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World J Hematol. Dec 20, 2019; 8(1): 1-9
Published online Dec 20, 2019. doi: 10.5315/wjh.v8.i1.1
Adult Langerhans cell histiocytosis and immunomodulatory drugs: Review and analysis of thirty-four case reports
Endri Mauro, Piero Maria Stefani, Filippo Gherlinzoni
Endri Mauro, Piero Maria Stefani, Filippo Gherlinzoni, Hematology Unit, Ca’Foncello Hospital, Treviso 31100, Italy
Author contributions: Mauro E designed the paper, performed the research and wrote the manuscript; Stefani PM and Gherlinzoni F analyzed the data; all authors contributed to this manuscript.
Conflict-of-interest statement: Authors declare no conflicts of interest for this article.
Open-Access: This is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Endri Mauro, PhD, Doctor, Hematology Unit, Ca’Foncello Hospital, Piazzale Ospedale 1, Treviso 31100, Italy. endri76@libero.it
Telephone: +39-42-2322609 Fax: +39-42-2322542
Received: March 18, 2019
Peer-review started: March 19, 2019
First decision: September 17, 2019
Revised: October 26, 2019
Accepted: December 6, 2019
Article in press: December 6, 2019
Published online: December 20, 2019
Core Tip

Core tip: Langerhans cell histiocytosis (LCH) is a rare neoplastic disease derived from dendritic cells that is seen in children as well as in adults. There is not a standard first-line treatment in adults; no prospective trials have been undertaken on this population, and chemotherapy schedules are often reported from pediatric experiences with suboptimal efficacy and a higher toxicity in adults than in children. Immunomodulatory drugs (IMiDs), as less toxic therapeutic options, have been considered for treating LCH. We analyzed the efficacy and safety of IMiDs in adults with LCH from previously published research.