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AP/CP Board Review Hemepath > Plasma cell neoplasms > Flashcards

Plasma cell neoplasms Flashcards

1
Q

What are some of the key epidemiological

things about Plasma cell myeloma?

A
  • older adults with a median onset of 70 years
  • significantly more common in blacks
  • familial clustering
    • 3-4x more common in first degree relatives
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2
Q

What are the diagnostic criteria

for symptomatic Multiple Myeloma?

A
  • clonal bone marrow plasma cells >10%
  • or biopsy proven plasmacytoma
  • plus greater than or equal to 1 of the following:
    • organ impairment with CRAB
      • high calcium, renal insufficiency, anemia and bone lesions
    • > = 1 of malignant bone markers
      • 60% bone marrow plasma cells
      • serum free light chain ratio >100
      • > 1 focal lesion on MRI
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3
Q

What are the diagnostic criteria

for Asymptomatic (smoldering)

Multiple Myeloma?

A

IMP: both criteria must be met for this diagnosis

  • Serum M protein > 3 g/dL (30 g/L)
    • or urinary M protein of > 500 mg/day and/or clonal bone marrow plasma cells 10-60%
    • or clonal plasma cells 10%
  • absence of myeloma defining evens or amyloidosis
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4
Q

What are the renal manifestations of

Multiple Myeloma?

A
  • Acquired renal Fanconi Syndrome
    • hypercalcemia and hyperuricemia
      • damage the proximal tubules
  • AL amyloidosis
    • often associated with Lambda L.C.
    • deposits in vessels of the glomeruli
  • Myeloma cast nephropathy
    • intratubular crystallization of M protein
    • deposits look angulated and cracked
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5
Q

What are the clinical manifestations of

Fanconi renal syndrome?

A
  • inadequate reabsorption in proximal renal tubules
  • acidosis
  • hypokalemia
  • hyperchloremia
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6
Q

What is the cause of a bleeding diathesis

and the hyperviscosity syndrome in

Multiple Myeloma ?

A
  • M protein interferes with coagulation factors
  • thrombocytopenia can occur due to marrow involvement
  • amyloidosis can lower factor X

Hyperviscosity Syndrome:

  • often with IgM, IgA and IgG3 protein
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7
Q

Why are patients with

Multiple Myeloma prone to infections?

A
  • they have hypogammaglobulinemia
  • this predisposes them to infection with encapsulated organisms
    • Streptococcus pneumoniae
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8
Q

What is the M protein often

encountered in Multiple Myeloma?

A
  • usually heavy chain
    • often IgG (55%)
    • then IgA (22%)
    • IgE is the least common type
  • if it is light chain
    • Kappa is most common
    • BUT IgD myeloma, associated with lambda
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9
Q

What are some of the cytoplasmic

changes seen in Multiple Myeloma?

A
  • Mott cells
  • Flame cells
    • usually with IgA
  • Morula
  • Russell bodies
  • Gaucher-like

Note: none of these are diagnostic for myeloma

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10
Q

What is the immunophenotype of

Multiple Myeloma?

A
  • Plasma cells (+)
    • CD38, CD138, CD56
    • Kappa or Lambda
  • DO not express:
    • CD45 or B cell antigens
      • CD19, CD20, CD21, or CD22 or SIg
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11
Q

What are some unique immunophenotypic markers

that can be expressed by Multiple Myeloma ?

A
  • CD56:
    • suggestive of aggressive behavior
    • absent in plasmablastic lymphoma and plasma cell leukemia
  • BCL-1/Cyclin D1
    • seen in some cases and correlates with t(11;14)
  • Myelomonocytic markers
    • CD117, CD13, CD33, CD11b and CD15
    • seen in 10-30%
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12
Q

What is the prognosis for Multiple Myeloma?

A
  • mean survival is 3-5 years
  • Adverse Factors:
    • High B2 microglobulin
    • High plasma labeling index
    • High stage
    • specific cytogenetics
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13
Q

What is the most common abnormal locus

genetically in Multiple Myeloma?

A
  • 14q32
    • IgH gene
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14
Q

What is the most common structural abnormality

seen in Multiple Myeloma?

A
  • t(11;14)(q13;q32)
    • CCND1/IgH
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15
Q

What FISH findings are high risk,

associated with OS of 3 years?

A
  • del 17p
  • t(14;16)
  • t(14;20)
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16
Q

What are FISH findings associated with

intermediate risk MM, OS 4-5 years?

A
  • del 13
  • t(4;14)
  • hypodiploid
17
Q

What are FISH associated findings for

standard risk MM, OS 8-10 years?

A
  • t(11;14)
  • t(6;14)
  • hyperdiploid
18
Q

What are key findings of Multiple Myeloma

on flow cytometry?

A
  • dim to absent CD45 with bright CD38
  • CD56 v CD19
    • neoplastic cells have dim CD19 (or absent)
    • express CD56
  • cytoplasmic lambda light chain restriction
19
Q

What is the definition of

plasma cell leukemia?

A
  • absolute plasma cell count >2 x 10^9/ L or 20% plasma cells in the blood
  • High incidence of IgE, IgD or light chain myeloma
    • CD20 +
    • CD56 (-)
  • aggressive disease with poor treatment response and survival
20
Q

What is the definition of Solitary Plasmacytoma?

A
  • often seen in the vertebrae, ribs and pelvis
  • two variants are seen
    • SP with no bone marrow plasmacytosis or end organ damage
      • 10% progress to MM
    • SP with minimal marrow plasmacytosis (clonal plasma cells <10%)
      • 60% progress to MM
21
Q

What is the definition of Extraosseous Plasmacytoma?

A
  • commonly arises in the nasal cavity, oropharynx and larynx
  • progression to myeloma less frequent
  • higher rate of minimal marrow involvement
22
Q

What is the general definition of an MGUS?

A
  • monoclonal gammopathy without criteria for a plasma cell neoplasm or presence of a B cell neoplasm capable of producing an M protein
  • seen in 3% of adults over the age of 50 and 5% of adults older than 70
23
Q

What are the diagnostic criteria for

Monoclonal gammopathy of unknown significance?

A
  • Monoclonal gammopathy
    • < 3 g/dL (30 g/L)
  • < 10% of plasma cells in the bone marrow
  • no CRAB
  • no B cell neoplasm
24
Q

What is POEMS syndrome and what is

it associated with?

A

POEMS

  • polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes
  • caused by Osteosclerotic myeloma
    • individuals usually have solitary intraosseous plasmacytomas with thickening of the bony trabeculae
25
Q

What other clinical situations can

POEMS syndrome be seen in ?

A
  • Plasma cell variant Castlemann’s disaease
  • HHV8

AP/CP Board Review Hemepath (12 decks)

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