Waldenstrom macroglobulinemia WM Flashcards
What is Waldenstrom macroglobulinemia?
rare B cell lymphoplasmacytic lymphoma that has infiltration into the bone marrow or lymphatic tissue and creates a monoclonal IgM. Therefore there is a high circulating monoclonal IgM.
How do people with Waldenstrom macroglobulinemia present?
constitutional symptoms and oozing blood from mucosal sites and see hepatosplenomagaly, LAD and cytopenias
What is the hepatosplenomegaly from in Waldenstrom macroglobulinmia?
from infiltration of hematopoietic tissue with neoplastic cells.
what does the excessive IgM in the blood from Waldenstrom macroglobulinemia do?
causes cryoglobulinemia, sensorimotor peripheral neuropathy, hyperviscosity syndrome (blurred vision, headache, and vertigo
what is seen on labs with Waldenstrom macroglobulinemia?
marked ESR and CRP elevation
rouleaux formation on peripheral blood smear
have high protein gap since there’s a monoclonal IgM gammopathy
Sausage link retinal veins
seen with waldenstrom macroglobinemia
diagnosis of Waldenstrom Macroglobinemia should get
screening with serum protein electrophoresis. IF there’s a monoclonal IgM gammopathy then needs a confirmatory bone marrow aspiration and biopsy.
> 10% plasmacytoid or plasma cell infiltration with immunophenotype patterns.
How do we treat Waldenstrom macroglobinemia
not curable
treatment is with controlling symptoms and limiting end organ involvement.
Treat hyperviscosity syndrome with emergent plasmapheresis to reduce circulating IgM
Mean survival time for Waldenstrom macroglobinemia
10 yrs.
hyperviscousity syndrome is
related to Waldenstrom macroglobulinemia
- see lymphoproliferation and elevated serum viscoisity and infiltration of bone marrow
causes mucosal bleeding, visual changes and neurological changes
why is there neurological changes associated with Waldenstrom macroglobulinemia?
because of hyperviscousity syndrome
what kind of neurological symptoms can present with Waldenstrom macroglobulinemia?
see vertigo, hearing loss, paresthesias, ataxia, headaches, seizures, somnolence (progressing towards stupor and coma) and other neurological sequelae.
Heart failure, shortness of breath, hypoxia, fatigue, anorexia, are symptoms.
how are labs different from Waldenstrom macroglobulinemia and CLL?
CLL - chronic lymphocytic leukopenia has monoclonal expansion and congestion of functionally incompetent lymphocytes. See lymphocytosis
Waldenstrom Macroglobulinemia - will have leukopenia
Difference in presentation between Waldenstrom macroglubulinemia and MM?
MM - see pathological fractures, renal failure and spinal cord compression. Won’t see signs of infection or organomegaly
Waldenstrom = no lytic bone disease or renal failure. Will have organomegaly
what syndromes have hyperviscoisity?
waldenstrom macroglobulinemia
polycythemia vera
leukemia
