Myeloma

Question 1

Explain the pathophysiology

Answer 1

Clonal proliferation of BM plasma cells

Abnormal monoclonal Igs (M proteins) proliferate in the blood = paraproteinaemia

Question 2

What are the RFs

Answer 2

M>F
Increased age
+ve FHx

Question 3

What is the median age for getting it?

Answer 3

70

Question 4

Give the mnemonic for signs and sx

Answer 4

C - calcium (elevated)
R - renal failure
A - anaemia
B - bone lesions

Question 5

Which bones are usually affected? How can it present?

Answer 5

Spine + ribs
Worse w activity
Bone fractures -> persistent localised pain
Spinal cord compression or kyphosis

Question 6

What type of anaemia is present?

Answer 6

normochromic normocytic.

Question 7

What are the causes of renal failure in MM?

Answer 7

Deposition of light chains in tubules
Hypercalcaemia
Hyperuricaemia
Amyloid deposition

Question 8

How does BM infiltration present

Answer 8

anaemia
thrombocytopenia
neutropenia

Question 9

What common infections occur due to MM?

Answer 9

pyelonephritis

pneumonia

Question 10

What neurological sx may be present? why do they occur?

Answer 10

Confusion, weakness and fatigue – hypercalcaemia

Headaches and visual disturbances – hyperviscosity (present in some paraproteinaemia)

Peripheral neuropathy – amyloid deposition

Limb weakness and loss of bowel/bladder control – spinal cord compression

Question 11

What ix would u do?

Answer 11

1. FBC, serum Ca2+, ESR
2. Protein electrophoresis in blood and urine
3. Peripheral blood film
4. Serum urea, creatinine, electrolytes, albumin, uric acid
5. XR symptomatic areas to rule out pathological fractures
6. Ig measurement
7. BM biopsy
8. Beta 2 microglobulin - elevated

Question 12

What would you see on peripheral blood film?

Answer 12

rouleaux - red cells stacked on top of each other

Question 13

why would u see a raised uric acid?

Answer 13

high cell turnover

Question 14

what would u see on protein electrophoresis

Answer 14

Bence Jones protein

may demonstrate a paraprotein band

Question 15

why would u measure calcium

Answer 15

bone profile

Question 16

how is MM classified?

Answer 16

based on which Ig is produced:

• IgG 2/3
• IgA 1/3

Question 17

When are free light chain levels measured?

Answer 17

When assessing response to rx

Question 18

What are diagnostic features?

Answer 18

1. paraproteinaemia or serum protein immunofixation or BJ proteins in urine
2. CT/MRI - lytic bone lesions
3. increase in BM plasma cells on BM aspirate or trephine biopsy

Question 19

What is symptomatic rx?

Answer 19

1. BISPHOSPHONATES - reduce bone pain and disease
2. CORRECT ANAEMIA - darbepoietin (erythropoeitin), blood transfusion
3. Chemo e.g. cyclophosphamide
4. CS e.g. dexamethasone + prednisolone
5. immunomodulatory drugs - thalidomide,
6. High dose drug therapy + stem cell transplant
7. DVT proph e.g. aspirin, LMWH, warfarin

Question 20

Whatdrugs should be avoided ?

Answer 20

aminoglycosides for inf

NSAIDs - renal toxicity

Question 21

What is the prognosis

Answer 21

usually incurable

Question 22

what indicates a worse prognosis?

Answer 22

increased levels of beta 2 micro globulin 
high plasma cell counts
diffuse multiple bone lesions 
hypercalcaemia
v high levels of M protein in blood 
renal impairment

Question 23

what are complications of MM

Answer 23

1. spinal cord compression - back pain leg weakness paraesthesia, urinary retention/incontinence
2. hyper viscosity - reduced cognition, loss of vision, bleeding

Question 24

What is the rx of hyperviscosity?

Answer 24

plasmapheresis to remove light chains

Question 25

What is seen on CXR in myeloma?

Answer 25

pepper pot skull

Question 26

what is the asymptomatic premalignant stage called?

Answer 26

monoclonal gammopathy of undetermined significance