Plasma Cell Disorders (Dyscrasias)

Question 1

A malignant proliferation of plasma cells in the bone marrow?

Answer 1

Multiple myeloma

Question 2

What may be elevated within the serum of a patient with multiple myeloma, which will stimulate plasma cell growth and immunoglobulin production?

Answer 2

IL-6

Question 3

What causes the bone pain with hypercalcemia seen with multiple myeloma?

Answer 3

The neoplastic plasma cells cause activation of the RANK receptor on osteoclasts, which leads to increased osteoclast activity and bone destruction.

Question 4

What is produced by the plasma cells in multiple myeloma that causes an elevation in serum proteins?

Answer 4

Production of immunoglobulin—most commonly IgG and IgA

Question 5

What is present on a serum protein electrophoresis demonstrating an increased production of immunoglobulin with respect to multiple myeloma?

Answer 5

An M Spike

Question 6

What is found on a blood smear of a patient with multiple myeloma?

Answer 6

Rouleaux formation of RBCs

Question 7

What causes the Rouleaux formation observed in patients with multiple myeloma?

Answer 7

The increase in serum proteins seen with multiple myeloma will cause a decrease in charge between the RBCs causing them to aggregate and form the Rouleaux.

Question 8

Light chain overproduction by neoplastic plasma cells in the context of multiple myeloma can cause what to occur?

Answer 8

Primary AL amyloidosis

Bence Jones protein in urine

Protein deposition in kidneys can lead to kidney failure

Question 9

An increased serum protein with an isolated M spike on SPEP is indicative of what disease?

Answer 9

Monoclonal gammopathy of undetermined significance (MGUS)

All other features of multiple myeloma are absent—no lytic bone lesions, hypercalcemia, AL amyloid, or Bence Jones proteinuria

Question 10

A B-cell lymphoma with monoclonal IgM production?

Answer 10

Waldenstrom Macroglobulinemia

Question 11

What are the clinical features of Waldenstrom Macroglobulinemia?

Answer 11

Generalized lymphadenopathy
Increased serum protein with M spike (comprised of IgM
Visual and neurologic deficits
Bleeding

Question 12

What serum protein causes the M spike on SPEP in Waldenstrom Macroglobulinemia?

Answer 12

IgM

Question 13

Why are visual and neurologic deficits seen with Waldenstrom Macroglobulinemia?

Answer 13

IgM is a large pentamer, which causes serum hyperviscosity

Question 14

Acute complications of Waldenstrom Macroglobulinemia are treated with plasmaphereis. How does this procedure help with the acute effects?

Answer 14

Removes the IgM from the serum