Myeloma Flashcards
How do plasma cells appear under the microscope?
accentrically placed nucleus and a halo around the nucleus – perinuclear hoff = ER that produces proteins, gives a clue as to what the cell does
what do plasma cells do?
produce Igs usually in the bone marrow
What happens to Igs in myeloma?
Cloned malignant plasma cells all producethe same immunoglobulin in massive quantities
What will a normal serum electrophoresis look like?
large spike for albumin and then the rest of the proteins are more or less equal in quantity, no hugely pronounced peaks after albumin
What will serum electrophersis of sb with myeloma look like?
normal spike for albumin and then a large M spike for the monoclonal component of Igs
What is the disease progression to myeloma?
- MGUS - monoclonal gammopathy of undetermined significance 2. Smoldering myeloma 3. myeloma
Is MGUS a pre-malignant condition?
yes
What is MGUS?
a pre-malignant condition for myeloma - ie yearly increased risk for developing myeloma incidental finding no end-organ damage so not a disease
How can myeloma be defined?
clonal plasma cells PLUS end organ damage
What is the pneumonic for myeloma related end organ damage?
Calcium Renal Anaemia Bone disease
How does myeloma present - both lab findings and symptoms?
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
Why are pts with myeloma more susceptible to infection?
they produce so many of one type of Ig, that they can’t produce other types
Why does renal disease result in myeloma?
due to deposition of light chains in the renal tubules high calcium dehydration NSAIDs
What type of anaemia does myeloma cause?
normocytic
What is the classical feature of myeloma on X-ray?
pepper pot skull, multiple lesions, areas of decreased density where we have lost the cortex of the bone - lytic bone disease
what is a complication of bone disease in myeloma?
spinal cord compression
Why is bone destroyed in myeloma?
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
What causes the high calcium levels in myeloma?
bone destruction
Why is there low platelets or anaemia with myeloma?
the bone marrow is invaded by malignant plasma cells
What are the signs of amyloidosis?
big tongue is amyloid until proven otherwise haematoma in subcutaneous tissue organolmegaly cardiac failure due to amyloid in heart swollen parotid gland weight loss
What is plasma cell leukaemia?
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.
What is the disease progression from acitve myeloma?
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
What is the treatment for myeloma?
combination chemo steroids bisphosphonates radiotherapy palliative care may be involved pain control and supportive measures eg antivirals rehydration as dehydrated due to hypercalcaemia
