beta-2 microglobulin is not diagnostic, although has prognostic info Beta-2 microglobulin is not diagnostic, although has prognostic info Waldenström’s Macroglobulinemia Treatment
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