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噬血细胞性淋巴组织细胞增生症表现为在感染爱泼斯坦-巴尔病毒和既往甲型肝炎后出现肝衰竭。

Haemophagocytic lymphohistiocytosis presenting as liver failure following Epstein-Barr and prior hepatitis A infections.

作者信息

Pinto-Patarroyo Gineth Paola, Rytting Michael E, Vierling John Moore, Suarez-Almazor Maria E

机构信息

Department of General Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

出版信息

BMJ Case Rep. 2013 Aug 13;2013:bcr2013008979. doi: 10.1136/bcr-2013-008979.

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is associated with high mortality even after prompt diagnosis. We present a young man with HLH triggered by two common viral diseases, infectious mononucleosis and hepatitis A. This patient presented with fever, rapidly progressive liver failure, anasarca and cholestasis, followed by anaemia and neutropenia. His carbohydrate antigen 19-9 reached over 9000 U/mL. Initial bone marrow and liver biopsies did not show histological features of malignancy or HLH. The patient was finally diagnosed and treated almost 1 year after the initial symptoms started, and had an excellent response with etoposide and dexamethasone. This case is unusual because it was triggered following mononucleosis in a patient with positive total antibodies against hepatitis A, with rapidly developing liver failure, and also because the patient's response was excellent despite the delay in treatment. It underscores the importance of suspecting HLH when severe systemic illness develops after a viral infection, even in the absence of clear histological features.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)即使在迅速诊断后仍与高死亡率相关。我们报告一名年轻男性,其HLH由两种常见病毒性疾病,即传染性单核细胞增多症和甲型肝炎引发。该患者表现为发热、迅速进展的肝衰竭、全身性水肿和胆汁淤积,随后出现贫血和中性粒细胞减少。其糖类抗原19-9超过9000 U/mL。最初的骨髓和肝脏活检未显示恶性肿瘤或HLH的组织学特征。该患者在初始症状出现近1年后才最终确诊并接受治疗,使用依托泊苷和地塞米松治疗后反应良好。该病例不同寻常,因为它是在一名甲型肝炎总抗体阳性的患者发生单核细胞增多症后引发的,伴有迅速发展的肝衰竭,还因为尽管治疗延迟,但患者的反应良好。它强调了在病毒感染后出现严重全身性疾病时,即使没有明确的组织学特征,怀疑HLH的重要性。

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