Langerhans Cell Histiocytosis without Eosinophilia and Lytic Lesions of Bone: A Rare Disease with Unusual Presentation

Abstract

Langerhans Cell Histiocytosis (LCH) is a disease of abnormal clonal proliferation of langerhans cell of the bone marrow derived monocyte-macrophage lineage. Cells with characteristic coffee bean/grooved/indented nuclei with a background of histiocytes, lymphocytes, eosinophils and other inflammatory cells are seen. Clinical presentation varies from unifocal unisystem to multifocal unisystem to multifocal multisystem. We present a rare case of multisystem LCH involving high risk organs in a two and a half year old child. presented with the chief complaints of fever and red raised maculopapular lesions on trunk, palm, forehead and scalp for one and a half yrs with hepatosplenomegaly. There were no lytic lesions of the bones. Haematological parameters showed anaemia with thrombocytopenia while Bone Marrow Aspirate showed increase in histiocytic cell with some cells showing characteristic coffee bean, cleaved irregularly contoured nuclei. However, eosinophilia was not seen. Sections from the skin lesions showed cells with similar morphology without usually accompanied eosinophilia. The cells were CD1a and S100 positive. So a FINAL DIAGNOSIS of langerhans cell histiocytosis was made. The emphasis here lies on thorough analysis of the clinical presentations, imaging studies and scrupulous histomorphological and immunohistochemical examination, so that early diagnosis and timely intervention is ensured in such cases where the characteristic features like lytic lesions of bone and eosinophilia is not found.

Keywords

• benign
• histopathology
• haematological
• multisystem
• pathogenesis
• prognosisbenign
• prognosis