Multiple Myeloma & other paraproteinaemias

Question 1

What is multiple myeleoma?

Most common ab class in excess?

Features (CRAB)

Answer 1

Neoplasia of plasma cells of BM > production of monoclonal abs = paraprotein - IgG most common

F - CRAB

• Calcium high - stones, moans, groans, thirst
• Renal failure - also amyloidosis & nephrotic syndrome
• Anaemia - & pancytopenia
• Bones - Pain, osteolytic lsions, fractures
• Also hyperviscosity syndrome

Question 2

MM Ix (4)

Answer 2

• Dense narrow band on electrophoresis (compared with braod band in polyclonal)
• Bence-Jones protein in urine
• High ESR
• Rouleaux - RBC stacking
• >10% plasma cells in BM

Question 3

MM Rx (4)

Answer 3

Supportive for CRAB - e.g. bisphosphonates

Chemo: melphalan, Bortezomib (proteosome inhibitor), SCT

Steroids - dex/pred

Question 4

Monoclonal gammaglobinopathy of unknown significance (MGUS)

• %plasma cells in BM? symptoms? progression to MM

Answer 4

• <10% plasma cells in marrow, <30g/l monoclonal paraprotein, No CRAB
• progress to multiple myeloma at a rate of 1-2%/yr

(seems like pre MM state)

Question 5

Smouldering Myeloma:

• plasma cells? paraprotein? Symptoms?
• Rx?

Answer 5

• >10% plasma cells & > 30g/l monoclonal paraprotein in BM, But no CRAB/ organ involvement
• No rx required for this or MGUS at this stage - monitor for transformation

Question 6

Waldenström’s macroglobulinemia: what is it? Which Ab class? epidemiology? Features? (3) Rx (2)

Answer 6

Waldenström’s macroglobulinemia is a malignant disease manifested by the proliferation of a clone of lymphocytes that produces a monoclonal IgM protein. (classified as a lymphoplasmitic lymphoma)

Occurs in elderly. Features:

• weight loss, fatigue, hyperviscosity syndrome

​Rx:

• plasmapheresis for hyperviscosity
• Chlorambucil & cyclophosphomide & other chemo