396 - 397 Langerhans cell histio and chronic myeloprolif d/os Flashcards

1
Q

What lineage do dendritic cells come from?

A

monocyte lineage

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2
Q

What is another name for a dendritic cell?

A

Langerhans cell

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3
Q

What are Langerhans cell histiocytoses?

A

proliferative disorders of dendritic cells

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4
Q

How does Langerhans cell histiocytosis present?

A

A child with:

  1. lytic bone lesions and skin rash, or
  2. recurrent otitis media with a mass involving the mastoid bone
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5
Q

What is wrong with the dendritic cells in Langerhans cell histiocytosis?

A

The cells are functionally immature and do not efficiently stimulate primary T lymphocytes via antigen presentation

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6
Q

What markers are expressed by Langerhans cells?

A

S-100 (mesodermal origin) and CD1a

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7
Q

What kind of granules are seen in Langerhans cell histiocytosis?

A

Birbeck granules (“tennis rackets” on EM)

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8
Q

What is the hematocrit level in polycythemia vera?

A

> 55%

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9
Q

Describe the genetics of PV?

A

Somatic (non-hereditary) mutation in JAK2 gene

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10
Q

What is erythromelalgia?

A

A rare but classic symptom in PV: severe, burning pain and reddish or bluish coloration due to episodic blood clots in vessels of the extremeties

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11
Q

What causes secondary polycythemia?

A

natural or artificial ↑ in EPO levels

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12
Q

Describe essential thrombosis

A

Overproduction of abnormal platelets → bleeding, thrombosis.
Enlarged megakaryocytes in bone marrow.

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13
Q

A chronic myeloproliferative disorder characterized by fibrotic obliteration of bone marrow:

A

myelofibrosis

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14
Q

Blood smear findings in myelofibrosis:

A
  1. teardrop RBCs

2. immature forms of the myeloid line

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15
Q

How are the levels of the following cell lines affected in PV?

  • RBCs
  • WBCs
  • Platelets
A
  • RBCs: ↑
  • WBCs: ↑
  • Platelets: ↑
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16
Q

How are the levels of the following cell lines affected in CML?

  • RBCs
  • WBCs
  • Platelets
A
  • RBCs: ↓
  • WBCs: ↑
  • Platelets: ↑
17
Q

How are the levels of the following cell lines affected in myelofibrosis?

  • RBCs
  • WBCs
  • Platelets
A
  • RBCs: ↓
  • WBCs: variable
  • Platelets: variable
18
Q

How are the levels of the following cell lines affected in ET?

  • RBCs
  • WBCs
  • Platelets
A
  • RBCs: nml
  • WBCs: nml
  • Platelets: ↑
19
Q

What are 3 causes of appropriate absolute polycythemia?

A
  1. lung disease
  2. congenital heart disease
  3. high altitude
20
Q

What are 4 causes of inappropriate absolute polycythemia?

A
  1. RCC
  2. WT
  3. HCC
  4. hydronephrosis
    (due to ectopic EPO)