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Case Reports
Haemophagocytic lymphohistiocytosis associated with liver injury in systemic sarcoidosis.
- Saumya Ranjan Tripathy, Prajna Anirvan, Manoj Kumar Parida, Dinesh Meher, Pankaj Bharali, Mrinal Gogoi, Kaumudee Pattnaik, Shivaram Prasad Singh, and Bidyut Kumar Das.
- Department of Clinical Immunology and Rheumatology, S.C.B. Medical College, Cuttack 753007, Odisha, India.
- Natl Med J India. 2023 Sep 1; 36 (5): 312314312-314.
AbstractHepatic involvement in sarcoidosis, though common, is usually asymptomatic. Hepatomegaly and deranged liver function tests are the usual manifestations. However, unexplained hepatomegaly in sarcoidosis not responding to immunosuppressive therapy could indicate an alternative pathology. Haemophagocytic lymphohistiocytosis (HLH), although seldom reported in sarcoidosis, can cause hepatosplenomegaly and cytopenias. HLH occurring concomitantly with hepatic sarcoidosis is extremely rare. We report a patient of systemic sarcoidosis who presented with fever, hepatosplenomegaly and jaundice despite being on steroid therapy. He was subsequently diagnosed with HLH. The clinical response to treatment with pulse steroid and oral cyclosporine was dramatic.
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