Parks 1 Flashcards
What is multiple myeloma?
B cell neoplasm of older people (~70yo)
What are two major features of multiple myeloma?
- osteolytic bone lesions
2. serum monoclonal protein with light chains (Bence Jones protein)
What types of cells are EVERYWHERE in multiple myeloma?
plasma cells
How do osteolytic lesions occur in multiple myelomas?
malignant plasma cells secrete cytokines that activate osteoclasts, which lead to increased bone resorption (break down)
What can osteolytic lesions lead to in pts with multiple myeloma?
pathologic fractures and compression fractures of the spine (these pts will present with back pain)
Multiple myeloma makes lots of plasma cells. What do these cells produce?
a BIG spike in one immunoglobulin subtype (lots of monoclonal antibody - IgG - but it is dysfunctional)
**this is called an M spike
What is an “M spike” in myeloma?
a big peak bc there is homogeneity of one type of immunoglobulin molecule that is being secreted by the plasma cells
Most myelomas secrete which immunoglobulins?
IgG or IgA
What happens to the lungs of patients with myeloma? Why?
they get pneumonia, or bacterial infection of the lungs (although they are producing excessive monoclonal Ig, it is not functional)
So what is the underlying immune abnormality in multiple myeloma?
hypogammaglobulinemia
WHY do you get hypogammaglobulinemia in myeloma if the plasma cells are secreting excess Ig?!
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)
What will you find in the urine of a patient with myeloma?
Bence Jones proteins - monoclonal light chains in the urine (heavy chains are too big to be filtered)
What can happen to the kidneys in myeloma?
renal problems
renal failure in 25%
renal disease in 50%
What is another reason why you would get kidney damage in myeloma?
hypercalcemia - calcium stones
What is a characteristic feature of the peripheral blood smear for myelomas?
Rouleaux - coin stacks of RBCs - leads to anemia
What would you see in the bone marrow of pts with myeloma?
lots of plasma cells - other cells like granulocytes will be infrequent because they are “pushed out”
What is cryoglobulin?
globulins that precipitate in the blood vessels when you are out in the cold - leads to ischemic necrosis in the extremities
What are two risks for developing myeloma?
hyperdiploidy
translocations
What is another name for lymphoplasmacytic lymphoma?
Waldenstrom’s macroglobulinemia
What cell type proliferates in Waldenstrom’s macroglobulinemia?
plasma cytoid cells
*between mature B cell and plasma cell
What is the immunoglobulin type produced in Waldenstrom’s macroglobulinemia?
IgM
What happens to your blood when you have Waldenstrom’s and lots of IgM?
your blood becomes very viscous - stacked coins or Rouleaux
What does Waldenstrom’s do to your eyes?
causes the linked-sausage effect (hyperviscosity in retinal blood vessels); visual disturbances, dizzy, headaches, altered states of consciousness
What are two skin manifestations that can be caused by Waldenstrom’s?
- cyroglobulin
2. cold agglutinins
Why does Waldenstrom’s give you fatigue and anemia?
no normoblastic precursors, which give rise to RBCs
Why do people get Raynaud’s phenomenon (digital cyanosis of the fingers)?
**occurs in Waldenstrom’s macrogammaglobulinemia
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
