Uncommon presentation of hepatic Langerhans cell histiocytosis in a young adult: a diagnostic challenge

Langerhans cell histiocytosis (LCH) is a rare clonal myeloid neoplasm with varied and often organ-specific manifestations. Hepatic involvement in adult LCH is rare and frequently results in delayed diagnosis due to its non-specific clinical and biochemical features. We describe the case of a woman in her late twenties who presented with intermittent upper abdominal discomfort, fatigue and weight loss. Despite extensive imaging, serological workup and initial liver biopsy, no definitive diagnosis could be established. Ultimately, diagnostic laparoscopy and histopathological examination with immunohistochemistry confirmed the diagnosis of multifocal hepatic LCH. This case highlights the importance of considering LCH in the differential diagnosis of unexplained hepatomegaly with cholestatic liver enzyme elevation and systemic symptoms. Early liver biopsy with appropriate immunophenotyping is critical for timely diagnosis and initiation of treatment.