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Hematology > Plasma cell dyscrasias > Flashcards

Plasma cell dyscrasias Flashcards

1
Q

What is the primary treatment for polycythemia vera?

A

Phlebotomy

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2
Q

How do you differentiate between essential thrombocytosis and 51 syndrome?

A

5q syndrome will have myelodysplasia

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3
Q

What is the cause of multiple myeloma?

A

Plasma cell overgrowth and produce M protein

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4
Q

Who usually gets multiple myeloma?

A

Older men

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5
Q

What are the two bits of the light chain abs?

A

Kappa and lambda

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6
Q

What are the Igs that are overproduced in multiple myeloma?

A

IgG, IgA, or IgE

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7
Q

How do you write down diagnosis for multiple myeloma?

A

Ab type + light chain type

e.g. IgG kappa

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8
Q

What is MGUS?

A

Precancerous condition, where there is an idiopathic overproduction of Igs

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9
Q

What is the chance of developing multiple myeloma with MGUS?

A

1% per year

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10
Q

What are the symptoms of MGUS?

A

Asymptomatic

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11
Q

Lytic bone lesions on bone survey = ?

A

Multiple Myeloma

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12
Q

What are the specific ssx of myeloma?

A

ostealgia

paresthesias

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13
Q

What are the criteria for digasnosis for multiple myelioma

A
  1. Urine or serum abnormal monoclonal protein overproduction
  2. End organ damage
  3. presence of plasma cells in plasma or bone marrow
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14
Q

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

A

Hypercalcemia
Lytic bone lesions
Anemia
Renal failure

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15
Q

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

A

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

No end organ damage

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16
Q

What are the two indicators used to stage multiple myeloma?

A

B2M and serum albumin

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17
Q

Stage I multiple myeloma = ?

A

B2M 3.5 g/dL

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18
Q

Stage III multiple myeloma = ?

A

B2M >5.5 mg/L

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19
Q

Is multiple myeloma curable?

A

No

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20
Q

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

A 
I = 62 months
II = 44 months
III = 29 months
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21
Q

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

A

True

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22
Q

What is the common translocation that can cause multiple myeloma?

A

chromosome 14

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23
Q

What is the treatment for smoldering multiple myeloma?

A

Supportive

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24
Q

What is the treatment for active multiple myeloma?

A

Chemo
Steroids
Immunomodulators
(auto bone marrow transplant)

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25
Q

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

A

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

26
Q

When is radiation used in multiple myeloma treatment?

A

Lytic bone lesions

27
Q

What is monitored during smoldering myeloma?

A

Quantitative igs
CBC
Annual bone surveys

28
Q

What is the basis for choosing drugs in multiple myeloma?

A

Based on risk and eligibility of stem cell transplant

29
Q

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

A

Dexamethasone

30
Q

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

A

melphalan

31
Q

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

A

Prolongation in survival

32
Q

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

A

Thalidomide

Lenalidomide

33
Q

What is plasmacytoma?

A

Bone or soft tissue mass that does not produce Igs

34
Q

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

A

vertebra
Pelvis
Femur
Humerus

35
Q

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

A

Bone

36
Q

How do you diagnose plasmacytoma?

A

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

Normal skeletal survey

37
Q

What is the treatment for plasmacytomas?

A

radiation / surgery

38
Q

What is the prognosis of bone plasmacytomas?

A

20% disease free at 10 years

39
Q

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

A

Extramedullary since it is more likely to be localized

40
Q

What is amyloidosis?

A

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

41
Q

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

A

False

42
Q

What are the usual organs usually affected by amyloidosis?

A

Kidneys

Cardiomyopathy

43
Q

What percent of pts with myeloma have concurrent amyloidosis?

A

10%

44
Q

What is the stain used for definitive diagnosis for amyloidosis?

A

Congo red with red-green birefringence under polarized light

45
Q

What are the two screens for amyloidosis?

A

MPEV + urine IF and serum light chain

46
Q

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

A

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

47
Q

What is the most important complication of primary amyloidosis?

A

Cardiac involvement

48
Q

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

A
  • Cardiac involvement
  • Autonomic neuropathy
  • Associated with multiple myeloma
49
Q

What is the treatment for primary amyloidosis?

A

No optimal treatment

Try to retard further deposition of amyloid

50
Q

What is Waldenstrom’s macroglobulinemia?

A

Excess IgM in bone blood

51
Q

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

A

Waldenstrom’s has a lymphoma component

52
Q

What are the complication of waldenstrom’s?

A

Neuropathy
GI bleed
Hyperviscosity syndrome

53
Q

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

A

Positive

54
Q

How do you diagnose Waldenstrom’s?

A

IgM monoclonal gammopathy

Bone marrow > 10% monoclonal lymphocytes and plasma cell differentiation

55
Q

What is the treatment for waldenstrom’s?

A 
Asymptomatic = observe
Symptomatic = chemo/plasmapheresis
56
Q

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

A

1-5 years

57
Q

True or false: Waldenstrom’s is incurable

A

True

58
Q

What are the presenting ssx of plasmacytoma?

A

Localized pain (especially if found in bone)

59
Q

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

A

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

60
Q

What is the “classical” presentation of amyloidosis?

A

Raccoon eyes

Hematology (32 decks)

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