Disseminated histoplasmosis mimicking autoimmune hepatitis as a trigger of secondary hemophagocytic lymphohistiocytosis in a pediatric patient

Abstract Patients presenting with hemophagocytic lymphohistiocytosis (HLH) are often critically ill, and rapid identification of both HLH and underlying triggers in secondary HLH is essential for successful treatment. We present a case of secondary HLH activated by disseminated histoplasmosis (DH) that mimicked autoimmune hepatitis (AIH) on liver histology and briefly led to an incorrect liver diagnosis. Once DH was identified, he was treated with antifungals in addition to HLH therapies and stabilized. He was subsequently found to have a heterozygous variant of uncertain significance in STAT1 . Our case is the first in the literature to describe liver biopsies in a pediatric patient who has DH that had triggered HLH, and due to striking histology that mimicked AIH, important to report.