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Clinical Features and Treatment Outcomes of Langerhans Cell Histiocytosis: A Nationwide Survey From Korea Histiocytosis Working Party

Kim, Bo Eun MD; Koh, Kyung-Nam MD, PhD; Suh, Jin Kyung MD; Im, Ho Joon MD, PhD; Song, Joon Sup MD, PhD; Lee, Ji Won MD; Kang, Hyoung Jin MD, PhD; Park, Kyung Duck MD, PhD; Shin, Hee Young MD, PhD; Choi, Hyoung Soo MD, PhD; Lee, Soo Hyun MD, PhD; Yoo, Keon Hee MD, PhD; Sung, Ki Woong MD, PhD; Koo, Hong Hoe MD, PhD; Jung, Hye Lim MD, PhD; Chung, Nak-Gyun MD, PhD; Cho, Bin MD, PhD; Kim, Hack Ki MD, PhD; Lyu, Chuhl Joo MD, PhD; Baek, Hee Jo MD, PhD; Kook, Hoon MD, PhD; Park, Jun Eun MD, PhD; Park, Hyeon Jin MD, PhD; Park, Byung-Kiu MD, PhD; Yoo, Eun Sun MD, PhD; Ryu, Kyung Ha MD, PhD; Lee, Kun Soo MD, PhD; Kim, Heung Sik MD, PhD; Lee, Jae Min MD, PhD; Park, Eun Sil MD, PhD; Yoon, Hoi Soo MD, PhD; Lee, Kwang Chul MD, PhD; Lee, Mee Jeong MD, PhD; Lim, Young Tak MD, PhD; Kim, Hwang Min MD, PhD; Park, Sang Kyu MD, PhD; Park, Jeong-A MD, PhD; Kim, Soon Ki MD, PhD; Park, Meerim MD; Lim, Yeon-Jung MD, PhD; Lee, Young Ho MD, PhD; Seo, Jong Jin MD, PhD; on the behalf of Korea Histiocytosis Working Party

Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0000000000000054
Original Articles
Abstract

A nationwide survey was conducted to clarify the clinical features and outcomes of Korean children with Langerhans cell histiocytosis (LCH). Korea Histiocytosis Working Party analyzed the data of 603 patients who were diagnosed with LCH between 1986 and 2010 from 28 institutions in Korea. Median age at diagnosis was 65 months (range, 0 to 276 mo). Bone was the most frequently affected organ (79.6%) followed by skin (19.2%). Initially, 419 patients (69.5%) had single-system involvement (SS), 85 (14.1%) with multisystem (MS) disease without risk organ involvement (MS-RO), and 99 (16.4%) multisystem disease with risk organ involvement (MS-RO+). The 5-year overall survival (OS) rates in the SS, MS-RO, and MS-RO+ groups were 99.8%, 98.4%, and 77.0%, respectively (P<0.001), and the 5-year reactivation rates were 17.9%, 33.5%, and 34.3%, respectively (P<0.001). The OS rate was lower in patients with RO involvement (P=0.025) and lack of response to initial treatment (P=0.001). MS involvement (P=0.036) was an independent risk factor for reactivation. Permanent consequences were documented in 99 patients (16.4%). Reactivation of disease, MS involvement, and age at diagnosis ≤2 years were associated with higher incidence of permanent consequences. This study emphasized that further efforts are required to improve survival of MS-RO+ patients and reduce reactivation in younger patients with MS involvement.

Author Information

*Department of Pediatrics, Division of Pediatric Hematology/Oncology, Asan Medical Center, University of Ulsan College of Medicine

Department of Pediatrics, Hallym Universtiy College of Medicine

Department of Pediatrics, Seoul National University College of Medicine

§Department of Pediatrics, Sungkyunkwan University School of Medicine

Department of Pediatrics, The Catholic University of Korea College of Medicine

Department of Pediatrics, Yonsei University College of Medicine

#Department of Pediatrics, Chonnam National University College of Medicine, Gwangju

**Department of Pediatrics, Ajou University College of Medicine, Suwon

††Department of Pediatrics, National Cancer Center, Goyang

‡‡Department of Pediatrics, Ewha Womans University School of Medicine, Seoul

§§Department of Pediatrics, Kyungpook National University College of Medicine

∥∥Department of Pediatrics, Keimyung University College of Medicine

¶¶Department of Pediatrics, Yeungnam University College of Medicine, Daegu

##Department of Pediatrics, Gyeongsang National University, Jinju

***Department of Pediatrics, Kyung Hee University College of Medicine

†††Department of Pediatrics, Korea University College of Medicine

‡‡‡Department of Pediatrics, Dankook University College of Medicine, Cheonan

§§§Department of Pediatrics, Pusan National University College of Medicine, Busan

∥∥∥Department of Pediatrics, Yonsei University Wonju College of Medicine, Wonju

¶¶¶Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan

###Department of Pediatrics, Inje University College of Medicine, Busan

****Department of Pediatrics, Inha University College of Medicine, Incheon

††††Department of Pediatrics, Chungbuk National University College of Medicine, Chungju

‡‡‡‡Department of Pediatrics, Chungnam National University College of Medicine, Daejeon

§§§§Department of Pediatrics, Hanyang University College of Medicine, Seoul

This study was supported by grants (2012E6301000 and 2013E6301400) from Korea Centers for Disease Control and Prevention.

B.E.K. and K.N.K. contributed equally.

The authors declare no conflict of interest

Reprints: Jong Jin Seo, MD, PhD, Department of Pediatrics, Division of Pediatric Hematology/Oncology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnap 2-dong, Songpa-gu, Seoul 138-736, Korea (e-mail: jjseo@amc.seoul.kr).

Received December 23, 2012

Accepted September 14, 2013

Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.