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Path U2 > CH6 - 6) Plasma cell disorders, Histiocytosis > Flashcards

CH6 - 6) Plasma cell disorders, Histiocytosis Flashcards

1
Q

What are the plasma cell disorders?

A

Dyscrasias: 1) multiple myeloma 2) MGUS 3) Waldenstrom Macroglobulinemia

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2
Q

What is multiple myeloma?

A

Malignant proliferation of plasma cells in the bone marrow

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3
Q

Whis the most common primary malignancy of bone?

A

metastatic cancer,

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4
Q

What is the most common malignant lesion of bone overall?

A

Multiple myeloma

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5
Q

In multiple myeloma what is usually present?

A

High serum IL-6 is sometimes present; stimulates plasma cell growth and immunoglobulin production

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6
Q

What are the clinical features for multiple myeloma?

A

1) Bone pain with hypercalcemia 2) Elevated serum protein 3) increased risk of infection 4) Rouleaux formation of RBC’s on blood smears 5) Primary AL amyloidosis 6) Proteinuria

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7
Q

Why is there bone pain with hypercalcemia in multiple myeloma?

A

Neoplastic plasma cells activate the RANK receptor on osteoclasts, leading to bone destruction.

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8
Q

With multiple myeloma what might you see on x-ray?

A

Lytic, ‘punched-out’ skeletal lesions are seen on x-ray, especially in the vertebrae and skull; increased risk for fracture

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9
Q

Why is there elevated serum protein in multiple myeloma?

A

Neoplastic plasma cells produce immunoglobulin;

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10
Q

In multiple myeloma, what is seen on electrophoresis?

A

M spike is present on serum protein electrophoresis (SPEP), most commonly due to monoclonal IgG or IgA

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11
Q

In multiple myeloma why is there increased risk of infection?

A

Monoclonal antibody lacks antigenic diversity

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12
Q

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

A

infection

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13
Q

Why is there rouleaux formation of RBCs on blood smear?

A

increased serum protein decreases charge between RBCs

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14
Q

Why is there primary AL amyloidosis in multiple myeloma?

A

Free light chains circulate in serum and deposit in tissues.

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15
Q

Why is there proteinuria in multiple myeloma? What does this increase the risk for?

A

Free light chain is excreted in the urine as Bence Jones protein; deposition in kidney tubules leads to risk for renal failure (myeloma kidney).

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16
Q

What is monoclonalgammopathy of undetermined significance?

A

Increased serum protein with M spike on SPEP; other features of multiple myeloma are absent (no lytic bone lesions, hypercalcemia, AL amyloid, or Bence Jones proteinuria)

17
Q

MGUS is common in what group of people?

A

elderly (seen in 5% of 70-year-old individuals);

18
Q

What are the odds of a patient with MGUS developing multiple myeloma?

A

1% of patients with MGUS develop multiple myeloma each year.

19
Q

What is Waldenstrom macroglobulinemia?

A

B-cell lymphoma with monoclonal IgM production

20
Q

What are the clinical features for waldenstrom macroglobulinemia?

A

1) Generalized lymphadenopathy, lytic bone lesions are absent 2) Increased serum protein with M spike (comprised of IgM) 3) Visual and neurologic deficits (e.g., retinal hemorrhage or stroke) IgM (large pentamer) causes serum hyperviscosity. 4) Bleeding

21
Q

Why is there bleeding in waldenstrom macroglobulinemia?

A

Viscous serum results in defective platelet aggregation

22
Q

What are the complications for waldenstrom macroglobulinemia treated with?

A

plasmapheresis, which removes IgM from the serum

23
Q

What are langerhans cells?

A

specialized dendritic cells found predominantly in the skin.

24
Q

What are langerhans cells derived from?

A

bone marrow monocytes

25
Q

What do langerhans cells present?

A

Present antigen to naive T cells

26
Q

What is langerhans cell histiocytosis?

A

it is a neoplastic proliferation of Langerhans cells

27
Q

What is seen on electron microscopy in langerhan cell histiocytosis?

A

Characteristic Birbeck (tennis racket) granules are seen on electron microscopy;

28
Q

What is langerhan cell histiocytosis immunochemistry?

A

cells are CDla+ and S100+

29
Q

What is Letterer-Siwe disease?

A

Malignant proliferation of Langerhans cells

30
Q

What is the classic presentation of Letterer-Siwe?

A

it is a skin rash and cystic skeletal defects in an infant (< 2 years old).

31
Q

Can Letterer-Siwe be fatal?

A

Multiple organs may be involved; rapidly fatal

32
Q

What is eosinophilic granuloma?

A

Benign proliferation of Langerhans cells in bone

33
Q

What is the classic presentation for eosinophilic granuloma?

A

it is a pathologic fracture in an adolescent; skin is not involved

34
Q

What does biopsy of eosinophilic granuloma show?

A

Langerhans cells with mixed inflammatory cells, including numerous eosinophils

35
Q

What is Hand-Schuller-Christian disease?

A

Malignant proliferation of Langerhans cells

36
Q

What is the classic presentation of Hand-Schuller-Christian disease?

A

it is scalp rash, lytic skull defects, diabetes insipidus, and exophthalmos in a child.

Path U2 (25 decks)

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