haematology - multiple myeloma and other paraproteinaemias

Question 1

define multiple myeloma

Answer 1

neoplasia of plasma cells of BM
monoclonal immunoglobulin (paraprotein) production - IgG

Question 2

epidemiology of MM

Answer 2

middle-aged to elderly
afro-caribbeans

Question 3

clinical features of MM

Answer 3

CRAB

high calcium - moans, groans, stones

renal failure - amyloidosis and nephrotic syndrome

anaemia - pancytopenia

bone disease - osteoporosis, osteolytic lesions, pepper pot skull, wedge compression

hyperviscosity syndrome

Question 4

investigations for MM

Answer 4

bone profile - >10% plasma cells
U&Es - Bence-Jones protein
FBC - high ESR
CT/MRI

electrophoresis

blood film - rouleaux RBX stacking

Question 5

electrophoresis for MM

Answer 5

dense narrow band in gamma region
further identified by Ig, kappa/lamda light chain

Question 6

staging for MM

Answer 6

Durie-Salmon staging system

Question 7

treatment of MM

Answer 7

aim: induce remission of potential autologous SCT

bisphosphonates - supportive for CRAB symptoms

Question 8

first line treatment of MM

Answer 8

bortezomib +/- dexamethasone, cyclophosphamide, lenalidomide

auto-SCT when in remission

if auto-SCT not suitable - anti-CD38, protease inhibitors

Question 9

prognosis of MM

Answer 9

not curable
average survival 5-7yrs

Question 10

systemic amyloidosis

Answer 10

AL amyloidosis - mis-folded light chains deposited in tissues
+/- presence of myeloma

Question 11

AL amyloidosis causes

Answer 11

abnormal kappa:lambda light chain ratio

Question 12

dx of amyloidosis

Answer 12

biopsy of affected organ
congo-red stain: apple green birefringence
SAP scan

Question 13

presentation of amyloidosis

Answer 13

macroglossia
carpal tunnel syndrome
peripheral neuropathy
HF/RF