Flashcards in Histiocytic disorders Deck (5)
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1
LCH
Name high risk organs
Name CNS risk lesions
High risk organs: liver, bone marrow, spleen
-mortality 10%
CNS risk lesions: front of face, skull base and pituitary
- Single agent/no treatment has 40% risk. 20% if treated with systemic therapy
2
Clinical manifestations that are suggestive of LCH
- vertebral plana
- floating teeth
- otitis externa with purulent drainage
- interstitial pattern with cysts and nodules with spontaneous pneumothorax
- diabetes insipidus
- hypoalbuminemia
3
Immunophenotype
- LCH
- ECD
- RDD
LCH: CD1a+ CD 207+ birbeck granules
ECD: CD 68+ CD163+ CD1a-
RDD: Emperipolesis, S100+ fascin+ CD68++ CD14+ CD163+ CD1a/207-
4
HLH Diagnostic criteria
5 out of 8
- fever
- splenomegaly
- cytopenias (2 lines)
- hypertriglyceridemias and/or hypofibrinogenemia
- hemophagocytosis
- ferritin > 500
- Elevated IL-2 R > 2400
-Decreased NK cell activity
OR
Gene mutation
5
