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Boards.CP.Heme > Plasma Cell Neoplasms > Flashcards

Plasma Cell Neoplasms Flashcards

1
Q

Where does Solitary Extraosseous (extramedullary) Plasmacytoma most commonly arise?

  • What % have detectable M protein
  • What is the likelyhood of developing MM
A

Nasal cavity, oropharynx, or larynx

  • No M protein
  • Most do NOT develop MM
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2
Q

T/F: Plasma cell leukemia presents abruptly and follows an aggressive course; and 1/2 cases are de novo.

A

True

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3
Q

Some cases of MM are bcl-1(+)(cyclin-D1), what does this correlate with?

A

t(11;14) translocation

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4
Q

MM Prognosis factor:

-B2-microglobulin

A

Higher levels - Adverse

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5
Q

In patients with MGUS after 20 years, how many will develop overt Myeloma?

A

33%

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6
Q

What is the most common paraprotein idiotype (light chain) in MM?

A

kappa

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7
Q

Multiple Myeloma IHC:

-Positive for (5)

A
  • CD38
  • CD138
  • CD56
  • kappa or lambda (cytoplasmic)
  • PCA1
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8
Q

What % of myeloma cases are Nonsecretory?

A

5%

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9
Q

What is the risk per year of progression to MM or another plasma cell neoplams in patients with MGUS?

A

0.5-1%

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10
Q

Myeloma prognosis by FISH:

-Shortest survival (24 months) (3)

A
  • t(4;14)
  • t(14;16)
  • 17p13 Deletion
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11
Q

MM Molecular/Cytogenetics:

-Most common Rearrangement

A

CCND1/IgH fusion

-t(11;14)

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12
Q

Diagnostic criteria for Plasma cell leukemia.

A

> 20% plasma cells in peripheral blood

>2.0x10^9/L

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13
Q

MGUS is found in __% of adults over the age of 50, and __% of adults over the age of 70.

A
  • 3% over 50

- 5% over 70

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14
Q

Diagnostic criteria for Asymptomatic (smoldering) Myeloma.

A

-M protein >3.0 g/dL
OR
->10% clonal plasma cells in BM

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15
Q

What % of patients with an M protein are classified as MGUS?

A

60%

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16
Q

Where does Solitary Osseous Plasmacytoma (solitary plasmacytoma of bone) most commonly arise?

  • What % have detectable M protein
  • What is the likelyhood of developing MM
A

Vertebrae, Ribs, and Pelvis

  • 50% have M protein
  • 75% develop MM in 10 years
17
Q

Criteria for Symptomatic MM.

A 
M protein (any amount)
\+
Clonal plasma cell in BM (any %) or plasmacytoma
\+
Organ impairment (CRAB)
18
Q

Multiple Myeloma - Renal Manifestations:

-Mechanism of Tubular Injury

A

Hypercalcemia and/or Hyperuricemia induced Tubular Injury

19
Q

What is the aberrant IHC finding in plasma cell leukemia?

A

CD56 Negative

20
Q

Multiple Myeloma - Renal Manifestations:

-AL Amyloidosis association

A

Lambda Light Chains

21
Q

Myeloma prognosis by FISH:

-Longest survival (>50 months)

A
  • t(11:14)

- No anomalies

22
Q

What does CRAB stand for in symptomatic MM.

A

Organ Impairment:

  • Calcium (High)
  • Renal insufficiency
  • Anemia
  • Bone lesions
23
Q

Multiple Myeloma IHC:

-Negative for (6)

A
  • CD19
  • CD20
  • CD21
  • CD22
  • CD45
  • sIg
24
Q

What cytogenetic abnormality has a high incidence in Plasma cell leukemia?

A

monosomy 13

25
Q

Plasma Cell Neoplasm Demographics:

  • Age
  • Race
  • Sex
  • Family Histor
A
  • 70 y/o (median)
  • Blacks>Whites
  • Males>Females
  • 1st degree relative - 3-4X Increased
26
Q

Multiple Myeloma - Renal Manifestations:

-kappa light chain association

A

Light Chain Deposition disease

27
Q

Diagnostic criteria for MGUS:

  • Monoclonal gammopathy
  • BM involvement
  • Organ Impairment
  • B-cell neoplasm
A

Diagnostic criteria for MGUS:

  • Monoclonal gammopathy <3.0 g/dL
  • <10% plasma cells in BM
  • NO CRAB
  • NO B-cell neoplasm
28
Q

What is the most common paraprotein isotype (heavy chain) in MM?

  • 2nd
  • 3rd
A

IgG (55%)

  • IgA (22%)
  • none-light chain only (18%)
29
Q

MM Molecular/Cytogenetics:

-Most common abnormality

A

14q32 (IgH)

  • MM (>70%)
  • MGUS (50%)
30
Q

Myeloma prognosis by FISH:

-Intermediate survival (42 months) (3)

A

13q14 deletions alone

Boards.CP.Heme (15 decks)

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