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36 Cards in this Set
- Front
- Back
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What cells give rise to plasma cells?
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B cells
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What is the role of plasma cells?
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Secreting antibody specific to a particular antigen
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What cytokines are particularly important for plasma cell growth and differentiation and what cells secrete these?
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IFN-α (from activated B cells)
IL-6 (from plasmoblasts) |
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What are the different classes of antibodies?
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IgG
IgA IgM IgE IgD |
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What is a myeloma?
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An accumulation of malfunctioning or "cancerous"plasma cells
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What is immuneparesis?
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Reduced normal serum immunoglobulin
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To what degree are the various classes of antibodies involved in B cell disorders?
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IgG > IgA > IgE > IgD
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What are the two major components of an antibody?
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Heavy chain
Light chain |
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What are the different classes of light chains?
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κ
λ |
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Why is the ratio of κ-light chains to λ-light chains clinically significant?
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A sway in either direction could indicate multiple myeloma
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What can an abnormal monoclonal spike in the γ-globulin region indicate?
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Multiple myeloma
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What are Bence-Jones proteins?
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Monoclonal light chain proteins present in urine
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What is the chief diagnostic sign of multiple gammopathy of unknown origin (MGUS)?
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IgM ≤ 3.0 g/dL
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To what does multiple gammopathy of unknown origin (MGUS) progress?
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Myeloma
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How is smoldering myeloma diagnosed?
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≥ 10% plasma cells in bone marrow
≥ 3.0 g/dL monoclonal protein in serum or urine |
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How is multiple myeloma diagnosed?
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"CRAB"
- HyperCalcemia - Renal-serum creatinine - Anemia - Lytic Bone lesions Increased β2-microglobulin |
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What are the major pathogenetic events in multiple myeloma?
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IL-6 from stromal cells of bone marrow
- Positive reinforcement for osteoclasts Overexpression of VEGF |
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How is multiple myeloma treated?
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Incurable, though for intensive for < 70 years old :
- Chemotherapy (4 rounds) - High dose chemotherapy - Allogenic stem cell transplant |
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How does lenalidomide work?
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Immunomodulatory
Antiangiogenic |
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What are drawbacks of lenalidomide?
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Rash
Thrombosis Myelosuppression |
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How does thalidomide work?
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Immunomodulatory (decreases TNF)
Antiangiogenic |
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What are drawbacks of thalidomide?
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Somnolence
Peripheral neuropathy Rash Constipation Thrombosis Myelosuppression |
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What is the mechanism of action of bartezomib?
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Specific proteosome inhibitor (26 S proteosome)
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What are drawbacks of bartezomib?
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Immunosuppression
Fatigue Peripheral neuropathy |
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What is the mechanism of action of dexamethasone?
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Increased apoptosis of plasma cells
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What is a solitary plasmacytoma?
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Isolated bone or soft tissue tumor
With or without paraprotein |
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How is a solitary plasmacytoma treated?
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Radiotherapy
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What does Waldenstrom Macroglobulinemia cause?
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Serum monoclonal IgM heavy chain paraprotein
Bone marrow lymphoplasmacytic infiltrate |
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What are symptoms of Waldenstrom macroglobulinemia?
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Anemia
Hepatomegaly Splenomegaly Hyperviscosity |
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What are clinical features of hyperviscosity?
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Visual disturbances
Lethargy Confusion Muscle weakness Congestive heart failure |
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How is hyperviscosity treated?
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Plasmapharesis
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How is Waldenstrom macroglobulinemia treated?
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Plasmapheresis
Drugs: - Alkylators - Purine analogs - Rituximab - Decadron - Bartezomib |
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What is primary amyloidosis?
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Amyloid deposition from light chains
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What does primary amyloidosis cause?
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Organ damage
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How is primary amyloidosis diagnosed?
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Quantified light chains
Bone marrow biopsy Serum amyloid P scan |
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How is primary amyloidosis treated?
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Alkylators
Decadron Bortezomib High dose chemotherapy with peripheral stem cell support Supportive care |
