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Table 8 Therapeutic recommendations in pLCH

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

Recommendation Grade
First step is smoking cessation in all patients C2
Watchful waiting in a- or minor symptomatic patients C2
Systemic steroid therapy in symptomatic patients C2
Chemotherapy (e.g. 2-CDA) in progressive disease C2
Consider lung transplantation in case of severe respiratory failure or major pulmonary hypertension C2