Plasma cell dyscrasias Flashcards

(60 cards)

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
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