Plasma Cell Disorders - Hubbard

Question 1

MGUS defined as what?

Tx

Answer 1

BM monoclonal plasma cells < 10% w/serum M-protein < 3 g/L
NO end organ damage

Observe brah, duh

Question 2

How do you diagnose Alpha-HCD?

Treatment?

Answer 2

Serum immunofixation showing monoclonal chain, no light chain

Antibiotics and CHOP

Question 4

What shows initially a lymphoma like illness, HS and LAD in 60%, anemia in 80%?

Treatment?

Answer 4

Gamma-HCD

Chemo and some drugs

Question 6

Rouleux formation w/ESR above 100 may be seen in what?

Tx?

Answer 6

Waldenstrom’s

Rituximab

Question 7

What are the major clinical manifestations of cryoglobulinemia

Answer 7

Vasculitis, pain, purpura, Raynaud’s, cyanosis, ulceration, and sloughing of skin on exposure to cold

Question 8

Which type of cryoglobulinemia is most common? Describe it a little

Describe type 2 and 3 very basic 1 word

Answer 8

Type I, Monoclonal IgM or IgG, most pts asymptomatic

2 - mixed

3 - polyclonal

Question 10

What HCD is the most common?

Occurs in whom?

Answer 10

Alpha

Middle East/Mediterranean men in 20-30s

Question 12

Common symptoms of Waldenstrom?

How old these peeps and what they come in for?

Answer 12

Gum bleeding
Epistaxis
HS
Purpura

60-70s c/o fatigue related to anemia

Question 13

When does cryoglobulinemia occur?

Answer 13

When the plasma proteins clump together in the cold

Dissolve when heated

Question 14

HCD is characterized by what?

Answer 14

M protein w/IgH in serum, urine or both
No Light chains
Lymphoplasmacytic cell proliferative process

Question 15

What is the best imaging study to do for multiple myeloma?

Answer 15

Skeletal xray surgery

NEVER bone scan

Question 17

Pts with marked symptomatic hyperviscosity in an emergent situation treated with what?

Answer 17

Plasmapharesis

Question 18

Treatment for Cryoglobulinemia?

Answer 18

Corticosteroids
Tx underlying HepC w/IFN or ribavirin
Plasmapharesis

Question 21

What is amyloid protein?

Answer 21

Kappa or lambda Ig Light chain

Question 22

Name the 4 common CFs of multiple myeloma?

Answer 22

Bone disease —> bone pain
Anemia
Hypercalcemia
Kidney injury

Question 24

What are the major clinical features of Waldenstrom?

Answer 24

Monoclonal IgM
MYD88 mutated
Indolent disease

Question 27

What shows Bence-Jones proteins 2/3 of the time w/inc. plasma cells, lymphocytes in BM and vacuolization of plasma cells?

Treatment?

Answer 27

Mu-HCD

Corticosteroids and alkylating agents

Question 28

Treatment of Waldenstrom?

Answer 28

Tx ONLY if they have anemia, constitutional sx, LAD/HS

Rituximab, cyclophosphamide, dexamethasone

Question 30

Hallmark of Waldenstrom?

Answer 30

Monoclonal IgM spike on serum PEP in beta globulin region

Question 31

Sausage linking on funduscopic exam indicates what?

Answer 31

Hyperviscosity syndrome

Question 32

Which HCD is this: GI tract involvement w/malabsorption, diarrhea, steatorrhea, plasma cell infiltration of the jejunal mucosa?

Answer 32

Alpha-HCD

Question 34

How do you treat hypercalcemia in multiple myeloma?

How do you treat the rest of the disease?

Answer 34

Bisphosphatase

Thalidomide, dexamethasone, protease inhibitor

Question 36

What confers a worse prognosis in multiple myeloma?

Answer 36

Abnormality of ch. 17
Multiple copies of ch. 1
IgH involvement (ch. 14)

Question 38

What is the initial tx for multiple myeloma?

Answer 38

Thalidomide
Lenalidomide
Protease inhibitor (bortezomib/carfilzomib)
W/dexamethasone

Question 47

What plasma cell disorder can occur in any age group, not the normal over 50 group?

Answer 47

Heavy chain disease

Question 48

What is the treatment for a solitary plasmacytoma?

What do 30-50% progress to?

Answer 48

Radiation

Disseminated Multiple myeloma

Question 50

When diagnosing a plasma cell disorder, what is the most important test to run as a 1st step in the workup?

Answer 50

Serum protein electrophoresis

Question 52

What does a bone marrow aspirate biopsy of multiple myeloma show?

Answer 52

Increased in marrow plasma cells > 10%

Look abnormal