paraproteinemia
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Paraproteinemia
John Matthews
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Definition of a paraprotein
• A monoclonal immunoglobulin or immunoglobulin light chain in the blood or urine resulting from a clonal proliferation of plasma cells or B-lymphocytes (synonyms: M-band, monoclonal band, monoclonal spike, monoclonal protein)
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Identification of paraproteins
• Serum protein electrophoresis
• Immunofixation• Urine protein
electrophoresis
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Three major disorders associated with paraproteinemia
• Monoclonal Gammopathy of Undetermined Significance (MGUS)
• Multiple Myeloma • Waldenströms
Macroglobulinemia
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Plasma CellNeoplasm
Tumor Effects Paraprotein Effects
Bone lesions (Ca)
Marrow failure
Renal failure
Immune suppression
Hyperviscosity
Amyloid
Multiple Myeloma
Plasmacytoma
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Plasma cell
Rouleaux
The peripheral blood fi lm in advanced myeloma
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Plasma Cells
The bone marrow aspirate in multiple myeloma
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Multiple ‘punched-out’ lytic lesions in the skull in myeloma
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Wedgecompressionf racture of athoracicvertebra
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• Incidence – 3-6/100,000 – mean age 60: M=F: Black:White 2:1
• Cause – Unknown (?radiation)
• Presentation – Bone pain 60% Anemia 15% Renal
10% Chance 10% Other 5%.
• Diagnosis – 2 of (a) paraprotein (b) bone lesions (c)
plasma cells in BM
Multiple Myeloma
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Multiple Myeloma - therapy
• Attack the neoplasm (chemo ASCT)
• Preserve the bones (bisphosphonate)
• Treat problems: (bones, anemia, infection, renal failure, bleeding, hyperviscosity)
• Prognosis: incurable: overall median survival 30 months
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Multiple Myeloma and the Kidney
• Obstruction of tubules by light chains
• Tubule-cell damage by light chain
• Light chain deposition • Amyloidosis • Hypercalcemia
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Waldenström's Macroglobulinemia(IgM paraprotein)
Lymphoplasmacytoid neoplasm
Tumor E ff ect s Paraprotein EffectsLymphadenopathySplenomegalyMarrow failure (esp anemia)
HyperviscosityNeuropathy
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Incidence: circa 10% of MM: elderly: M/F 2/1
Presentation: HVS: fatigue, bleed, neuro, visualdisturbance, Raynaud's
Diagnosis: IgM paraprotein + Bone Marrowlymphoplasmacytoid cells
Therapy: Plasma exchange: chemotherapy
Prognosis: Long survival if HVS controlled
Waldenström's Macroglobulinemia
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Stable Paraprotein at low concentration
Not associated with marrow failure
Not causing immunosuppression
Not associated with bone lesions
Marrow plasma cell numbers small
Monoclonal Gammopathy of UncertainSignificance (MGUS) (aka Benign Monoclonal Gammopathy)
Common in elderly (1-3% of population): approx 20% developmultiple myeloma by 15 years
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bone marrow failure
pathological fractures
hypercalcemia
bone pain
renal failure
plasmacytoid lymphoma
hyperviscosity
When to suspect a paraproteinemia
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If Patient is well No lymphadenopathy, splenomegaly Normal blood count Normal renal function Normal serum calcium Paraprotein is small
Make a provisional diagnosis of MGUS recheck the paraprotein concentration after a
few months
What to do when a paraprotein is discovered
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