6.9 - Langerhans cell histiocytosis Flashcards
1
Q
what are langherhans cells?
A
specialized dendritic cells found predominantly in the skin
2
Q
langerhans cells are derived from what cells?
A
bone marrow monocytes
3
Q
what is the classic EM finding in langerhans cell histiocytosis?
A
birbeck granule (tennis racket)
4
Q
how do langerhans cells stain on IHC?
A
- CD1a
- S100
5
Q
what is letterer-siwe disease? what is the presentation?
A
- malignant proliferation of langerhans cells
- skin rash and cystic skeletal defects in an infant
6
Q
what is eosinophilic granuloma? what is the presentation?
A
benign proliferation of langerhans cells in bone
- pathologic fracture in an adolescent (skin NOT involved)
7
Q
what is hand schuller christian disease? what is the presentation?
A
- malignant proliferation of langerhans cells
- scalp rash, lytic skull defects, diabetes insipidus, exophthalmos in a child
