Clinical features and treatment outcomes of langerhans cell histiocytosis: A nationwide survey from Korea histiocytosis working party

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

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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.

Original languageEnglish
Pages (from-to)125-133
Number of pages9
JournalJournal of Pediatric Hematology/Oncology
Volume36
Issue number2
DOIs
Publication statusPublished - 2014 Mar 1

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Keywords

  • Langerhans cell histiocytosis
  • outcome
  • permanent consequence
  • reactivation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Kim, B. E., Koh, K. N., Suh, J. K., Im, H. J., Song, J. S., Lee, J. W., Kang, H. J., Park, K. D., Shin, H. Y., Choi, H. S., Lee, S. H., Yoo, K. H., Sung, K. W., Koo, H. H., Jung, H. L., Chung, N. G., Cho, B., Kim, H. K., Lyu, C. J., ... Seo, J. J. (2014). Clinical features and treatment outcomes of langerhans cell histiocytosis: A nationwide survey from Korea histiocytosis working party. Journal of Pediatric Hematology/Oncology, 36(2), 125-133. https://doi.org/10.1097/MPH.0000000000000054