Plasma Cell Disorders Flashcards

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1
Q

What is the most common primary malignancy of bone?

A

Multiple myeloma

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2
Q

What interleukin may be high in multiple myeloma?

A

IL-6 (important grwoth factor for plasma cells)

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3
Q

What is multiple myeloma?

A

Malignant proliferation of plasma cells in bone marrow

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4
Q

What can plasma cells produce in relation to bone?

A

Osteoclast activating factor

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5
Q

How does multiple myeloma present?

A
  • Bone pain w. hypercalcemia

- Increased risk of fracture

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6
Q

What will be seen on X-ray in multiple myeloma?

A
  • Lytic ‘punched-out’ lesions

- Increased risk of fracture

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7
Q

What does multiple myeloma activate?

A
  • Neoplastic plasma cells activate RANK receptor on osteoclasts
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8
Q

What would be seen on serum protein electrophoresis in those with multiple myeloma?

A
  • ‘M spike’
  • Sharp spike in gamma region due to increase in immunoglobulins
  • Monoclonal immunoglobulin (usually IgG, may be IgA)
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9
Q

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

A

Infection (monoclonal antibody lacks antigenic diversity)

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10
Q

What is seen on blood smear in multiple myeloma?

A
  • Rouleaux formation (RBCs stacking on top of each other)

- Due to increased protein decreasing charge between RBCs

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11
Q

What is primary AL amyloidosis?

A
  • Result of multiple myeloma
  • Free light chain circulates in serum and deposits in tissues
  • Free light chain is excreted in urine as Bence-Jones proteins
  • Deposition in kidney tubules leads to risk for renal failure (myeloma kidney)
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12
Q

What is MGUS - Monoclonal Gammopathy of Undetermined Significance?

A

Increased serum protein with M spike w/o other symptoms associated with multiple myeloma

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13
Q

What percentage of 70 year-olds have MGUS?

A

5%

- 1% of patients each year will go on to develop multiple myeloma

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14
Q

What is Waldenstrom Macroglobulinemia?

A

B-cell lymphoma w. monoclonal IgM production

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15
Q

What are the clinical features of Waldenstrom Macroglobulinemia?

A
  • Generalised LAD; lytic bone lesions absent
  • Increased serum protein w. M spike (compromised of IgM)
  • Visual and neurological defecits (e.g. retinal hemorrhage or stroke)
  • Bleeding
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16
Q

How is Waldenstrom Macroglobulinemia treated?

A
  • Acute complications treated with plasmapheresis (removes IgM from the serum)
17
Q

Where are Langerhan cells found?

A

Skin

18
Q

What is Langerhans cell histiocytosis?

A
  • Neoplastic proliferation of Langerhans cells
19
Q

What is seen on EM in Langerhans cell histiocytosis?

A
  • Characteristic Birbeck (tennis racket) granules
20
Q

What does immunohistochemistry show with Langerhans cell histiocytosis?

A

Cells are CD1a+ and S100+

21
Q

What is Letterer-Siwe disease?

A

Malignant proliferation of Langerhans cells

22
Q

What is the classic presentation of Letterer-Siwe disease?

A
  • Skin rash and cystic skeletal defects in an infant (<2 yrs)
  • Multiple organs may be involved
  • Rapidly fatal
23
Q

What is eosinophilic granuloma?

A

Benign proliferation of Langerhans cells in bone

24
Q

How does eosinophilic granuloma classically present?

A

Pathologic fracture in an adolescent; skin is not involved

25
Q

What does a biopsy show in eosinophilic granuloma?

A

Lanerhans cells with mixed inflammatory cells, including eosinophils

26
Q

What is Hand-Schuller-Christian disease and how does it present?

A
  • Malignant proliferation of Langerhans cells

- Classic presentation is scalp rash, lytic skull defects, diabetes insipidus, and exopthalmus in a child