Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement.

Eun Shil Hong; Jung Hun Ohn; Jung Hee Kim; Yul Hwang-Bo; Jin Joo Kim; Jung Hee Kwon; Jung Won Lee; Se Youn Choi; Eun Kyung Lee; Sun Wook Cho; Chan Soo Shin; Kyong Soo Park; Hak Chul Jang; Bo Youn Cho; Hong Kyu Lee; Choong Ho Shin; Sei Won Yang; Seong Yeon Kim

doi:10.3803/EnM.2011.26.1.38

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Original Article Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement.: Eun Shil Hong, Jung Hun Ohn, Jung Hee Kim, Yul Hwang-Bo, Jin Joo Kim, Jung Hee Kwon, Jung Won Lee, Se Youn Choi, Eun Kyung Lee, Sun Wook Cho, Chan Soo Shin, Kyong Soo Park, Hak Chul Jang, Bo Youn Cho, Hong Kyu Lee, Choong Ho Shin, Sei Won Yang, Seong Yeon Kim; Endocrinology and Metabolism 2011;26(1):38-43
DOI: https://doi.org/10.3803/EnM.2011.26.1.38
Published online: March 1, 2011

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¹Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea. seongyk@plaza.snu.ac.kr
²Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

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Abstract

BACKGROUND
Langerhans cell histiocytosis (LCH) is a rare disease that involves a clonal proliferation of Langerhans cells. LCH has a predilection for hypothalamo-pituitary axis (HPA) dysfunction, and this leads to diabetes insipidus (DI) and/or anterior pituitary dysfunction. Here, we describe the endocrine dysfunction and clinical characteristics of adult patients with LCH and we analyzed the differences between an adult-onset type and a childhood-onset type. METHODS: The data was obtained from a retrospective chart review of the patients with LCH that involved the HPA and who attended Seoul National University Hospital. The patients were classified into the adult-onset type (age at the time of diagnosis > or = 16) and the childhood-onset type (age at the time of diagnosis < or = 15). RESULTS: Ten patients (9 males and 1 female) were diagnosed with LCH involving the HPA. Five patients were classified as an adultonset type and the other five patients were classified as a childhood-onset type. The median follow-up duration was 6 (3-12) years for the adult-onset type and 16 (15-22) years for the childhood-onset type. All the patients presented with DI as the initial manifestation of HPA involvement. Four adult-onset patients and three childhood-onset patients had a multi-system disease. Panhypopituitarism developed in three adult-onset patients and in one childhood-onset patient. The pituitary lesion of the three adult-onset patients had spread to the brain during the follow-up duration. In contrast, the pituitary lesion of the other two adult-onset patients without panhypopituitarism and all the childhood-onset patients had not changed. CONCLUSION: DI was the initial presentation symptom of HPA involvement. Anterior pituitary hormone deficiency followed in some patients. Compared with the childhood-onset patients, the adult-onset patients were more likely to have panhypopituitarism and a poor prognosis.

Keywords: Anterior pituitary hormone deficiency;Diabetes insipidus;Hypopituitarism;Hypothalamo-pituitary axis;Langerhans cell histiocytosis

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