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10 Cards in this Set

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*Definition of plasma cell myeloma
=multiple myeloma

marrow-based multifocal plasma cell neoplasm with an M-protein in the serum and/or urine
*What are the clinical presentations of multiple myeloma?
symptomatic: pain (fractures, lytic bone lesions)
fatigue (anemia from overcrowding by plasma cells and decreased EPO, uremia/renal failure (light chain deposits)
infections (recurrent, lingering)
hyperCa++: mental status changes, dehydration
hyperviscosity --> mental status changes, headache, renal failure, bleeding, visual changes, CHF

asymptomatic: no symptoms. median time of progression to symptomatic is 4.8 years
*How do you diagnose multiple myeloma?
Serum protein electrophoresis (SPEP): look for M-spike at IgA, IgG, or IgM and kappa or lambda

immunofixation determines what the protein is

free kappa and lambda chains in the serum/urine can be good for monitoring non-secreting myelomas (look for kappa: lambda ratio)
What are the diagnostic criteria for multiple myeloma?
symptomatic: M protein in serum/urine, BM clonal plasma cells or plasmacytoma, related BM/tissue impairement (CRAB)

asymptomatic: M protein in serum at myeloma levels, >10% clonal plasma cells in BM, no symptoms related to BM/tissue involvement or hyperviscosity
*What's the difference between symptomatic multiple myeloma, asymptomatic multiple myeloma and MGUS?
symptomatic: has sign of BM/tissue involvement (hyperCa++, renal insufficiency, anemia, bone lesions)

asymptomatic: just M-protein and some marrow plasmacytosis

MGUS: precursor lesion, has M-protein but no MM, Waldenstrom's, AL, or anything else (plasma cells <10%, no symptoms, no other B-cell lymphomas)
*What are the principles of treatment for multiple myeloma?
supportive: vaccines, hydration, EPO, bisphosphonates

immunomodulatory: lenalinomide, thalidomide, proteasome-inhibitor (bortezomib)

autologus transplant increases survival and QOL

Allogeneic transplant may be curative
What's a plasma cell leukemia?
multiple myeloma that's gotten into the blood, more likely with light chain, IgD and IgE (rarer) myelomas

renal failure, lymphadenopathy, organomegaly, short survival
*What's primary amyloidosis?
beta pleated sheets that stain congo red with birefringence

80% have monoclonal Ig, 20% have over multiple myeloma

AL deposits in heart (CHF, arrythmisas), GI (malabs, bleeds), liver, kidneys (nephrotic syndrome), and nerves (peripheral neuropathy)

differentiate from AA (inflammatory) or beta-2 (dialysis) amyloid-- different treatments!

tx: melphalan, steroids, thalidomide, autologous BM transplant
*What's osteosclerotic myeloma?
POEMS: polyneuropathy, organomegaly, endocrinopathies, monoclonal gammopathy, skin changes

younger patients, better survival than MM

plasma cell neoplasm with fibrosis and osteoclerosis due to monoclonal Ig (usually lambda light chain)
*What's Waldenstrom's macroglobulinemia?
lymphoplasmacytic lymphoma-- it's not a plasma cell myeloma, it's a malignant lymphoma
monoclonal paraprotein is IgM

see small lyphocytes with plasmacytoids lymphocytes and plasma cells, indolent (treat like indolent lymphoma).
fatigue, lymphadenopathy, bleeding.

Unlike MM, there's hyperviscosity and no lytic bone pain