Mauro E, Stefani PM, Gherlinzoni F. Adult Langerhans cell histiocytosis and immunomodulatory drugs: Review and analysis of thirty-four case reports. World J Hematol 2019; 8(1): 1-9 [DOI: 10.5315/wjh.v8.i1.1]
This article is an open-access article which 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/
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/
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
Abstract
Langerhans cell histiocytosis (LCH) is a rare neoplastic disease in dendritic cells. LCH is classified as either a single-system (SS) or multisystem (MS) disease. There is not a standard first-line treatment for LCH in adults. We analyzed the efficacy and safety of immunomodulatory drugs (IMiDs) by searching PubMed/MEDLINE for case reports previously published. The clinical response (nonactive disease or active disease that regressed) was 94% in SS and 53% in MS. IMiDs should only be considered for adults with cutaneous SS involvement; in MS, they should be used only for patients not eligible for more aggressive treatments.
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.
