Plasma Cell Neoplasms (CH8, PFC and Gorczyca) Flashcards

1
Q

What is the immunophenotype of

normal plasma cells ?

A
  • bright for CD38 and CD138
    • Note: CD138 is more sensitive and specific for plasma cells but it can be positive in non-hematopoietic malignancies as well
  • CD19+, CD27+, CD43+, CD81+
  • negative for CD20, CD22, CD56, CD117, CD200, HLA-DR and surface k/l
    • CD27 is a memory associated antigen
    • benign plasma cells can be dimly positive for CD45
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2
Q

What are the immunophenotypic changes

for abnormal/neoplastic plasma cells ?

A
  • gain expression of additional markers by flow:
    • CD28, CD33, CD56, and CD117
  • usually show weaker CD38 and brighter CD138 as compared to normal plasma cells
    *positive for CD43, MUM1, OCT2 and BOB1
  • By IHC
    • may be positive for EMA and Cyclin D1
    • Occasional cases may be positive for CD20 and Pax-5
    • rarely can be positive for CD19, CD20, CD27, CD33, HLA-DR and CD10 (IHC and flow), CD4 (may mimic AML)
    • occasional cases may show loss of CD38 or CD138 expression
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3
Q

What is the importance of lack of CD56

expression by flow cytometry in neoplastic P.C. ?

A
  • associated with fewer osteolytic lesions
  • lambda light chain expressionn
  • poorer prognosis
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4
Q

What is the expression of

CD28 in neoplastic plasma cells ?

A
  • a gain of CD28 expression is seen about 50% of plasma cell neoplasms
  • it correlates with poorer overall survival
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5
Q

What does the loss of CD27 expression

correlate with ?

A
  • correlates with disease progression and worse overall prognosis
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6
Q

What aberrant expression of markers can be rarely seen

with plasma cell neoplasms ?

A
  • positivity for:
    • CD10
    • CD33
    • CD23
    • HLA-DR
  • lack of CD38
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7
Q

What is often seen with CD19+, CD20+

plasma cell myeloma ?

A
  • rare to have both positive
  • usually positive for CD27
  • surface immunoglobulins are usually negative
    • can be dim rarely
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8
Q

What does the expression of CD45 by

plasma cells correlate with ?

A
  • proliferation rate
    • in addition, proliferating myeloma cells are characterized by CD11a and BCL2 dim+
  • CD45 expression
    • recently shown to be an independent predictor of inferior overall survival among newly diagnosed plasma cell myelomas
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9
Q

How common in IgM MM ?

A
  • very rare (< 0.5% of all myeloma patients)
  • usually negative for:
    • CD20
    • CD56
    • CD117
  • may show aberrant expression of:
    • CD19
    • CD45
    • Pax-5

NOTE: majority of these cases end up being positive for t(11;14) and Cyclin D1 by IHC

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10
Q

What is typically seen by flow cytometry

in patients with MGUS ?

A
  • two populations of plasma cells
    • polyclonal- normal
    • monoclonal- neoplastic
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11
Q

What is the differential diagnosis

for PCM ?

A
  • reactive plasmacytosis
  • MGUS
  • LPL/WM
  • Mantle cell lymphoma
  • B cell lymphomas with extensive plasmacytic differentiation
  • Metastatic carcinoma (CD138 +)
  • AML
  • Pure erythroid leukemia (also CD45 -)
  • B cell lymphomas with plasmacytic morphology
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12
Q

What conditions can lead to polytypic

plasmacytosis ?

A
  • HIV infection
  • chronic infection
  • inflammatory disorders
  • autoimmune disorders
    • SLE, Sjogren’s syndrome
  • drug eruptions
  • Neoplasms
    • AITL
    • P. vera
    • Aplastic anemia
    • Hodgkin lymphoma
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13
Q

How can a plasma cell myeloma and a B cell

lymphoma with extensive plasmacytic differentiation

be differentiated by flow ?

A
  • demonstrate that there is a clonal B cell population with monotypic expression of the same light chain immunoglobulin as the plasma cells.
  • Lack of significant M protein
    • Ex: Marginal zone lymphoma will not have a significant M protein and the clinical and radiographic findings will be different as well
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14
Q

What flow findings favor a PCM vs.

a B cell lymphoma with plasmacytic differentiation ?

A
  • expression of two different light chains
  • Lack of CD19 and CD45 expression
  • presence of CD56 and or CD117 expression
  • increased FSC
    • plasma cells accompanying a small B cell lymphoma tend to be smaller with lower FSC
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15
Q

What can occasionally be seen

in Castleman Disease ?

A
  • plasma cells may be monotypic IgA lambda
  • must differentiate this from a PCM
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16
Q

What carcinoma can share similar IHC

features to PCM ?

A
  • poorly differentiated carcinoma with neuroendocrine features can be CD56 + and CD45-
17
Q

What immunophenotype can be seen

with plasma cell leukemia ?

A
  • have reduced expression of cell adhesion molecule CD56
  • less often positive for:
    • CD71, CD117, and HLA-DR
  • more often positive for:
    • CD19, CD20, CD23 and CD45
  • IMP:
    • higher proportion of light chain only, IgD, or IgE myeloma present as plasma cell leukemias
18
Q

In what conditions can you see peripheral blood

with reactive plasmacytosis ?

A
  • Infections
    • HIV, Hepatitis A, staphyloccocal sepsis
  • autoimmune disorders
  • drug reactions
  • IL-6 producing malignancies
  • AITL
    • notorious for producing an exuberant plasmacytosis
  • PTCL, NOS
19
Q

What is the definition of a monoclonal gammopathy?

A
  • any situation in which a clonal paraprotein is present in the blood or urine
  • indicative of a clonal lymphoid or plasma cell proliferation
  • reactive populations by definition should not produce a paraprotein
20
Q

What are the morphologic features of a plasmablastic cell?

A
  • fine reticulin chromatin pattern with no clumping
  • large nucleus
  • prominent nucleolus
  • scant cytoplasm
  • nor minute hof region
21
Q

What is the best method of quantification of plasma cells?

A
  • core biopsy
  • the aspirate smear and flow cytometry tend to underestimate the percentage of plasma cells
22
Q

What other entities can CD38 be positive in?

A
  • CD38 is a relatively broad expressed marker
  • seen in immature CD34+ precursors (myeloblasts, lymphoblasts)
  • B cell progenitors (hematogones)
  • monocytes
  • NK cells
  • subset of activated B and T cells
23
Q

What is the pattern of CD38 expression on B cells?

A
  • expression is high in B cell committed precursors and it gradually decreases on immature B lymphocytes in BM
  • no expression is seen in naive B cells
  • mature activated B cells start expression CD38 again in high levels typically seen on germinal center B cells
  • maturation to memory B cells leads to down regulation of CD38
  • progression to plasma cell lineage leads to high expression again.
24
Q

What can cause down regulation/expression of CD138?

A
  • often bright expression on plasma cells
  • down-regulation is often seen in aged samples or those exposed to Heparin
25
Q

What is the pattern of CD19 expression in normal plasma cells?

A
  • generally heterogeneous
  • minor subset of plasma cells may be CD19 negative (up to 30%)
    Note: also a small subset of benign plasma cells may be CD56+ and negative for CD45 and CD81.
26
Q

The expression of CD23 is seen in association with what?

A
  • expression of CD23 can be seen in 10% of PCM associated with t(11;14)
27
Q

What are some phenotypic differences between LPL or CLL and PCM?

A
  • in contrast to lymphoid cells, PCM are CD19- and HLA-DR -
  • PCM also often aberrant expression of CD56 and CD117
  • Occasional cases of PCM may show dim expression of surface light chains