malignant disease of plasma cells of marrow
1% malignant disaese
proliferating plasma cells
producing monoclonal paraprotein
paraproteinaemia associated with excetion of light chains in urine (bence jones)
20% no paraproteinaemia
only light chains in urine
\< 10% no light chains or paraproteinaemia
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disease of elderly
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features:
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bone destruction
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fractures - long bones
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vertebral collapse (+/- spinal cord compression)
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hypercalcaemia
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bone marrow infiltration
- anaemia, neutropenia, thrombocytopenia
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renal impairment
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deposition of light chains in renal tubules
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hypercalcaemia
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hyperuricaemia
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NSAIDs
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deposition of amyloid
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bone disease
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dysregulation of bone remoelling
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leads to typical lesions
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spine
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skull
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long bones
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ribs
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increased osteoclastic activbity
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no increase osteoblast
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bisphophonates inhibit osteoclast formation
- therefore useful but no increase in bone deposition
Symptoms
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bone pain
- most commonly backache
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anaemia
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recurrent infections
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renal failure
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hypercalcaemia
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symptoms of hyperviscosity (rare)
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extramedullary
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plasmacytoma (cutaneous)
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carpal tunnel
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peripehral neuropathy
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investigations
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FBC
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ESR - almost always high
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blood film
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rouleuax
- consequence of paraprotein
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UEC
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LDH - useful prognosis
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ALP - usually normal
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protein electrophoresis
- monoclonal band
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serum free light chain
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uric acid
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skeletal survey
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lytic lesions
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most easily seen in sk=ull
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urine BJP (24hr)
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Bone marrow aspirage
- amyloid may be found
Diagnosis
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2 out of 3:
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paraproteinaemia or BJP
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radiological evidecne of lytic bone lesions
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increase bone marrow plasma cells
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Monoclonal gammopathy of unknown significant (MGUS)
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isolated finding of monoclonal paraprotein
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20-30% develop myeloma in 25yr period
Management
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remains incurable
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bone pain
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radiotherapy
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high dose dexamethasone
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bisphosphonates
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