Haematology 12: Myeloma And Amyloid, Monocloncal Gammopathy Flashcards

1
Q

What is monoclonal gammopathy of uncertain significance (MGUS) ?

A
  • IgG/IgA < 30G/L
  • <10% clonal plasma cells
  • No symptoms (CRAB)
  • Genetic errors occur in plasma cell due to infection, inflammation or chromosomal instability.
  • This causes monoclonal accumulation of the plasma cells
  • This is considered harmless but pre-malignant
  • If further mutation, can become multiple myeloma
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2
Q

Which mutation can trigger transformation of MGUS to multiple myeloma ?

A

K-RAS

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3
Q

What are the clinical features of Multiple myeloma ?

A

Calcium - elevated
Renal impairment
Anaemia
Bone lesions

Monoclonal paraprotein (gammaglobulins) 
Bence jones protein in urine (immunoglobulin light chains)
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4
Q

What are some of the characteristic features of Multiple myeloma on histology ?

A

Overcrowding of plasma cells in bone marrow

Plasmacytic cells: Nucleus on one side (eccentric nucleus), dense chromatin, low nuclear to cytoplasmic ratio

Plasmablastic cells: prominent nucleoli, reticular chromatin, less cytoplasm than mature cells

Red cell rouleaux

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5
Q

List 3 characteristic finding of multiple myeloma on Immunohistochemistry ?

A

CD138
CD38
light chain restricted Ig

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6
Q

What is fanconi syndrome ?

A

Renal tubule acidosis with light chain Chrystal depositions

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7
Q

What are the 4 main types of treatment for multiple myeloma ?

A

Cytostatic drug - Melphalan
Steroids
Immuno modulators- Thalidomide
Proteasome inhibitors

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8
Q

List the 3 features required to diagnose Multiple myeloma ?

A
  • Clonal plasma cells on bone marrow biopsy
  • Paraprotein in blood or urine
  • Evidence of end-organ damage - CRAB
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9
Q

List the 3 features that are required to diagnose Smouldering myeloma ?

A
  • Serum paraprotein > 30 g/L
  • Clonal plasma cell > 10 % on bone marrow biopsy
  • No evidence of end organ damage (CRAB)

(This is basically asymptomatic MM)

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10
Q

List 3 features required to diagnose MGUS ?

A
  • Serum paraprotein < 30g/l
  • Clonal plasma cells < 10 % on bone marrow biopsy
  • No signs of end organ damage (CRAB)
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11
Q

List 3 features of waldenstrom macroglobinaemia ?

A
  • Low grade NHL (lymphoplasmacytoid)
  • Monoclonal IgM
  • Hyperviscosity symptoms (visual problems, CCF, weakness, confusion)
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