Multiple Myeloma

Question 1

What is multiple myeloma?

Answer 1

It is a neoplasm of the plasma cells in the blood, producing a monoclonal immunoglobulin, also called paraprotein.

Question 2

What is the epidemiology surrounding multiple myeloma?

Answer 2

It usually affects those aged 65-70 and comes with a mean survival of 3-4 years (6-7 with intensive treatment)
There is an increased incidence in Afro-Caribbean’s

Question 3

What is the pathology underlining multiple myeloma?

Answer 3

There is a genetic mutations causing a large amount of plasma cells, called monoclonal gammopathy of undetermined significance, affecting 5% of everyone above the age of 75. Further mutations causes these cells to progress further into multiple myeloma, where there is a predominance of plasma cells in the blood.

Question 4

What are the clinical features of multiple myeloma?

Answer 4

CRAB syndrome

• Calcium is high (due to renal failure)
• Renal failure (due to light chain deposition in kidneys)
• Anaemia (and infection, due to cytopaenia)
• Bone disease (pain, osteolytic lesions, osteoporosis, fractures)

Question 5

What investigations are performed in a suspected case of multiple myeloma?

Answer 5

• Serum electrophoresis (dense narrow band)
• Gamma wave
• Blood film: RBC form rouleaux
• Urine: Bence-Jones protein
• FBC: ESR is very high
• BM: >10% are plasma cells
• Immunophenotyping: positive (CD 38 CD 138, cytoplasmic Ig) and negative (CD 19 CD 20)

Question 6

What is the progression of multiple myeloma from normal?

Answer 6

• Monoclonal gammopathy of undetermined significance
• Smouldering multiple myeloma
• Multiple myeloma

Question 7

What is the treatment for multiple myeloma?

Answer 7

Five methods:

1) Cytostatic drugs: Melphalan
2) Steroids

3) Proteosome inhibitors
- Inhibit the removal of misfolded proteins, killing the cell (they precipitate in the cytoplasm and kill it)

4) Thalidomide
5) Autologous stem cell transplant (high dose melphalan in between to kill all, then replace with frozen stem cells)

Question 8

Describe some other paraproteinaemias (other than multiple myeloid)

Answer 8

Waldenstrom’s Macroglobinaemia (lymphoplasmocytoid lymphoma)

• A type of NHL
• Serum IgM
• Hyperviscosity syndrome and weight loss
• treated with chemo

Systemic Amyloidosis

• Ig light chains deposited in tissues
• Diagnosed with Congo red stain
• Macroglossia, carpal tunnel and peripheral neuropathy
• Treated with chemo or auto-SCT