Copyright ©The Author(s) 2019.
World J Hematol. Dec 20, 2019; 8(1): 1-9
Published online Dec 20, 2019. doi: 10.5315/wjh.v8.i1.1
Table 1 Recommendations for first-line therapy
Disease categoryTreatment
Unifocal LCH
SkinLocal therapy (e.g., topical mustard nitrogen 20% in children)
Phototherapy: PUVA, narrow band ultraviolet B
BoneIntralesional 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 asymptomaticQuit smoking
Careful observation
PLCH symptomaticSistemic steroids
Chemotherapy in case of progressive disease
In case of severe respiratory failure or major pulmonary failure consider lung transplantation