Table 5 First line systemic therapy

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