Myeloma and other plasma cell dyscrasia

Question 1

What is the shape of IgM?

Answer 1

pentamer

Question 2

What is the structure of IgA?

Answer 2

dimer

Question 3

How is the Ig variable element generated?

Answer 3

VDJ region recombination

Question 4

Where do B cells travel to in the periphery?

Answer 4

follicle germinal centre of the lymph node

Question 5

What are the features of plasma cells on H&E?

Answer 5

eccentric clock face nucleus; open chromatin; plentiful blue cytoplasm; pale perinuclear area

Question 6

Why do plasma cells ahve open chromatin?

Answer 6

synthesising mRNA

Question 7

Why do plasma cells have a big blue cytoplasm?

Answer 7

laden with protein

Question 8

Why do plasma cells have a pale perinucelar area?

Answer 8

golgi apparatus

Question 9

What are monoclonal immunoglobulins also known as?

Answer 9

paraproteins

Question 10

How are immunoglobulins detected?

Answer 10

serum electrophoresis

Question 11

Once detected, how can the abnormal protein band be classified?

Answer 11

serum immunofixation

Question 12

What is the difference between kappa and lamda light chains?

Answer 12

kappa- monomer whilst lambda are dimers

Question 13

How much free light chain is produced daily by normal plasma cells?

Answer 13

0.5g

Question 14

What are the direct umour ell effects in myeloma?

Answer 14

bone lesions; increased calcium; bone pain; marrow failure

Question 15

what are the paraprotein mediated effects in myleoma?

Answer 15

renal failure; immune suppression; hypervscosity; amyloid

Question 16

what is typically seen on skull xray with myeloma?

Answer 16

pepperpot appaearnce due to lytic lesions

Question 17

What are the causes of renal impairment in myeloma?

Answer 17

tubular cell damage by light chains; light chain deposition causing cast nephropathy; sepsis; hypercalcaemia and dehydration; drugs- NSAIDs; amyloid;

Question 18

What protein do light chains in the tubules bind with to produce insoluble casts?

Answer 18

Tamm-Horsfall protein

Question 19

What is the most common cause of renal failure in mutliple myeloma?

Answer 19

cast nephropathy

Question 20

What is average age at diagnosis of myeloma?

Answer 20

65

Question 21

What is the definition of MGUS?

Answer 21

paraprotein <30g/L; bone marrow plasma cells <10% and no evidence of end organ damage

Question 22

What is the risk of progression to myeloma with MGUS?

Answer 22

1% per year

Question 23

What is AL amyloidosis?

Answer 23

small plasma cell clone which has a mutation in the light chain leading to an altered structure which precipitate in tissues as an insoluble beta pleated sheet

Question 24

What does AL amyloid typically cause in the kidney?

Answer 24

nephrotic syndrome

Question 25

what does AL amyloid cause in the heart?

Answer 25

cardiomyopathy

Question 26

What stain is used t o detect amyloid?

Answer 26

congo red stain

Question 27

What is seen with staining of amyloid?

Answer 27

apple green birefringence under polarised ligth

Question 28

What is waldenstroms macroglobulinaemia?

Answer 28

lymphoplasmacytoid neoplasm

Question 29

What is a lymphoplasmacytoid neoplasm?

Answer 29

clonal disorder of cells intermediate between a lymphocyte and a plasma cell

Question 30

What type of paraprotein is seen in waldenstroms?

Answer 30

IgM

Question 31

What are the features of hyperviscosity syndrome seen with WM?

Answer 31

fatigue; visual disturbance; sonfusionl coma; bleeding; cardiac failure