396 - 397 Langerhans cell histio and chronic myeloprolif d/os Flashcards
What lineage do dendritic cells come from?
monocyte lineage
What is another name for a dendritic cell?
Langerhans cell
What are Langerhans cell histiocytoses?
proliferative disorders of dendritic cells
How does Langerhans cell histiocytosis present?
A child with:
- lytic bone lesions and skin rash, or
- recurrent otitis media with a mass involving the mastoid bone
What is wrong with the dendritic cells in Langerhans cell histiocytosis?
The cells are functionally immature and do not efficiently stimulate primary T lymphocytes via antigen presentation
What markers are expressed by Langerhans cells?
S-100 (mesodermal origin) and CD1a
What kind of granules are seen in Langerhans cell histiocytosis?
Birbeck granules (“tennis rackets” on EM)
What is the hematocrit level in polycythemia vera?
> 55%
Describe the genetics of PV?
Somatic (non-hereditary) mutation in JAK2 gene
What is erythromelalgia?
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
What causes secondary polycythemia?
natural or artificial ↑ in EPO levels
Describe essential thrombosis
Overproduction of abnormal platelets → bleeding, thrombosis.
Enlarged megakaryocytes in bone marrow.
A chronic myeloproliferative disorder characterized by fibrotic obliteration of bone marrow:
myelofibrosis
Blood smear findings in myelofibrosis:
- teardrop RBCs
2. immature forms of the myeloid line
How are the levels of the following cell lines affected in PV?
- RBCs
- WBCs
- Platelets
- RBCs: ↑
- WBCs: ↑
- Platelets: ↑