Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement.
- Author:
Eun Shil HONG
1
;
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
Author Information
- Publication Type:Original Article
- Keywords: Anterior pituitary hormone deficiency; Diabetes insipidus; Hypopituitarism; Hypothalamo-pituitary axis; Langerhans cell histiocytosis
- MeSH: Adult; Brain; Diabetes Insipidus; Follow-Up Studies; Histiocytosis, Langerhans-Cell; Humans; Hypopituitarism; Langerhans Cells; Male; Prognosis; Rare Diseases; Retrospective Studies; Axis, Cervical Vertebra
- From:Endocrinology and Metabolism 2011;26(1):38-43
- CountryRepublic of Korea
- Language:Korean
- 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.
