Table 1.
Reported case series of systemic therapies for adult Langerhans cell histiocytosis (LCH)
| Reference | No. of pts | Disease type | Median age, y (range) | Treatment | Overall response | Median follow‐up (y) |
|---|---|---|---|---|---|---|
| Saven and Burian 58 | 12 | 3 SS, 4 MS | 44 (19‐72) | 2‐CdA | 75% | 3.6 |
| McClain et al 57 | 7 | 7 MS | Not given | VBL, PSL followed by VBL, PSL, 6‐MP | 43% | 0.5 |
| Cantu et al55 | 58 | 58 SS with bone lesions | 32 (18‐72) |
VBL, PSL for 19 pts (1st 15, 2nd 4) 2‐CdA for 22 pts (1st 9, 2nd 9, 3rd 4) HDAC for 24 pts (1st 12, 2nd 5, 3rd 7) |
VBL, PSL 16%a
2‐CdA 31%a AraC 79%a |
8.5 |
| Morimoto et al56 | 14 | 4 SS, 10 MS | 43 (20‐70) | VBL, PSL, MTX, 6‐MP | 72% | 2.8 |
| Derenzini et al 59 | 11 | 6 SS, 5 MS | 27 (18‐62) | CPA, ADR, MTX, VCR, BLEO, PSL (MACOP‐B) | 100% | 6.7 |
| Duan et al 60 | 45 | 4 SS, 41 MS | 31 (18‐69) |
CPA, VDS, ETP, PSL (CEVP) for 31 pts VDS, PSL (VP) for 13 pts |
CEVP 70% VP 64% |
6.2 |
| Tazi et al54 | 35 | 7 SS, 28 MS | 33 (28‐42) | VBL, PSL | 72% | 7.6 |
Patients who relapsed within 1 year were excluded.
2‐CdA, cladribine; 6‐MP, 6‐mercaptopurine; ADR, adriamycin; AraC, cytarabine; BLEO, bleomycin; CEVP, cyclophosphamide, epirubicin, etoposide, and cis‐platinum; CPA, cyclophosphamide; ETP, etoposide; HDAC, high‐dose cytarabine; MACOP‐B, methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin; MS, multisystem LCH; MTX, methotrexate; PSL, prednisolone; Pts, patients; SS, single system LCH; VBL, vinblastine; VCR, vincristine; VDS, vindesine; VP, vindesine and prednisone.