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Table 5 First line systemic therapy

From: Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

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