Plasma Cell Disorders

Question 1

Plasma Cell Physiology

Answer 1

Plasma cells develop from B Lymphocytes

produce antibodies that help fight infection

present in Bone Marrow and lymph nodes

Divide repeatedly to form a clone of many identical cells

Many clones=vast number of antibodies

Question 2

Multiple Myeloma

Answer 2

Characterized by neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin.

Question 3

Multiple Myeloma: Presentation

Answer 3

Anemia – 73%
Bone pain – 58%
Elevated creatinine – 48%
Fatigue/generalizedweakness – 32%
Hypercalcemia – 28%
Weight loss – 24%

Question 4

Light chain cast nephropathy

Answer 4

also called myeloma kidney…causes kidney failure in MM

Question 5

SPEP: M-protein, M-spike

Answer 5

Will confirm Dx. of MM (SO will bone marrow biopsy)

Question 6

To Truly Dx. Multiple Myeloma All 3 criteria must be met:

Answer 6

1. Presence of a serum or urinary monoclonal protein
2. Presence of clonal plasma cells in the bone marrow or a plasmacytoma
3. Presence of end organ damage felt related to the plasma cell dyscrasia, such as:
   Increased Calcium concentration
   Renal failure
   Anemia
   Lytic bone lesions (Bone)
   (M-CRAB)

Question 7

Smoldering Multiple Myeloma SMM Both Criteria must be met:

Answer 7

1. Serum monoclonal protein ≥3 g/dL and/or bone marrow plasma cells ≥10 percent
2. No end organ damage related to plasma cell dyscrasia

Question 8

MM: Indications for Treatment

Answer 8

Anemia (hemoglobin 11.5 mg/dL)
Renal insufficiency (serum creatinine>2 mg/dL)
Lytic bone lesions or severe osteopenia
Extramedullary plasmacytoma

Question 9

Waldenström macroglobulinemia (WM)

Answer 9

a distinct clinicopathologic entity demonstrating lymphoplasmacytic lymphoma (LPL) in the bone marrow with an IgM monoclonal gammopathy in the blood. Patients may present with symptoms related to the infiltration of the hematopoietic tissues or the effects of monoclonal IgM in the blood

Question 10

WM: Presentation

Answer 10

The most common presenting features include weakness, fatigue, weight loss, and chronic oozing of blood from the nose or gums

Asymptomatic — 25%
●Constitutional symptoms — 23%
●Bleeding — 23%
●Neurologic symptoms — 22%
●Hyperviscosity (HA, vertigo, diplopia) — 31%
●Lymphadenopathy — 25%
●Hepatomegaly — 24%
●Splenomegaly — 19%
●Funduscopic abnormalities — 34%

Question 11

WM: Diagnostic Criteria

Answer 11

●An IgM monoclonal gammopathy must be present in the serum.

●10% or > of the bone marrow biopsy must demonstrate infiltration by small lymphocytes that exhibit plasma cell differentiation with an intertrabecular pattern (LPL).

●This infiltrate should express a typical immunophenotype . The plasmacytic component will be CD138+, CD38+ and CD45- or dim.

Question 12

WM: Treatment…Symptomatic WM

Answer 12

rituximab(either alone or with other agents) rather than chemotherapy alone (cyclophosphamide)

Question 13

WM: Treatment…Hyperviscosity

Answer 13

therapeutic plasmapheresis