Plasma Cell Dyscrasias

Question 1

What is the normal function of plasma cell?

Answer 1

Generate complete antibody in response to antigenic stimulation

Question 2

How is the plasma cell function assesses?

Answer 2

Serum protein electrophoresis

Normal function will show the highest peak being albumin and a broad shape gamma peak representing immunoglobulin

Question 3

What are the clinical manifestations of plasma cell dysfunction?

Answer 3

Expansion of neoplastic cells
Secretion of cell products
Host’s response to tumor

Question 4

What are the plasma cell dyscrasias?

Answer 4

Multiple myeloma
Monoclonal gammopathy of unknown significance (MGUS)
Waldenstrom’s gammaglobulemia

Question 5

What is multiple myeloma?

Answer 5

Malignant proliferation of plasma cells
Derived from a single clone
Bone marrow>10% plasma cell
Involved primarily IgG and IgA

Question 6

What are the clinical manifestations of multiple myeloma?

Answer 6

Hypercalcemia ( increased osteclast activity)
Renal failure (paraprotenemia)
Anemia
Bone lytic lesions ( punched out lesions)

Question 7

What are the characteristics of blood and urine testing in multiple myeloma?

Answer 7

Low normal Ig levels
Paraproteinemia
Elevated BUN and creatinine
Urine elevation for Bence Jones proteins (UPEP)

Question 8

How is multiple myeloma diagnosed?

Answer 8

Monoclonal plasma cells in bone marrow >10% or
Monoclonal antibody in the serum or urine
Plus one or more of the clinical manifestations

Question 9

What is smoldering multiple myeloma?

Answer 9

1-Serum monoclonal protein (IgA or IgG)>3.0g/L or urinary monoclonal protein>500 mg/24hours and/or clonal bone marrow plasma cells 10-60%
2- Absence of myeloma defining events or amyloidosis
Both criteria must be met

Question 10

What is the staging system for multiple myeloma ?

Answer 10

International staging system is used
Range from stage 1-3
As stage increases serum albumin and beta 2 microglobulin increase and chance of survival decreases

Question 11

What are the median survival based on stages?

Answer 11

Stage 1- 62 months
Stage 2- 44 months
Stage 3- 29 months

Question 12

How is multiple myeloma treated?

Answer 12

Chemotherapy ( Bortezomib)
Support ( bisphosphonates)
Patients are encouraged to participate in clinical trials

Question 13

What is monoclonal gammopathy oc unknown significance ( MGUS)?

Answer 13

Asymptomatic ( no CRAB diagnosis)
No evidence of an associated disorder to cause the monoclonal protein
Increases with age
More common in men and African Americans

Question 14

How is MGUS diagnosed?

Answer 14

Diagnostic evaluation same as multiple myeloma 
M protein <3
Plasma cells in bone marrow <10%
No CRAB
No Bence Jones proteins

Question 15

How is MGUS treated?

Answer 15

Treatment consist of monitoring and follow up
Repeat M protein assessment in 6 months
If no change, monitor annually for life
If M protein increasing, repeat entire evaluation to exclude myeloma

Question 16

What is Waldenstrom’s macroglobulinemia?

Answer 16

Hyperviscocity syndrome marked by thick blood, weakness, headaches, visual changes and bleeding
Characterized by monoclonal spike especially IgM
Diagnosed by bone marrow biopsy

Question 17

What are the clinical characteristics of Waldenstrom’s macroglobulinemia ?

Answer 17

Raynaud phenomenon from cryoglobulins
Bleeding due to abnormal clotting
Fewer light chains
No bony destruction

Question 18

How is Waldenstrom’s macroglobulinemia treated?

Answer 18

No cure presently
Treat hyperviscocity with plasmapheresis
Suppress the disease with a Rituximab, alkylating agents, Fludarabine