- Histiocytic Disorders – over-production of histiocytes; wide group of diseases
- Dendritic cell disorders (Langerhans cell histiocytosis)
- Macrophage cell disorder (Rosai-Dorfman and hemophagocyticlymphohistiocytosis (HLH))
- Sinus Histiocytosis with massive lymphadenopathy
- Presents in children with massive, nontender cervical adenopathy but can also include mediastinal and inguinal lymphadnopathy
- Also involves pulmonary nodules, rash, lytic bone lesions, and fevers
- Emperipolesis (engulfment of inflammatory cells by histiocytes) is a pathologic hallmark
- Clofarabine (purine analog) has been used as treatment
Posted 04/18/17 10:17:03 AM by Ying Liu
Hemophagocytic Lymphohistiocytosis (HLH)
- Aggressive and life-threatening syndrome of excessive immune activation due to lack of normal downregulation of activated macrophages and lymphocytes
- 3 Types:
- Associated with rheumatological conditions (Macrophage Activating Syndrome)
- Infection is a common trigger
- Commonly viral such as EBV
- Incidence of 1 child in 3000 admitted to a tertiary care pediatric hospital
Posted 04/18/17 10:17:49 AM by Ying Liu
For further reading...
Posted 04/18/17 10:19:06 AM by Ying Liu
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