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

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*

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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.

Copyright © 2013 by Lippincott Williams & Wilkins

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