Plasma Cell Disorders Flashcards
Essential monoclonal gammopathy is defined by two things?
Presence of monoclonal immunoglobulin or lightchain in the serum and the absence of evidence suggesting malignancy of by cells or plasma cells.
What is the prevalence of essential monoclonal gammopthy is Americans, Europeans and Africans?
As you go from 50 -80s it goes from 2% to 7%
Africans it is 3 times higher
6 effects of the malignancy proliferation of plasma cells in MM?
Normocytic anemia with other penias Low immunoglobulin, so infections Osteoclast activity, so hyerpcalcemia, bone pain and fractures Plasma cell tumors, plasmacytomas M proteins cause renal tubular injury M proteins cause hyperviscosity
For diagnosis purposes, what will the following show?
CBC, blood smear, serum calcium, serum creatinine, serum protein electrophoresis, quantitative immunoglobulin measurement, 24 hour urine protein electrophoresis, bone scan, bone marrow?
Anemia is most important Rouleaux formation Hypercalcemia Over 2 M spike Confirms monogammopathy and there is usually one uninvolved Ig that is suppressed M protein in urine Lyric bone lesions Over 10% at now plasma cells
What two markers suggest high myeloma tumor burden for MM?
LDH and beta 2 microglobulin
patients found to have M protein are put into 3 categories regarding MM, what are they and what are the major differences?
First, MGUS. Small amount of M protein and less than 10% plasma cells in bone marrow. Asymptomatic
Smoldering myeloma, or in between, larger amount of M protein and plasma cells in marrow, but still asymptomatic
MM, they are symptomatic, with larger M protein amount and hypo Ig.
What is considered a good prognosis stage and bad prognosis stage for MM?
Low beta 2 micro and normal albumin is good stage 1
Beta 2 micro over 5.5 is poor prognosis
What is the best therapy for MM and how do we do it?
Stem cell transplantation for symptomatic patients, usually under 75.
The goal here is to do chemo for 2-4 months to shrink the tumor burden and confirm response to chemo and then proceed to transplant.
Use high dose dexamethasone and thalidomide or lenalidomide and bortezomib for the induction of chemo.
Then melphalen during transplant.
What is considered the classic triad of MM for diagnostic purposes?
Marrow plasma cells over 10%
Lyric bone lesions
Serum and or urine M spike
5 preventative things to do for MM?
Pneumococcal, influenza vaccines should be given along with TMP-SMX prophylaxis
Acyclovir to present varicella
Bisphsphates for fractures, bone pain, and high calcium
MRI for back
Kidney treatment is things get bad
2 things specific to IgM myeloma that will distinguish it from W. Macroglobulinemia?
Lyrics bone lesions and cd138 plasma cells
How do we characterize w. Macroglobulinemia?
B cell secreting a ton Ig IgM where there is LAD, hepatosplenomegaly and hyperviscotiy. No bone lesions.
What is the most common mutation for WM? What is an additional mutation to know that is important in the clinical picture? Explain each mutation’s to the clinical picture?
MYD 88, lower bone marrow disease burden
CXCR4, higher bone marrow disease burden
What specific protein does the IgM go after and what clinical symptom does it cause?
MAG, peripheral neuropathy
What two main things are not found in macro that are found in MM?
Renal disease and lytic bone lesions
