While on the topic of paraproteinaemia is seems appropriate to cover hyperviscosity syndrome, which lends itself to a quick forray into polycythaemia and then on to two completely random topics to round off the day.
Enjoy!
MRCP revision battle 33.1: Myeloma
MRCP revision battle 33.2: Hyperviscosity syndrome
MRCP revision battle 33.3: Waldenstrom's macroglobulinaemia
MRCP revision battle 33.4: MGUS
MRCP revision battle 33.5: Amyloidosis
MRCP revision battle 33.6: Polycythaemia
MRCP revision battle 33.7: Levels of evidence
MRCP revision battle 33.8: Holmes Adie pupil
MRCP revision battle 33.1: Myeloma
Myeloma is a malignant monoclonal proliferation of plasma cells.
The commonest subclass is IgG (IgG>IgA>IgM)
Symptoms:
- osteolytic bone lesions --> backache/pathological fractures
- symptoms of hypercalcaemia
- bacterial infections due to immunoparesis
- renal impairment due to light chains
Investigations:
- Bloods:
- normocytic normochromic anaemia
- rouleaux on blood film
- raised calcium (40%)
- raised urea and creatinine
- raised ESR
- Urine
- Bence Jones proteins (=free serum light chains) in urine (66%)
- XR
- ?pepper pot skull, vertebral collapse
The diagnostic criteria for myeloma is:
- monoclonal protein band in serum or urine electrophoresis
- increased plasma cells on BM biopsy
- evidence of end organ damage from myeloma
Treatment:
- supportive
- chemo
- younger patients: aggressive (VAD - vincristine, adriamycin, dexamethasone)
- older patients: less aggressive (CDT - cyclophosphamide, dexamethasone, thalidomide)
Complications of myeloma include:
- Hyperviscosity syndrome
- Hyperviscosity syndrome occurs most commonly in IgM myeloma (IgM>IgA>IgG)
- Transfusions should be avoided in hyperviscosity syndrome.
- hypercalcaemia
- spinal cord compression
- acute renal failure
- AL amyloidosis (15%)
Survival with myeloma tends to be 3-4 yrs.
Higher beta 2 microglobulin implies a worse prognosis.
Onwards for a bit more about hyperviscosity syndrome....
