Haemophagocytic lymphohistiocytosis associated with liver injury in systemic sarcoidosis

Hepatic 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. Haemophagocyt...

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Published in:The National medical journal of India 2023-09, Vol.36, p.312-314, Article 312
Main Authors: TRIPATHY, SAUMYA RANJAN, ANIRVAN, PRAJNA, PARIDA, MANOJ KUMAR, MEHER, DINESH, BHARALI, PANKAJ, GOGOI, MRINAL, PATTNAIK, KAUMUDEE, SINGH, SHIVARAM PRASAD, DAS, BIDYUT KUMAR
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container_title The National medical journal of India
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creator TRIPATHY, SAUMYA RANJAN
ANIRVAN, PRAJNA
PARIDA, MANOJ KUMAR
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PATTNAIK, KAUMUDEE
SINGH, SHIVARAM PRASAD
DAS, BIDYUT KUMAR
description Hepatic 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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title Haemophagocytic lymphohistiocytosis associated with liver injury in systemic sarcoidosis
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