48 - Plasma Cell Disorders

Question 1

What is amyloidosis?

Answer 1

An extracellular accumulation of insoluble protein fibrils ***

Question 2

What is AL amyloidosis?

Answer 2

AL amyloidosis is a plasma cell disorder involving the accumulation of light chains in the body

Question 3

How do you diagnose amyloidosis?

Answer 3

Biopsy rectal or subcutaneous fat and stain with CONGO RED STAIN to see the fibrils ***

KNOW congo red stain ***

Question 4

What is multiple myeloma?

Answer 4

A malignant proliferation of plasma cells which are derived from a single clone (usually producing IgG)

Question 5

What is the mnemonic we use to diagnose multiple myeloma?

Answer 5

CRAB

C - calcium (like hypercalcemia from release of calcium from bone lesions)
R - renal failure (from deposition of light chains in the kidney)
A - anemia (bone marrow is affected)
B - bone lesions

Question 6

What is the test of choice for diagnosing multiple myeloma?

Answer 6

Serum electrophorysis (SPEP)
- You will see the presence of an "M spike" or M protein

Other diagnostic measure

• Plasmacytosis (tons of plasma cells)
• Myeloma related to organ or tissue involvement

Question 7

Test question: what will you see in multiple myeloma?

Answer 7

• Hypercalcemia
• Lytic bone lesions
• Rolo formation
• Beta-2 microglobulin

Question 8

Test question: what is MGUS?

Answer 8

• A benign disorder
• No anemia
• Normal calcium
• No bony lesions
• No extra plasma cells in bone marrow (less than 10%)
• M protein or M spike is less than 2.5
• Only 1%/year will progress to MM
• There is an absence of organ or tissue involvement

Question 9

Test question: what is Waldenstrom disorder?

Answer 9

• A monoclonal gammopathy involving involving IgM ***
• Commonly associated with hyperviscosity syndrome