Plasma Cell Disorders - Hubbard Flashcards
MGUS defined as what?
Tx
BM monoclonal plasma cells < 10% w/serum M-protein < 3 g/L
NO end organ damage
Observe brah, duh
How do you diagnose Alpha-HCD?
Treatment?
Serum immunofixation showing monoclonal chain, no light chain
Antibiotics and CHOP
What shows initially a lymphoma like illness, HS and LAD in 60%, anemia in 80%?
Treatment?
Gamma-HCD
Chemo and some drugs
Rouleux formation w/ESR above 100 may be seen in what?
Tx?
Waldenstrom’s
Rituximab
What are the major clinical manifestations of cryoglobulinemia
Vasculitis, pain, purpura, Raynaud’s, cyanosis, ulceration, and sloughing of skin on exposure to cold
Which type of cryoglobulinemia is most common? Describe it a little
Describe type 2 and 3 very basic 1 word
Type I, Monoclonal IgM or IgG, most pts asymptomatic
2 - mixed
3 - polyclonal
What HCD is the most common?
Occurs in whom?
Alpha
Middle East/Mediterranean men in 20-30s
Common symptoms of Waldenstrom?
How old these peeps and what they come in for?
Gum bleeding
Epistaxis
HS
Purpura
60-70s c/o fatigue related to anemia
When does cryoglobulinemia occur?
When the plasma proteins clump together in the cold
Dissolve when heated
HCD is characterized by what?
M protein w/IgH in serum, urine or both
No Light chains
Lymphoplasmacytic cell proliferative process
What is the best imaging study to do for multiple myeloma?
Skeletal xray surgery
NEVER bone scan
Pts with marked symptomatic hyperviscosity in an emergent situation treated with what?
Plasmapharesis
Treatment for Cryoglobulinemia?
Corticosteroids
Tx underlying HepC w/IFN or ribavirin
Plasmapharesis
What is amyloid protein?
Kappa or lambda Ig Light chain
Name the 4 common CFs of multiple myeloma?
Bone disease —> bone pain
Anemia
Hypercalcemia
Kidney injury
What are the major clinical features of Waldenstrom?
Monoclonal IgM
MYD88 mutated
Indolent disease
What shows Bence-Jones proteins 2/3 of the time w/inc. plasma cells, lymphocytes in BM and vacuolization of plasma cells?
Treatment?
Mu-HCD
Corticosteroids and alkylating agents
Treatment of Waldenstrom?
Tx ONLY if they have anemia, constitutional sx, LAD/HS
Rituximab, cyclophosphamide, dexamethasone
Hallmark of Waldenstrom?
Monoclonal IgM spike on serum PEP in beta globulin region
Sausage linking on funduscopic exam indicates what?
Hyperviscosity syndrome
Which HCD is this: GI tract involvement w/malabsorption, diarrhea, steatorrhea, plasma cell infiltration of the jejunal mucosa?
Alpha-HCD
How do you treat hypercalcemia in multiple myeloma?
How do you treat the rest of the disease?
Bisphosphatase
Thalidomide, dexamethasone, protease inhibitor
What confers a worse prognosis in multiple myeloma?
Abnormality of ch. 17
Multiple copies of ch. 1
IgH involvement (ch. 14)
What is the initial tx for multiple myeloma?
Thalidomide Lenalidomide Protease inhibitor (bortezomib/carfilzomib) W/dexamethasone
