Plasma Cell Disorders

Question 1

Represents a malignant proliferation of plasma cells derived from a single clone

Answer 1

Multiple myeloma (MM)

Question 2

Most common symptom in myeloma (70% of patients)

Answer 2

Bone pain

Question 3

Typical MM bone lesion (e.g., skull) representing purely osteolytic with little to no osteoblastic activity

Answer 3

“Punched out” lesions

Question 4

Most common cause of renal failure in MM

Answer 4

Hypercalcemia

Question 5

List the 4 myeloma defining events (mnemonic: CRAB)

Answer 5

1) HyperCalcemia
2) Renal insufficiency
3) Anemia
4) Bone lesions

Question 6

Known as asymptomatic myeloma; with serum monoclonal protein >30 g/L and clonal bone marrow plasma cells 10-60%, WITHOUT myeloma defining events or amyloidosis

Answer 6

Smoldering Multiple Myeloma (SMM)

Question 7

Prodrome of MM; with serum monoclonal protein <30 g/L and/or clonal bone marrow plasma cells <10%, WITHOUT myeloma defining events or amyloidosis

Answer 7

Monoclonal Gammopathy of Undetermined Significance (MGUS)

Question 8

2 important variants of myeloma

Answer 8

1) solitary bone plasmacytoma

2) solitary extramedullary plasmacytoma

Question 9

Useful diagnostics for characterizing M spikes

Answer 9

Serum protein electrophoresis
Serum immunoglobulins
Serum Free light chains
*Immunoelectrophoresis- sensitive for low concentrations of M component

Question 10

Diagnostic Necessary to quantitate Bence Jones protein excretion

Answer 10

24-hour urine

Question 11

Diagnostic to assess bone damage and detect extramedullary sites of myeloma; distinguishes between smoldering and active MM, and to confirm a suspected diagnosis of solitary plasmacytoma

Answer 11

F-FDG PET/CT scan

*F-fluorodeoxyglucose

Question 12

Single most powerful predictor of survival and can substitute MM staging. It is the light chain of the class I major histocompatibility antigens on the surface of every cell.

Answer 12

Serum beta-microglobulin

Question 13

Proteasome inhibitor that is part of the preferred induction regimen

Answer 13

Bortezomib

*Most Preferred induction regimen: RVD (R-lenalidomide, V-bortezomib, D-dexamethasone)

Question 14

Treatment of choice for hyperviscosity syndrome

Answer 14

Plasmapheresis

Question 15

Malignancy of lymphoplasmacytoid cells that secrete IgM but unlike MM, the disease is associated with lymphadenopathy, hepatomegaly, and primarily with hyperviscosity syndrome. It does not cause bone lesions or hypercalcemia.

Answer 15

Waldenström’s Macroglobulinemia (WM)

Question 16

Burton’s tyrosine kinase (BTK) inhibitor approved by US FDA for use in symptomatic WM patients

Answer 16

Ibrutinib

Question 17

Patient with this syndrome presents with severe, progressive sensorimotor polyneuropathy associated with sclerotic bone lesions from myeloma

Answer 17

POEMS Syndrome

*Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes

Question 18

Heavy chain disease associated with rheumatoid arthritis with distinctive symptom of palatal edema

Answer 18

Gamma Heavy Chain Disease (Franklin’s Disease)