Langerhans cell histiocytosis involving the liver

Ramya Iyyadurai1, Ruth Asirvatham2, Sowmya Satyendra1, V Surekha3 1 Department of Medicine, CMC, Vellore, Tamil Nadu, India 2 Department of Pathology, University of Michigan, USA 3 Department of Geriatrics, CMC, Vellore, Tamil Nadu, India

Figure 1: Chest radiograph showing cystic changes in the left lung base.

Abstract


Langerhans cell histiocytosis is a group of disorders caused by proliferation of the histiocytes. This is a rare neoplastic disease with multisystem involvement. We present a case of an adult male with intermittent fever, recurrent jaundice suggestive of predominant liver involvement. He had undergone multiple courses of anti-tuberculosis treatment with no improvement. Biopsy of the lymph node in our hospital showed Langerhan's cell histiocytosis and liver biopsy showed bridging fibrosis. This case report highlights liver involvement in Langerhans cell histiocytosis with lung and lymph node involvement occurring later, which is an uncommon presentation of this rare disease.

Keywords: Langerhans cell histiocytosis, Langerhans cell histiocytosis prognosis, liver Langerhans cell histiocytosis

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Introduction

Langerhans cell histiocytosis (LCH) is a disease caused by clonal proliferation and migration of dendritic antigen presenting histiocytes. Involvement of the liver in children is well recognized; however, in adults, liver involvement is poorly understood and a cause of severe morbidity and mortality.[1]