Parks 1

Question 1

What is multiple myeloma?

Answer 1

B cell neoplasm of older people (~70yo)

Question 2

What are two major features of multiple myeloma?

Answer 2

1. osteolytic bone lesions

2. serum monoclonal protein with light chains (Bence Jones protein)

Question 3

What types of cells are EVERYWHERE in multiple myeloma?

Answer 3

plasma cells

Question 4

How do osteolytic lesions occur in multiple myelomas?

Answer 4

malignant plasma cells secrete cytokines that activate osteoclasts, which lead to increased bone resorption (break down)

Question 5

What can osteolytic lesions lead to in pts with multiple myeloma?

Answer 5

pathologic fractures and compression fractures of the spine (these pts will present with back pain)

Question 6

Multiple myeloma makes lots of plasma cells. What do these cells produce?

Answer 6

a BIG spike in one immunoglobulin subtype (lots of monoclonal antibody - IgG - but it is dysfunctional)

**this is called an M spike

Question 7

What is an “M spike” in myeloma?

Answer 7

a big peak bc there is homogeneity of one type of immunoglobulin molecule that is being secreted by the plasma cells

Question 8

Most myelomas secrete which immunoglobulins?

Answer 8

IgG or IgA

Question 9

What happens to the lungs of patients with myeloma? Why?

Answer 9

they get pneumonia, or bacterial infection of the lungs (although they are producing excessive monoclonal Ig, it is not functional)

Question 10

So what is the underlying immune abnormality in multiple myeloma?

Answer 10

hypogammaglobulinemia

Question 11

WHY do you get hypogammaglobulinemia in myeloma if the plasma cells are secreting excess Ig?!

Answer 11

Malignant plasma cells secrete factors that suppress the normal plasma cells from making normal immunoglobulin

**If low on effective immunoglobulins, you will get FREQUENT INFECTIONS(acute pneumonia, etc)

Question 12

What will you find in the urine of a patient with myeloma?

Answer 12

Bence Jones proteins - monoclonal light chains in the urine (heavy chains are too big to be filtered)

Question 13

What can happen to the kidneys in myeloma?

Answer 13

renal problems

renal failure in 25%
renal disease in 50%

Question 14

What is another reason why you would get kidney damage in myeloma?

Answer 14

hypercalcemia - calcium stones

Question 15

What is a characteristic feature of the peripheral blood smear for myelomas?

Answer 15

Rouleaux - coin stacks of RBCs - leads to anemia

Question 16

What would you see in the bone marrow of pts with myeloma?

Answer 16

lots of plasma cells - other cells like granulocytes will be infrequent because they are “pushed out”

Question 17

What is cryoglobulin?

Answer 17

globulins that precipitate in the blood vessels when you are out in the cold - leads to ischemic necrosis in the extremities

Question 18

What are two risks for developing myeloma?

Answer 18

hyperdiploidy

translocations

Question 19

What is another name for lymphoplasmacytic lymphoma?

Answer 19

Waldenstrom’s macroglobulinemia

Question 20

What cell type proliferates in Waldenstrom’s macroglobulinemia?

Answer 20

plasma cytoid cells

*between mature B cell and plasma cell

Question 21

What is the immunoglobulin type produced in Waldenstrom’s macroglobulinemia?

Answer 21

IgM

Question 22

What happens to your blood when you have Waldenstrom’s and lots of IgM?

Answer 22

your blood becomes very viscous - stacked coins or Rouleaux

Question 23

What does Waldenstrom’s do to your eyes?

Answer 23

causes the linked-sausage effect (hyperviscosity in retinal blood vessels); visual disturbances, dizzy, headaches, altered states of consciousness

Question 24

What are two skin manifestations that can be caused by Waldenstrom’s?

Answer 24

1. cyroglobulin

2. cold agglutinins

Question 25

Why does Waldenstrom’s give you fatigue and anemia?

Answer 25

no normoblastic precursors, which give rise to RBCs

Question 26

Why do people get Raynaud’s phenomenon (digital cyanosis of the fingers)?

**occurs in Waldenstrom’s macrogammaglobulinemia

Answer 26

mutation of MYD88 leads to a gain of function mutation –> too much NF-kB –> too much B cell proliferation, not enough apoptosis of B cells