EXAM #3: PLASMA CELL DYSCRASIAS Flashcards

1
Q

What is Multiple Multiple Myeloma?

A

Hyperproliferation of plasma cells

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2
Q

What is the abnormal protein that is produced in Multiple Myeloma?

A

M-protein i.e. elevation of monoclonal protein

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3
Q

What are the most common types of Multiple Myeloma?

A

1) IgG
2) IgA
3) Light chain only

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4
Q

What are the two types of light chains?

A

Kappa or Lambda

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5
Q

What is the difference between MGUS and Myeloma?

A

MGUS is “pre-cancerous”

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6
Q

What are the symptoms of Multiple Myeloma?

A

1) Lytic lesions on BONE SURVEY i.e. x-ray
2) Increased risk of fracture
3) Paresthesias

“Punched out” lesions

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7
Q

What are the criteria for diagnosis of Multiple Myeloma?

A
  • Elevated serum monocloncal protein
  • Abnormal plasma cells in bone marrow
  • End organ damage
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8
Q

What are the indications of end organ damage?

A

1) Increased Ca++
2) Lytic bone lesions
3) Anemia
4) Renal failure

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9
Q

What is smoldering myeloma? How does this differ from MGUS?

A
  • Smoldering is asymptomatic Multiple Myeloma without end organ damage and a monoclonal antibody concentration greater than 3 g/dL
  • MGUS increased M-protein but less than 3 g/dL
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10
Q

What lab values are used for determining the stage of Multiple Myeloma?

A
  • Serum albumin

- B2M

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11
Q

What is the general trend for serum albumin and B2M for staging of Multiple Myeloma?

A

Worse prognosis for:

  • Decreasing albumin
  • Increasing B2M
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12
Q

What chromosome is commonly mutated in Multiple Myeloma?

A

Chromosome 14

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13
Q

What is the “treatment” for smoldering Multiple Myeloma?

A

Watch and wait

  • SPEP every 3-6 months
  • Annual bone survey
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14
Q

Generally, how is Multiple Myeloma treated?

A
Chemotherapy 
Steroids 
Immunomodulators 
BM transplant 
Bisphosphonates/ Rad.
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15
Q

What is the difference between autologous and allogenic stem cell transplant?

A
Auto= from oneself* 
Allo= from matched donor

**This is what is more commonly done now

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16
Q

What is the goal of BM transplant?

A

Prolongation of survival

17
Q

What drugs are used as maintenance therapy after auto stem cell transplant?

A

Thalidomide

Lenalidomide

18
Q

What is a Plasmacytoma?

A

Single plasma cell tumor in the bone

19
Q

What lab value is characteristic of Plasmacytoma?

A

Low or no levels of M-protein in the serum or urine

20
Q

What type of Plasmacytoma has a better prognosis?

A

Extramedullary

21
Q

How is Plasmacytoma diagnosed?

A

1) Biposy proven lesion of bone or soft tissue
2) Normal bone survey
3) No end organ damage
4) Bone marrow with no evidence of plasma cells

22
Q

What is the treatment for Plasmacytoma?

A
  • Radiation

- Surgery

23
Q

What is the prognosis of Plasmacytoma?

A

Poor, most progress to Multiple Myeloma in 2 years

24
Q

What is Amyloidosis?

A
  • Amyloid = misfolded protein in the extracellular space

- Primary amyloidosis is the deposition of AL amyloid, which is derived from Ig light chain

25
Q

How is Amyloidosis diagnosed?

A

1) SPEP
2) Urine free light chains
3) Serum free light chain

26
Q

What is the definitive diagnosis for Amyloidosis?

A

Congo Red Stain with red-green birefringence on tissue biopsy

27
Q

What are the poor prognostic factors of Amyloidosis?

A

1) Cardiac*
2) ANS neuropathy
3) Liver involvement w/ hyperbilirubinemia
4) Lack of suppression of underlying clonal disease

28
Q

What is the current treatment for Amyloidosis?

A

Prevention of further amyloid deposition

29
Q

What is Waldenstrom’s Macroglobulinemia?

A

Excess of IgM with lymphoma

30
Q

What is the key symptom of Waldenstrom’s Macroglobulinemia?

A

Hyperviscosity Syndrome

31
Q

How is Waldenstrom’s Macroglobulinemia diagnosed?

A

1) IgM monoclonal gammopathy

2) Lymphocyte involvement?

32
Q

What is the treatment for Waldenstrom’s Macroglobulinemia?

A

Chemotherapy and plasmapharesis for symptomatic Waldenstrom’s Macroglobulinemia