Langerhans Cell Histiocytosis With Solitary Hepatic Involvement in an Adult Patient: Diagnosis, Transplantation, and Recurrence

Langerhans cell histiocytosis (LCH) rarely involves the liver without systemic manifestations, especially in adult population. We describe an unusual case of LCH with solitary hepatic involvement. Histopathologic materials from both native and allograft specimens, including all biopsies, explant hepatectomies, and correlating clinical data accumulated over a 10-year interval, were reviewed. A 46-year-old woman presented with jaundice and pruritus. An initial workup including liver biopsy led to the diagnosis of antimitochondrial antibody-negative primary biliary cholangitis with advanced fibrosis. Within a year, the patient progressed to decompensated cirrhosis requiring transplantation. Her posttransplant course was complicated by frequent bouts of markedly elevated alkaline phosphatase and.-glutamyl transferase with mild liver transaminases elevation. These abnormalities and the liver biopsy findings of bile duct injury and portal inflammation were thought to be acute cellular rejection. Her liver enzymes showed some improvement but never completely normalized on high-dose steroid and became elevated once the therapy stopped. Three years after transplantation, the diagnosis of LCH was established based on the histopathology and immunohistochemistry in the allograft biopsy. Four more years later, the patient lost the liver allograft and required a second transplantation. The explanted allograft revealed patchy distribution of the disease, confirming difficulty of LCH diagnosis on a liver biopsy without systemic involvement. For clinicians and pathologists, it is critical to exclude hepatic involvement by LCH before rending a diagnosis of antimitochondrial antibody-negative primary biliary cholangitis or small duct primary sclerosing cholangitis.