Langerhans Cell Histiocytosis: Clinical, Histopathological and Radiological Case Series Study: An Institutional Experience

Abstract

Background: Langerhans cells histiocytosis (LCH) is a rare disease characterized by the uncontrolled proliferation of Langerhans cells (LCs). This study aimed to explore the clinic-epidemiological and pathological data of that disease in our population. Methods: Sixteen patients were referred to the Dermatology and the Clinical Oncology & Nuclear Medicine Departments of our university during the 6 years from 2007 to 2012. Records data were retrospectively analyzed. Pathologic specimens and radiologic films were reassessed by consultants. Results: Pediatric age and male sex predominated. The multifocal uni system (MUS) was the commonest presentation (50% of cases) while the multifocal multisystem (MMS) was found in 37.5% of cases including a case of Letterer-Siwe disease. Microscopic examination revealed dense infiltrate mainly of LCs with characteristic features and positive immunostaining for S100, CD1a and CD68. The treatment was heterogeneous. The follow-up time was well documented for 12 cases (mean=5 years). The prognosis was variable. Limitation of this study was the retrospective nature. Conclusions: This case series showed the predominance of males, pediatric age, bone involvement and good treatment response. Diversity of system involvement necessitates a multidisciplinary approach. This diversity also could be a cause of underdiagnosis.