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Table 4 Summary of trials exploring regiments treated for adult LCH patients

From: The combination of methotrexate and cytosine arabinoside in newly diagnosed adult Langerhans cell histiocytosis: a prospective phase II interventional clinical trial

Year Author patient number Fisrt line regimen Overall response rate PFS or reactivation 3–4 grade hematological toxicity
1999 Alan Saven 13 Cladribine 75% N/A Neutropenia 7/13 (53.8%)
2012 Maria A. Cantu 19 Vinblastine+ prednisone 16% N/A 75%
22 2-CdA 41% N/A 37%
24 ARA-C 79% N/A 20%
2013 Z. ADAM 7 Cladribine 86% N/A Neutropenia 6/7 (86%)
2015 Enrico Derenzini 11 MACOP-B 100% Overall 64% Neutropenia 4/11 (36.4%)
2016 Duan MH 31 CEVP 68.8% Reactivation rate 73.3% Neutropenia 35.6%
Thrombocytopenia 8.9%
14 VP 70% Reactivation rate 71% Neutropenia 48.4%
Thrombocytopenia 12.9%
  1. N/A Not Applicable, MACOP-B cyclophosphamide, doxorubicin, methotrexate, vincristine, bleomycin, prednisone, CEVP cyclophosphamide, vindesine, etoposide, prednisone, VP vindesine, prednisone