266 - Multiple Myeloma Flashcards

1
Q

What is the therapy for myeloma?

A

High dose combination therapy with autologous stem cell transplant

  • Prognosis variable*
  • Do more chemo if pt not elligible for stem cell transplant*
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2
Q

What is the significance of Ig light chains in multiple myeloma?

A

Myeloma cells produce more light chain than heavy chain

-> extra light chains floating around

  • Excreted in urine as Bence-Jones proteins
    • Can be nephrotoxic
  • Misfolded light chains -> amyloid
    • -> Nephrotic syndrome
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3
Q

What is the normal kappa:lamba ratio in plasma cells?

What is the significance of a deviation from this ratio?

A

Kappa:Lambda = 2:1

Deviation => Clonal population (possible malignancy, cannot diagnosis without consistent clinical features)

  • Note: Dr. Gao’s lecutre (264) says ~1:1, and Dr. Wolniak’s lecture (266) says ~2:1*
  • Wikipedia says normal is 0.26 - 1.65 so pick your poison here I guess!*
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4
Q

What hematologic malignancy arises from plasma cells?

A

Multiple myeloma

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5
Q
A
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6
Q

What cytokine drives myeloma cell growth, survival, and Ig production?

A

IL-6

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7
Q

What is the most frequent cause of death in multiple myeloma?

A

Infection

Malignant plasma cells

  • > no healthy/functional plasma cells
  • > not enough working antibody
  • > cannot protect against infection
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8
Q

What testing method do we use to find monoclonal gammopathies?

A

SPEP or UPEP

  • If <10% clonal plasma cells = monoclonal gammopathy of undetermined significance (MGUS)*
  • If >10% clonal plasma cells = may be multiple myeloma (need to have consistent clinical features though)*
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9
Q

What are the characteristics of plasma cells?

(2 morphologic features, 2 markers)

A
  • Fried egg appearance
  • Clock-face chromatin
  • CD38+
  • CD138+

Markers are cytoplasmic => detected on immunostain, not flow

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10
Q

What do plasma cells produce in abundance that can have pathologic effects on organs (especially the kidney)?

A

Immunoglobulins (antibodies) -> deposition -> tissue damage

Ig Light chains can also form amyloid -> tissue damage

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11
Q

What finding on peripheral blood smear may be concerning for multiple myeloma?

A

Roleaux formation (stack of coins)

  • Immunoglobulins in serum alter normal (-) charge of RBCs -> they stick together
  • Not specific, but warrants further screening*
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12
Q

What is the diagnosis if a patient has 10-60% monotypic plasma cells but no clinical signs of myeloma?

A

Smoldering myeloma (<20%)

75% chance of turning into myeloma

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13
Q

What will plasma cells look like on flow cytometry?

A

CD 19+

CD56-

If CD19- and CD56+, likely a neoplastic plasma cell

Immunostain will show CD138+, CD38+ (but cytoplasmic)

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14
Q

What are the diagnostic criteria for multiple myeloma?

A

≥ 10% clonal plasma cells in the bone marrow, in the setting of myeloma-defining clinical criteria

  • Myeloma-defining criteria
    • Hypercalcemia
    • Renal insufficiency
    • Anemia
    • Bone lesions

Monoclonal gammopathy is not sufficient to diagnose - need clinical criteria

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15
Q

What do Bence Jones protiens in the urine indicate?

A

Multiple myeloma

If consistent clinical picture (ex: lytic bone lesions, hypercalcemia)

Ig Light Chains enter the urine -> Bence Jones proteins

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16
Q

How does the presentation of Waldenstrom’s macroglobulinemia differ from that of myeloma?

A

Waldenstrom will have:

  • Hyperviscosity syndrome 2/2 IgM penatmers
    • Dizziness, blurred vision, Raynaud’s
  • Bleeding 2/2 platelet dysfunction
  • No lytic bone legions

Waldenstrom is an IgM monoclonal gammopathy

17
Q

List the clinical findings of myeloma (6)

A
  • Anemia
  • Hypercalcymia
  • Lytic bone lesions
  • Renal insufficiency
  • Amyloidosis
  • Hypogammaglobulinemia / infections
18
Q

How does multiple myeloma lead to bone destruction?

A

Myeloma cells produce factors that drive bone destruction

  • Expression of MIP1-alpha
  • -> Activation of RANKL
  • -> RANKL activates osteoclasts
  • Osteoclasts absorb bone