Adult Langerhans cell histiocytosis and immunomodulatory drugs: Review and analysis of thirty-four case reports

Table 1 Recommendations for first-line therapy

Disease category	Treatment
Unifocal LCH
Skin	Local therapy (e.g., topical mustard nitrogen 20% in children)
	Phototherapy: PUVA, narrow band ultraviolet B
Bone	Intralesional steroid injection (40-160 mg methylprednisolone)
	Radiotherapy (in case of neurological deficit, soft tissue involvement)
Multifocal SS-LCH without “organ risk”
SS-LCH (bone lesions)	Zoledronic acid
SS-LCH (skin)	Methotrexate 20 mg per week p.o/i.v.
	Azathioprine 2 mg/kg/d p.o.
	Thalidomide 100 mg/die p.o. (skin or soft tissue multifocal SS-LCH if symptomatic)
Symptomatic MS-LCH without “risk organs”	Cytarabine 100 mg/m2 d1-5 q4w i.v.
	Etoposide 100 mg/m2 d1-5 q4w i.v.
	Vinblastin/Prednisone (“pediatric like schedule”)
MS-LCH with “risk organs”	2-CDA 6 mg/m2 d1-5 q4w s.c./i.v.
PLCH asymptomatic	Quit smoking
	Careful observation
PLCH symptomatic	Sistemic steroids
	Chemotherapy in case of progressive disease
	In case of severe respiratory failure or major pulmonary failure consider lung transplantation

"Risk organs" contains hematopoietic system, spleen, liver, and central nervous system. LCH: Langerhans cell histiocytosis. SS-LCH: Single-system Langerhans cell histiocytosis; MS-LCH: Multisystem Langerhans cell histiocytosis; P-LCH: Pulmonary Langerhans cell histiocytosis; PUVA: Psolaren plus ultraviolet A.