Myeloma

Question 1

What is myeloma?

Answer 1

A neoplastic proliferation of plasma cells in the bone, which secrete molecules/fragments of immunoglobulin causing organ dysfunction.

Question 2

What abnormal proteins are made in myeloma?

Answer 2

M proteins/paraproteins
These are most commonly IgG.
Can be the whole molecule or just light chains (kappa or lambda).

Question 3

What are the clinical features of myeloma?

Answer 3

• Bone lesions: may present with pain such as back ache.
• Blood film can show increased plasma cells and rouleux of RBCs.
• Hypercalcaemia: due to increased osteoclast activity.
• Anaemia: due to marrow infiltration (normochromic, normocytic). May get bleeding, bruising etc.
• Recurrant infection: impaired immune system and neutropenia.
• Renal impairment: due to light chain deposition.

Question 4

How is myeloma investigated?

Answer 4

• Blood test: Normochromic, normocytic anaemia, high ESR, high Urine:creatinine, high calcium.
• Marrow biopsy: many plasma cells with abnormal forms.
• Bence jones protein (free lambda or kappa chians) in urine.
• Serum/urine electrophoresis and see monoclonal bands.
• Imaging: >1 lytic bone lesions.

Question 5

What is the diagnostic criteria for myeloma?

Answer 5

1. Monoclonal protein band in serum/urine electrophoresis.
2. Increased plasma cells on bone marrow biopsy.
3. Evidence of end organ damage
   - hypercalcaemia
   - renal infufficiency
   - anaemia.
4. > 1 bony lytic lesion.

Question 6

Describe the histology of a normal plasma cell?

Answer 6

• clock face nucleus

- perinuclear hof (the ER that produces Immunoglobulin)

Question 7

What CD’s do normal plasma cells express?

Answer 7

CD19 CD138

Question 8

What CD’s do neoplasmic plasma cells express?

Answer 8

CD56 and cyclin 1

Question 9

What is smouldering myeloma?

Answer 9

Asymptomatic myeloma where there is 10-60% clonal plasma cells in BM, but no other myeloma features.
Can progress to plasma cell myeloma.

Question 10

What is Monoclonal gammaopathy of uncertain significance?

Answer 10

Serum P protein <30g/L
Clonal plasma cells in BM <10%.
No end organ damage.
Only 1% progress to myeloma.

Question 11

What is plasmacytoma?

Answer 11

Neoplastic plasma cells arising in tissue.

M protein may be detectable, but no other myeloma features.

Question 12

What treatment do fit patients get in myeloma?

Answer 12

Chemotherapy then Stem cell transplant.

Question 13

What treatment do all patients get for myeloma?

Answer 13

Chemo + steroids + thalidomide.
Radiotherapy

Supportive treatment:

• NSAIDs
• Bisphosphonates: reduce fracture rate and bone pain.
• Blood transfusion +/- Epo: treat anaemia.
• Surgical bone pinning
• Interventional radiology

Question 14

What treatment do patients with smouldering myeloma get?

Answer 14

None - watch and wait.