Plasma cell dyscrasias Flashcards Preview

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Flashcards in Plasma cell dyscrasias Deck (60):
1

What is the primary treatment for polycythemia vera?

Phlebotomy

2

How do you differentiate between essential thrombocytosis and 51 syndrome?

5q syndrome will have myelodysplasia

3

What is the cause of multiple myeloma?

Plasma cell overgrowth and produce M protein

4

Who usually gets multiple myeloma?

Older men

5

What are the two bits of the light chain abs?

Kappa and lambda

6

What are the Igs that are overproduced in multiple myeloma?

IgG, IgA, or IgE

7

How do you write down diagnosis for multiple myeloma?

Ab type + light chain type

(e.g. IgG kappa)

8

What is MGUS?

Precancerous condition, where there is an idiopathic overproduction of Igs

9

What is the chance of developing multiple myeloma with MGUS?

1% per year

10

What are the symptoms of MGUS?

Asymptomatic

11

Lytic bone lesions on bone *survey* = ?

Multiple Myeloma

12

What are the specific ssx of myeloma?

ostealgia
paresthesias

13

What are the criteria for digasnosis for multiple myelioma

1. Urine or serum abnormal monoclonal protein overproduction
2. End organ damage
3. presence of plasma cells in plasma or bone marrow

14

What are the four criteria for end organ damage with multiple myeloma?

Hypercalcemia
Lytic bone lesions
Anemia
Renal failure

15

What are smoldering myelomas? What is the criteria for diagnosis?

Serum monoclonal protein >3 g/dL or >10%

No end organ damage

16

What are the two indicators used to stage multiple myeloma?

B2M and serum albumin

17

Stage I multiple myeloma = ?

B2M 3.5 g/dL

18

Stage III multiple myeloma = ?

B2M >5.5 mg/L

19

Is multiple myeloma curable?

No

20

What are the median survival times for Stage I-III multiple myeloma?

I = 62 months
II = 44 months
III = 29 months

21

True or false: multiple myeloma can be caused by translocation of genes

True

22

What is the common translocation that can cause multiple myeloma?

chromosome 14

23

What is the treatment for smoldering multiple myeloma?

Supportive

24

What is the treatment for active multiple myeloma?

Chemo
*Steroids*
Immunomodulators
(auto bone marrow transplant)

25

What is the basis for autogenic transplant for multiple myeloma? Is this curative?

Hope to get rid of the bad plasma cells, but this is still not curative

26

When is radiation used in multiple myeloma treatment?

Lytic bone lesions

27

What is monitored during smoldering myeloma?

Quantitative igs
CBC
Annual bone surveys

28

What is the basis for choosing drugs in multiple myeloma?

Based on risk and eligibility of stem cell transplant

29

What is the drug that is used to treat multiple myeloma?

Dexamethasone

30

What drug should you avoid giving multiple myeloma pts if they're transplant eligible?

melphalan

31

What is the goal of stem cell transplants with multiple myeloma?

Prolongation in survival

32

What are the two maintenance therapies after autotransplant in multiple myeloma pts?

Thalidomide
Lenalidomide

33

What is plasmacytoma?

Bone or soft tissue mass that does not produce Igs

34

What are the four bones (in descending order) where plasmacytomas are found?

vertebra
Pelvis
Femur
Humerus

35

Which is more likely, a bone plasmacytoma or soft tissue?

Bone

36

How do you diagnose plasmacytoma?

Biopsy proven solitary lesion of bone or soft tissue clonal plasma cells

Normal skeletal survey

37

What is the treatment for plasmacytomas?

radiation / surgery

38

What is the prognosis of bone plasmacytomas?

20% disease free at 10 years

39

Which has a better prognosis: bone plasmacytomas, or extramedullary plasmacytomas? Why?

Extramedullary since it is more likely to be localized

40

What is amyloidosis?

Protein conformational disorder associated with clonal plasma cell dyscrasia, causing deposition of fragments

41

True or false: there is a good correlation between the amount of amyloid and the degree of impairment of organ function

False

42

What are the usual organs usually affected by amyloidosis?

Kidneys
Cardiomyopathy

43

What percent of pts with myeloma have concurrent amyloidosis?

10%

44

What is the stain used for definitive diagnosis for amyloidosis?

Congo red with red-green birefringence under polarized light

45

What are the two screens for amyloidosis?

MPEV + urine IF and serum light chain

46

What are the tissues that are sampled for amyloid in primary amyloidosis?

Bone marrow (50% sens) or fat pad (70% sens)

47

What is the most important complication of primary amyloidosis?

Cardiac involvement

48

What are the poor prognostic factors for primary amyloidosis? (3)

-Cardiac involvement
-Autonomic neuropathy
-Associated with multiple myeloma

49

What is the treatment for primary amyloidosis?

No optimal treatment

Try to retard further deposition of amyloid

50

What is Waldenstrom's macroglobulinemia?

Excess IgM in bone blood

51

What is the difference between IgM producing multiple myeloma and Waldenstrom's?

Waldenstrom's has a lymphoma component

52

What are the complication of waldenstrom's?

Neuropathy
GI bleed
*Hyperviscosity syndrome*

53

What is the coomb's test result with Waldenstrom's?

Positive

54

How do you diagnose Waldenstrom's?

IgM monoclonal gammopathy

Bone marrow > 10% monoclonal lymphocytes and plasma cell differentiation

55

What is the treatment for waldenstrom's?

Asymptomatic = observe
Symptomatic = chemo/plasmapheresis

56

How long does it take for waldenstrom's take to progress?

1-5 years

57

True or false: Waldenstrom's is incurable

True

58

What are the presenting ssx of plasmacytoma?

Localized pain (especially if found in bone)

59

What is the threshold of serum monoclonal antibody to have a diagnosis of multiple myeloma, as opposed to MGUS?

>3g and/or 10-60% bone marrow clonal plasma cells

60

What is the "classical" presentation of amyloidosis?

Raccoon eyes