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Pathoma Ch 6 - WBC Pathologies > 6-8, 6-9: Plasma Cell Disorders and Histiocytosis > Flashcards

6-8, 6-9: Plasma Cell Disorders and Histiocytosis Flashcards

1
Q

Multiple Myeloma-

  • proliferation of what cell?
  • What cytokine is sometimes present in the serum?
A
  • malignant proliferation of plasma cells in bone marrow

- IL-6 sometimes in serum

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2
Q

Most common primary malignancy of bone?

A

Multiple Myeloma

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3
Q

*Multiple Myeloma is associated with what cytokine?

A

*IL-6

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4
Q

Multiple myeloma -

-bone presentation?

A
  • bone pain with hypercalcemia
  • neoplastic plasma cell activate RANK receptor (via OSTEOCLAST ACTIVATING FACTOR) on osteoclasts –> lytic ‘punched out’ lesions seen on x-ray, especially in the vertebrae and skull (increased risk of fracture
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5
Q

Punched out bone lesions?

A

multiple myeloma

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6
Q

Multiple myeloma -

-serum issues?

A
  • elevated serum protein
  • neoplastic plasma cells produce immunoglobulin
  • M spike s present on SPEP (serum peptide electrophoresis), usually due to IgA or IgG (gamma globulin peak on serum electrophoresis is way higher than it should be and it makes a sharp spike = M-spike)
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7
Q

Most common cause of death due to multiple myeloma and why

A

the neoplastic plasma cells pump out a bunch of IgA or IgG but there is no antigenic diversity = infection most common cause of death

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8
Q

Roleaux formation on blood smear with what disease? How does this happen?

A

Multiple myeloma – increased serum protein decreases charge between RBCs and so the RBC stack like “poker chips”
-this is a sign of high serum immunoglobulin

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9
Q

Multiple myeloma-

  • whats going on with primary AL amyloidosis
  • consequences of primary amyloidosis?
A
  • usually have equal production of heavy and light chains
  • plasma cells overproduce light chain –> so there is free light chain floating around in serum –> 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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10
Q

Bence-Jones proteins are seen with which cancer?

A

Multiple myeloma - production of a lot of light chain that deposits in tissues and is excreted in the urine

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11
Q

MGUS - monoclonal gammapathy of undetermined significance

  • whats going on here?
  • common in what population?
A
  • patient has M-spike serum protein on electrophoresis but ALL OTHER FEATURES OF MULTIPLE MYELOMA ARE ABSENT (IE: no lytic bone lesions, no hypercalcemia, no AL amyloid, no Bence-Jones proteins)
  • common in the elderly - 1% develop multiple myeloma
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12
Q

Waldenstrom Macroglobinemia

-proliferation of what cell and production of what?

A

-B-cell lymphoma with monoclonal IgM production

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13
Q

Waldenstrom Macroglobinemia -

  • Clinical features
  • Tx of acute complications?
A
  • Generalized LAD
  • NO Lytic bone lesions
  • Increased serum protein with M-spike (IgM) == inc viscosity of blood
  • visual and neurologic deficits (retinal hemorrhage or stroke)
  • Bleeding - platelets cant aggregate correctly

-Tx= plasmapheresis to remove IgM from serum

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14
Q

Langerhans cell histiocytosis

  • proliferation of what cells? what do these cells normally do?
  • characteristic appearance?
  • markers for these cells?
A
  • neoplastic proliferation of langerhans cells - these cels are usually found in the skin and are derived from bone marrow monocytes - present antigen to naive T-cells
  • characteristic Birbeck (TENNIS RACKET) appearance on electron microscopy
  • immunohistochem markers: CD1a and S100
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15
Q

Birbek appearance - what is the cancer?

A

Langerhans cell histiocytosis (TENNIS RACKET APPEARANCE)

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16
Q

how to know whether histiocytosis cancer is malignant or not?

A
  • If the cancer is named after someone then it is usually malignant - malignant usually includes skin
  • If name of malignancy made of up of 2 names = affects children less than 2 yro
  • if the name of malignancy made of 3 names then children greater than the age of 3
17
Q

Letterer-Swide Disease -

  • proliferation of what cells?
  • classic presentation
  • what happens to patient?
A
  • MALIGNANT proliferation of langerhans cells
  • classically presents as skin rash and cystic skeletal defects in an infant (<2yro)
  • multipe organs involved = rapidly fatal
18
Q

Eosinophilic Granuloma

  • proliferation of what cells?
  • classic presentation
  • biopsy shows what?
A
  • BENIGN proliferation of langerhans cells
  • classic presentation is pathologic fracture in adolescent - SKIN NOT INVOLVED
  • biopsy shows langerhans cells with mixed inflammatory cells, including eosinophils
19
Q

Hand_schuller-Christian disease

  • -proliferation of what cells?
  • classic presentation
A
  • MALIGNANT proliferation of langerhans cells in children older than 3 years
  • classic presentation = scalp rash, lytic skull defects, diabetes insipidus, and exophthalmos in a child

Pathoma Ch 6 - WBC Pathologies (6 decks)

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