Langerhans Cell Histiocytosis in Taiwan: A Retrospective Case Series in a Medical Center

Abstract

Background: Langerhans cell histiocytosis (LCH) comprises a diverse group of disorders where pathologic Langerhans cells accumulate in a variety of organs. Objectives: To characterize the disease presentation in Taiwanese, and to analyze the differences of LCH between Taiwanese and western populations. Methods: A retrospective chart review was performed on patients diagnosed with LCH in a medical center in Taiwan between 1990 and 2006. Results: Thirty-five patients with biopsy-proven LCH were included. The median age of diagnosis was 3 years (range, 2 months to 58 years), and five patients aged 18 or older at the time of diagnosis. The male: female ratio was 3: 2. Multisystemic involvement was found in 15 patients (42.8%) and isolated bone lesions were found in 12 patients (34.2%), 3 patients had isolated cutaneous involvement; the remaining 5 patients had solitary lymph node, lung, thyroid gland, submandibular gland or extraocular soft tissue involvement, respectively. Ten of the 15 patients with multisystemic diseases had skinlmucosal involvement; 10 had bony involvement. All three patients that expired had multisystemic disease. Conclusion: Compared to western literature, we found significantly less isolated lung involvement (2.8%) in our cohort. Further multi-centered, prospective researches may be needed to better characterize the disease presentation in Asian population.