Hepatic Sarcoidosis as a Rare Cause of Secondary Hemophagocytic Lymphohistiocytosis
Hamzah Shariff, Smriti Nair, Akash Patel, Alberto Martinez Mencia, Wei Jiang, Julianne Qualtieri, Adam Binder, Fabian Mendoza, David A. SassABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome often triggered by an underlying disease process. Macrophage activation syndrome (MAS) is a secondary HLH phenotype associated with autoimmune disease. A 72-year-old man presented with fever, pancytopenia, hyperferritinemia, splenomegaly, and acute liver injury. Bone marrow biopsy demonstrated hemophagocytosis, supporting HLH. Despite corticosteroid therapy, progressive liver injury prompted liver biopsy, which revealed sinusoidal hemophagocytosis and noncaseating granulomatous inflammation consistent with hepatic sarcoidosis. Infectious and malignant evaluations were unrevealing. This case highlights hepatic sarcoidosis as a rare underlying driver of secondary macrophage activation syndrome/HLH and the importance of tissue diagnosis in patients with severe hepatic involvement.