Table 5.

First line systemic therapy

Recommendation	Grade
Mild Symptoms, No Risk Organ Involved:
• Methotrexate 20 mg per week p.o/i.v.	C1
• Azathioprine 2 mg/kg/d p.o	D1
• Thalidomide 100mg/d p.o in skin or soft tissue multifocal single system LCH	C2
Additionally In Multifocal Bone LCH
• zoledronic acid 4 mg i.v.	C2
q 1 (- 6) month (depending on extent and response)	C1
Symptomatic, MS-LCH, No Risk Organs involved
• Cytarabine 100 mg/m2 d1-5 q4w i.v.	C1
• Etoposide 100 mg/m2 d1-5 q4w i.v.	D1
• Vinblastin/Prednisolone (like in pediatric studies)	C1
MS-LCH, Risc Organs Involved
• 2-CDA 6 mg/m2 d1-5 q4w s.c./i.v.	C2