Plasma Cell Neoplasms

Question 1

What is an “M” spike?

Answer 1

A monoclonal protein spike seen in serum protein electrophoresis of a pt with multiple myeloma. On the gel, it will look like a very dense area. Polyclonal proteins have more diffuse bands. On a computer reading, it will look like a very high, distinct sharp peak. Polyclonal proteins will show broad humps, instead (as seen in inflammation and liver disease).

Question 2

What does an IFE tell you?

Answer 2

ImmunoFixation Electrophoresis tells you exactly what type of monoclonal protein you have in excess.

Question 3

Name the four components of CRAB

Answer 3

1) bony destruction (pain, fractures, spinal cord compression)
2) hypercalcemia (altered mental status, renal insufficiency)
3) Renal insufficiency (light chain nephropathy, amyloid, uric acid, hypercalcemia, infection)
4) Anemia

Question 4

Describe the mechanism of bone destruction in myeloma

Answer 4

Myeloma cells secrete DKK1 which causes osteoblasts to release IL-6 and RANKL which activates osteoclasts to break down bone.

Question 5

Stage a myeloma with International staging system (ISS) aka international space station aka Islamic State of Syria but not Iraq.

Answer 5

ISS: the higher Beta2 microglobulin goes, the worse off the pt is. As albumin drops, pt gets worse. (albumin tells you about general body health, according to Weir)

Question 6

Name the worst prognostic chromosome abnormality in myeloma

Answer 6

Del 17p
t(4;14)
t(14;16)
**anything with 14 in it isn’t good, according to Weir. Fuck 14.

Question 7

What two classifications of new drugs have changed the way we treat myeloma? MOA?

Answer 7

IMiDs (immunomodulatory drugs such as thalidomide) and proteosome inhibitors (Bortezomib)

Question 8

When is an autologous transplant indicated in myeloma?

Answer 8

Young pts who you can get into deep remission on chemo.

Question 9

Why do Waldenstrom pts become hyperviscous more commonly than myeloma pts?

Answer 9

-

Question 10

Name three causes of amyloidosis

Answer 10

1) Light chain amyloidosis - may be primary or secondary to myeloma
- involves nervous system, heart, GI tract, and liver
2) Transthyretin amyloidosis - familial
- same systematic involvement as light chain
3) Amyloidosis AA- due to chronic inflammation
- renal disease, hepatic and GI involvement

Question 11

Myeloma is…

Answer 11

Terminally differentiated B cell malignancy

Question 12

What diagnostic test should I order to screen for myeloma?

Answer 12

Serum protein electrophoresis * REMEMBER THIS*

Question 13

Which result would make me want most to treat a paraproteinemia? 
IG > 4GM%
20% plasma cells in bone marrow
Abnormal serum free light chain ration
CRAB +
Bence Jones proteinuria

Answer 13

You will see all of these in multiple myeloma, but you treat based on what is doing most harm to the body. That is CRAB+, in this case.

Question 14

What race is at higher risk for multiple myeloma?

Answer 14

African Americans

Question 15

In a peripheral smear in a pt with multiple myeloma, you will see what RBC configuration? I.e. what are the red blood cells doing, together.

Answer 15

Rouleaux formation. RBC are stacking up due to increased protein presence in serum. Something about electrostatic forces and such.

Question 16

How do you make a definitive Dx of multiple myeloma?

Answer 16

1) M-protein (paraprotein) in serum or urine. This is in no way related to Strep. pyogenes. It is the abnormal monoclonal protein produced in excess by proliferation of monoclonal plasma cells. Makes for hyper viscous blood. MMMMnotgood.
2) Marrow clonal plasma cells (all cells express either kappa or lambda, rather than a mixture in the cell population)
3) Related organ or tissue impairment (CRAB)

Question 17

Why are bone xrays abnormal in myeloma?

Answer 17

Increased RANKL
Myeloma cells secrete DKK1 and cause osteoblasts and marrow stromal cells to secrete cytokines (such as IL-6) that increase RANKL expression by osteoblasts that activate RANK on osteoclasts and tell them to get busy breaking down all the bone. Results in lytic lesions in bone and hypercalcemia.

Question 18

Which of the following is an indicator of very poor prognosis for myeloma?
17p deletion
Anemia
B2 microglobulin
Pain
Thrombocytosis

Answer 18

17p deletion (**Seen in both CLL and Myeloma**)
lose ability for damaged cells to die, so they hang out and continue to acquire more mutations. Same as p53 mutation/deletion.

Question 19

Which treatment presently provides best survival in myeloma?
Oral alkylator
IMID
proteosome inhibitor
Autologous BMT
Allogenic BMT

Answer 19

autologous BMT

Question 20

Which signs are similar in Waldenstrom's and myeloma?
Lytic bone lesions
Lymphadenopathy
Monoclonal gammopathy
Splenomegaly
Cryoglobulinemia

Answer 20

Monoclonal gammopathy

Question 21

What’s going on in Waldenstrom’s Macroglobulinemia?

Answer 21

Low grade lymphoma which produces excess monoclonal IgM paraprotein. 97% > 40 yo
Fatigue, weight loss
Abnormal bleeding
Lymphadenopathy, hepatosplenomegaly
Hyperviscous syndrome (headache, blurred vision, mental status change, ataxia)
Serum viscosity 4x normal.
Monoclonal IgM

Question 22

How do we treat Waldenstrom Macrog?

Answer 22

Pretty much the same way as multiple myeloma. Bortezomib. alkylating agents, rituximab, Plasmapheresis.

Question 23

Which of the following is not a cause of amyloid deposits?
Monoclonal serum light chains
Transthyretin
Chronic osteomyelitis
Rheumatoid arthritis
Polyneuropathy

Answer 23

Polyneuropathy

Monoclonal serum light chains
Transthyretin - transthyretin
Chronic osteomyelitis - AA amyloidosis
Rheumatoid arthritis - AA amyloidosis