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
Table 2 List of case reports on immunomodulatory drugs in langerhans cell histiocytosis
Ref.Publication yearAgeSexSS-LCH (1) MS-LCH (2)Previous therapiesIMiDS dose1Duration (mo)Out come
Gnassia et al[27]198732F1No5060NAD
Viraben et al[43]198848M1NA3001NAD
Bensaid et al[19]199229F2No1003ADR
Misery et al[33]199367M1No2003NAD
Thomas et al[42]199324F2No503ADS
Thomas et al[42]199359F2Prednisone5018ADS
Dallafior et al[23]199565F1Cladribin200-10010NAD
Meunier et al[32]199566M1No1002NAD
Bouyssou-Gauthier et al[21]199668M2Cyclophosph-mide/steroidsNANAADS
Gerlach et al[26]199873F2Etoposide/topi-cal mustard20012ADR
Lair et al[29]199833F2RT/surgery10028ADS
Claudon et al[22]200244F2Metotrexate/ste-roids1004ADR
Kolde et al[28]200223M2No5003ADS
Kolde et al[28]200261F2Prednisone50016ADS
Mortazavi et al[35]200229M2No1002ADS
Santillan et al[38]200333F1RT/surgery10012NAD
Padula et al[36]200431F1RT/surgeryNANANAD
Sander et al[37]200438M1Cladribin2006NAD
Mauro et al[31]200573F2CVP1007ADR
Alioua et al[17]200643F2No10012ADR
Moravvej et al[34]200627M1PUVA1003ADR
Wollina et al[44]200638M2Cladribin2009NAD
Broekaert et al[20]200757F2No5024ADR
McClain et al[10]200745F1Vinorelbine/ prednisone10022NR/PD
McClain et al[10]200731F2Vinorelbine/ prednisone/RT1000,5NR/PD
McClain et al[10]200721F2Vinorelbine/ prednisone/RT1005NAD
McClain et al[10]200746F2Vinorelbine/ prednisone/RT10012NAD
Li et al[30]201027M1No1505NAD
Fernandes et al[25]201160F1No1004NAD
Shahidi-Dadras et al[39]201120M1Topical steroid2006NAD
Szturz et al[41]201235M2Cladribin/ Cyclophospha-mide methilpredniso-lone/RT/CHO-EP/BEAMLena 259ADR
El-safadi et al[24]201259F1Surgery/RT/ MTXLena 1019NAD
Subramaniyan et al[40]201571M1No20010NAD
Ruiz Beguerie et al[18]201756F1No15012NAD
Table 3 Clinical features of all 34 patients
Clinical featuresAll patients (%)
Female21 (62)
Age at diagnosis mean (range)45.3 (20-73)
Muco-cutaneous involvement32 (94)
Multiorgan involvement17 (50)
Lung involvement3 (9)
CNS/pituitary involvement8 (23.5)
Bone involvement6 (18)
Lymph nodes involvement3 (9)
Splenomegaly/hepatomegaly2 (6)
Others sites involved (bone marrow, parotid gland)2 (6)
Table 4 Previous treatments
Treatment before IMiDsn (%)
None14 (41)
Radiotherapy8 (23.5)
Steroids8 (23.5)
Vinorelbine4 (12)
Surgery4 (12)
Cladribin4 (12)
Metotrexate2 (6%)
Polichemotherapy regimens2 (6)
Cyclophosphamide (single agent)2 (6)
Topical drugs2 (6)
Etoposide1 (3)
PUVA1 (3)