Plasma cell dyscarias

Question 1

What is MGUS

Answer 1

Monoclonal Gammopathy of Undetermined Significance

Question 2

MGUS - Paraprotein less than ____ g/l

Answer 2

30

Question 3

MGUS - Bone marrow plasma cells less than ___ %

Answer 3

10

Question 4

MGUS - There is evidence of myeloma. True or false?

Answer 4

False

Question 5

MGUS - There is evidence of end organ damage. True or false?

Answer 5

False

Question 6

MGUS - more common in men/women?

Answer 6

Men

Question 7

AL amyloidosis - definition

Answer 7

Mutation in the light chain leads to altered structure. This altered light chain precipitates in the tissues to form an insoluble beta pleated sheet

Question 8

AL amyloidosis - if the beta pleated sheet accumulates in tissues what happens?

Answer 8

There could be serious organ damage

• kidney
• heart
• liver
• neuropathy
• GI tract

Question 9

AL amyloidosis - management

Answer 9

Chemotherapy

- in order to switch off the light chain supply

Question 10

How do you diagnose AL amyloidosis

Answer 10

Organ biopsy

• confirms AL amyloid deposition
• congo red stain (apple green birefringence under polarised light)

Question 11

How do you check for evidence of AL amyloidosis deposition in other organs?

Answer 11

SAP scan

Question 12

Waldenstrom’s Macroglobulinaemia

Answer 12

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

IgM paraprotein (pentameric) 
- thus it is very large and therefore more likely to increase blood viscosity

Question 13

Waldenstrom’s Macroglobulinaemia - tumour effects

Answer 13

Lymphadenopathy
Splenomegaly
Marrow failure

Question 14

Waldenstrom’s Macroglobulinaemia - paraprotein effects

Answer 14

Hyperviscosity 
- fatigue 
- visual disturbance 
- confusion
- coma 
- bleeding 
- cardiac failure 
Neuropathy

Question 15

Waldenstrom’s Macroglobulinaemia - management

Answer 15

Chemotherapy
Plasmapheresis
- to remove paraprotein from the circulation and replace with donor plasma