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Heme / Onc > SM_266b: Multiple Myeloma > Flashcards

SM_266b: Multiple Myeloma Flashcards

1
Q

This is a ___

A

Plasma cell

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2
Q

Describe plasma cells

A

Plasma cell

  • Perinuclear hof
  • Fried egg appearance
  • Clock face chromatin pattern in the nucleus
  • Express bright CD38, CD138, and cytoplasmic immunoglobulins
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3
Q

B cells are antigen selected in the ___ and some differentiate into ___

A

B cells are antigen selected in the germinal center and some differentiate into plasma cells

  • Plasma cells are terminally differentiated B cells
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4
Q

Normal plasma cells produce ___

A

Normal plasma cells produce immunoglobulins (antibodies) that are highly specific for antigen

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5
Q

Immunoglobulins consist of ____ and ____

A

Immunoglobulins consist of light chain and heavy chain

  • Light chain: kappa or lambda
  • Heavy chain: IgG, IgM, IgE, IgA, or IgD
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6
Q

B cells have ___ Ig while plasma cells have ___ Ig and ___ Ig

A

B cells have surface Ig while plasma cells have cytoplasmic Ig and secreted Ig

  • Plasma cells are responsible for producing immunoglobulins
  • Immunoglobulins are critical for immunity
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7
Q

Each plasma cell expresses ___ light chains

A

Each plasma cell expresses kappa OR lambda light chains

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8
Q

In normal reactive plasma cell population, kappa:lambda ratio is about ___

A

In normal reactive plasma cell population, kappa:lambda ratio is about 2:1

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9
Q

____ can help identify a clonal plasma cell population

A

Skewing of the kappa:lambda ratio can help identify a clonal plasma cell population

  • Flow cytometry and immunohistochemistry can be used to evaluate light chains
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10
Q

Plasma cell neoplasm is ___

A

Plasma cell neoplasm is uncontrolled growth and expansion of a single clone of plasma cells

  • Clonal plasma cells typically produce clonal immunoglobulin
  • Presence of clonal plasma cells is NOT sufficient for diagnosis of multiple myeloma
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11
Q

Monoclonal gammopathy is ___

A

Monoclonal gammopathy is a monoclonal immunoglobulin in the blood or urine identified by serum or urine protein electrophoresis or immunofixation

  • Also called paraprotein, M protein, M component, M spike
  • Not always IgM
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12
Q

Describe serum protein electrophoresis

A

Serum protein electrophoresis

  • Serum proteins separated by running on a gel electrophoresis
  • Bands on gel are quantified (peaks)
  • Immunofixation used to identify heavy and light chain
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13
Q

Monoclonal gammopathy of undetermined significance is ___ and ___

A

Monoclonal gammopathy of undetermined significance is most common plasma cell disorder and is asymptomatic

  • Monoclonal expansion of plasma cells with associated monoclonal gammopathy
  • < 10% of clonal plasma cells in the bone marrow
  • No evidence of end organ damage
  • 1-2% progress to multiple myeloma per year
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14
Q

Multiple myeloma is ____ with ____

A

Multiple myeloma is bone marrow-based malignant proliferation of plasma cells (10-60%) with evidence of organ or tissue damage

  • Production of a monoclonal immunoglobulin
  • If plasma cells > 60% of bone marrow, other findings not necessary for diagnosis
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15
Q

Multiple myeloma involves ___ with ___, ___, ___, and ___

A

Multiple myeloma involves evidence of end organ damage including hypercalcemia, renal insufficiency, anemia, and bone lesions

  • CRAB
  • Osteolytic lesions identified on radiographic imaging
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16
Q

Describe epidemiology and etiology of multiple myeloma

A

Multiple myeloma

  • Peak incidence at 65-70 years old
  • Etiology unknown
  • Predisposing factors: family history of myeloma or monoclonal gammopathy of undetermined significance, decreased immunity, or radiation
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17
Q

Smoldering myeloma is ___ with ___

A

Smoldering myeloma is bone marrow-based malignant proliferation of plasma cells (10-60%) with no evidence of organ or tissue damage

  • Production of a monoclonal immunoglobulin
  • 75% progress to symptomatic myeloma
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18
Q

Multiple myeloma is diagnosed via ___

A

Multiple myeloma is diagnosed via aspirate or core biopsy of bone marrow

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19
Q

This is bone marrow biopsy showing ___

A

Multiple myeloma

20
Q

____ immunostain highlights plasma cells

A

CD138 immunostain highlights plasma cells

21
Q

Antibodies against ___ can detect clonal plasma cell populations

A

Antibodies against light chains can detect clonal plasma cell populations

22
Q

Bone marrow aspirate in multiple myeloma shows ___

A

Bone marrow aspirate in multiple myeloma shows increased plasma cells

23
Q

Dutcher bodies and Russell bodies are characteristic of ____

A

Dutcher bodies and Russell bodies are characteristic of multiple myeloma

24
Q

Plasma cells are identified with ___ and ___

A

Plasma cells are identified with CD138 and CD38

25
Normal plasma cells are \_\_\_\_, \_\_\_\_, and \_\_\_\_
Normal plasma cells are CD19+, CD56-, and polyclonal
26
Neoplastic plasma cells are \_\_\_\_, \_\_\_\_, and \_\_\_\_
Neoplastic plasma cells are CD19-, CD56+, and monoclonal
27
Myekoma cells produce factors that \_\_\_
Myekoma cells produce factors that drive bone destruction * Expression of MIP1-alpha -\> activation of RANKL -\> activates osteoclasts (cells that resorb bone)
28
Myeloma cells produce \_\_\_
Myeloma cells produce IL-6 * Also dependent on IL-6 for growth, survival, and immunoglobulin
29
Multiple myeloma involves \_\_\_\_
Multiple myeloma involves massive production of immunoglobulins * Contributes to pathology of disease * IgG is 55%, IgA is 25% * Clonal plasma cells often produce light chains in excess of whole Ig molecule * 20% only produce light chains
30
Quantitative serum immunoglobulin analysis is useful for ___ and \_\_\_
Quantitative serum immunoglobulin analysis is useful for diagnosis and disease monitoring of plasma cell neoplasms * IgG: 800-1600 mg/dL * IgA: 200-400 mg/dL * IgM: 100-200 mg/dL
31
Monoclonal light chains are smaller and can be excreted into the urine (\_\_\_\_\_)
Monoclonal light chains are smaller and can be excreted into the urine (Bence-Jones proteins)
32
Immunoglobulins and immunoglobulin components produced by myeloma cells are often ___ and can be \_\_\_
Immunoglobulins and immunoglobulin components produced by myeloma cells are often abnormal and can be nephrotoxic
33
Multiple myeloma involves misfolded ____ leading to \_\_\_\_
Multiple myeloma involves misfolded monoclonal light chains deposited in extracellular tissues (amyloid) * Beta pleated sheat form from some clonal light chains * Causes nephrotic syndrome
34
Congo red stain shows ___ under polarization in the presence of amyloid in multiple myeloma
Congo red stain shows apple green birefringence under polarization in the presence of amyloid in multiple myeloma
35
Multiple myeloma presents with ___ that appears as \_\_\_
Multiple myeloma presents with anemia that appears as Rouleaux formation * Anemia secondary to bone marrow suppression and renal failure * Rouleaux: RBCs stacking like coins, results from alteration of negative charge of RBCs by immunoglobulins in serum
36
Multiple myeloma presents with \_\_\_\_, \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_
Multiple myeloma presents with anemia, hypercalcemia, lytic bone lesions, renal insufficiency, and amyloidosis
37
Describe clinial findings in multiple myeloma
Multiple myeloma * Anemia * Hypercalcemia (corrected serum Ca \> 11.5 mg/dL): secondary to bone destruction * Bone lytic lesions (punched out): axial skeleton, pathological fractures * Renal insufficiency (elevated serum Cr): secondary to light chain deposition in kidney * Amyloidosis: certain monoclonal light chains can deposit in beta-pleated sheets in tissue, contribute to renal failure and cause multi-organ failure
38
Monoclonal immunoglobulins ___ against bacteria, so people with multiple myeloma most commonly die from \_\_\_
Monoclonal immunoglobulins are NOT protective against bacteria, so people with multiple myeloma most commonly die from infection * Production of other normal immunoglobulins is suppressed: hypogammaglobulinemia
39
Survival in mutliple myeloma is \_\_\_
Survival in mutliple myeloma is variable * Prognostic factors: stage (tumor burden), patient factors, tumor biology, response to therapy
40
Del(17p) in multiple myeloma predicts ___ prognosis
Del(17p) in multiple myeloma predicts poor prognosis
41
Multiple myeloma is treated with \_\_\_
Multiple myeloma is treated with high-dose combination chemo with autologous stem cell transplant * Followed by maintenance therapy
42
Daratumumab is a ___ used to treat multiple myeloma
Daratumumab is a monoclonal antibody targeting CD38 which binds to plasma cells used to treat multiple myeloma * Kills cells directly and helps immune system attack the cells
43
Describe solitary plasmacytoma
Solitary plasmacytoma * Solitary plasmacytoma of bone: 50% have monoclonal gammopathy, 2/3 develop multiple myeloma * Extramedullary (outside of bone): upper respiratory site, cured often, progression to multiple myeloma rare
44
Primary amyloidosis (immunoglobulin light chain amyloidosis) is \_\_\_
Primary amyloidosis (immunoglobulin light chain amyloidosis) is deposition of immunoglobulin light chains (kappa or lambda) which form a beta-pleated sheet in various tissues * Can be seen with or without multiple myeloma
45
Waldenstrom's macroglobulinemia is \_\_\_
Waldenstrom's macroglobulinemia is IgM monoclonal gammopathy in the presence of lymphoplasmocytic lymphoma * Serum hyperviscosity caused by IgM pentamers * Bleeding due to platelet dysfunction * Lytic bone lesions are absent * MYD88 gene mutation
46
Neoplastic cells ___ like plasma cells
Neoplastic cells do NOT always look like plasma cells

Heme / Onc (41 decks)

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