Myeloma

Question 1

How do plasma cells appear under the microscope?

Answer 1

accentrically placed nucleus and a halo around the nucleus – perinuclear hoff = ER that produces proteins, gives a clue as to what the cell does

Question 2

what do plasma cells do?

Answer 2

produce Igs usually in the bone marrow

Question 3

What happens to Igs in myeloma?

Answer 3

Cloned malignant plasma cells all producethe same immunoglobulin in massive quantities

Question 4

What will a normal serum electrophoresis look like?

Answer 4

large spike for albumin and then the rest of the proteins are more or less equal in quantity, no hugely pronounced peaks after albumin

Question 5

What will serum electrophersis of sb with myeloma look like?

Answer 5

normal spike for albumin and then a large M spike for the monoclonal component of Igs

Question 6

What is the disease progression to myeloma?

Answer 6

1. MGUS - monoclonal gammopathy of undetermined significance 2. Smoldering myeloma 3. myeloma

Question 7

Is MGUS a pre-malignant condition?

Answer 7

yes

Question 8

What is MGUS?

Answer 8

a pre-malignant condition for myeloma - ie yearly increased risk for developing myeloma incidental finding no end-organ damage so not a disease

Question 9

How can myeloma be defined?

Answer 9

clonal plasma cells PLUS end organ damage

Question 10

What is the pneumonic for myeloma related end organ damage?

Answer 10

Calcium Renal Anaemia Bone disease

Question 11

How does myeloma present - both lab findings and symptoms?

Answer 11

high ESR anaemia rouleaux on blood film poor renal function - often acute and severe monoclonal protein in urine (Bence Jones protein) monoclonal protein in blood tiredness and fatigue secondary to anaemia bone pain/pathological fractures causing back pain confusion, thirsty, constipated = high calcium infection hyperviscosity - due to XS protein amyloidosis

Question 12

Why are pts with myeloma more susceptible to infection?

Answer 12

they produce so many of one type of Ig, that they can’t produce other types

Question 13

Why does renal disease result in myeloma?

Answer 13

due to deposition of light chains in the renal tubules high calcium dehydration NSAIDs

Question 14

What type of anaemia does myeloma cause?

Answer 14

normocytic

Question 15

What is the classical feature of myeloma on X-ray?

Answer 15

pepper pot skull, multiple lesions, areas of decreased density where we have lost the cortex of the bone - lytic bone disease

Question 16

what is a complication of bone disease in myeloma?

Answer 16

spinal cord compression

Question 17

Why is bone destroyed in myeloma?

Answer 17

neoplastic B cells reach the bone marrow the stromal cells in the BM produce IL-6 Il-6 causes plasma cells to grow plasma cells produce TNFa and ILs cause stroma to produce RANK-L RANK-L causes osteoclasts to proliferate and osteoblasts and OPG to be inhibited

Question 18

What causes the high calcium levels in myeloma?

Answer 18

bone destruction

Question 19

Why is there low platelets or anaemia with myeloma?

Answer 19

the bone marrow is invaded by malignant plasma cells

Question 20

What are the signs of amyloidosis?

Answer 20

big tongue is amyloid until proven otherwise haematoma in subcutaneous tissue organolmegaly cardiac failure due to amyloid in heart swollen parotid gland weight loss

Question 21

What is plasma cell leukaemia?

Answer 21

an advanced stage of late myeloma - significant proportion of the abnormal plasma cells leave the bone marrow and are released into the blood. This is unlike myeloma, where the majority remain in the bone marrow.

Question 22

What is the disease progression from acitve myeloma?

Answer 22

there is remission with first line therapy then relapse which is then treated with second line therapy and the M-protein level plateaus after this but was not as low as in the first remission then relapse again and then potential progression to plasma cell leukaemia = refractory relapse 9disease or condition which does not respond to attempted forms of treatment.) so relapsing and remitting course

Question 23

What is the treatment for myeloma?

Answer 23

combination chemo steroids bisphosphonates radiotherapy palliative care may be involved pain control and supportive measures eg antivirals rehydration as dehydrated due to hypercalcaemia