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, Bidyut Kumar DAS
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Abstract

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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系统性肉瘤病中伴有肝损伤的嗜血细胞淋巴组织细胞增多症。
肉样瘤病的肝脏受累虽然常见,但通常没有症状。肝脏肿大和肝功能检查异常是常见的表现。不过,对免疫抑制治疗无效的肉样瘤病患者出现原因不明的肝脏肿大可能预示着另一种病理变化。嗜血细胞性淋巴组织细胞增多症(HLH)虽然很少见于肉样瘤病,但可引起肝脾肿大和细胞减少。与肝肉样瘤病同时发生的 HLH 极其罕见。我们报告了一名全身性肉样瘤病患者,尽管他正在接受类固醇治疗,但仍出现发热、肝脾肿大和黄疸。他随后被诊断为 HLH。脉冲类固醇和口服环孢素的治疗效果显著。
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