Angulo Evelyn, Joyner Sydney, Majeed Nasma K, Nyenhuis Sharmilee
Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI, USA.
College of Medicine, University of Illinois at Chicago, Chicago, IL, USA.
SAGE Open Med Case Rep. 2020 Jun 17;8:2050313X20931996. doi: 10.1177/2050313X20931996. eCollection 2020.
Peliosis hepatis is a rare condition characterized by blackish-blue blood-filled cavities in hepatic parenchyma caused by dilatation of hepatic sinusoids. Peliosis hepatis has been described in secondary immunodeficiencies and certain medications. We present the first case of peliosis hepatis in a patient with a primary immunodeficiency, common variable immunodeficiency. A 44-year-old African-American male presented with gastrointestinal bleeding and elevated liver function tests. His medical history included common variable immunodeficiency and chronic kidney disease. The patient had jaundice, regenerative nodules on liver pathology, and low immunoglobulin levels. A magnetic resonance imaging of the abdomen with contrast revealed a cirrhotic liver, a 5 × 3 cm lesion, and poorly defined nodules which had decreased enhancement. A computed tomography-guided liver biopsy revealed peliosis hepatis, focal nodular hyperplasia, and fibrosis. No other etiology of his liver disease was found. The etiology of peliosis hepatis in patients with primary immunodeficiencies remains unclear. Additional studies are needed to understand the underlying mechanisms.
肝紫癜是一种罕见病症，其特征为肝实质内出现由肝血窦扩张导致的充满蓝黑色血液的腔隙。肝紫癜已在继发性免疫缺陷及某些药物使用情况中被描述。我们报告了首例原发性免疫缺陷（常见变异型免疫缺陷）患者发生肝紫癜的病例。一名44岁的非裔美国男性因胃肠道出血及肝功能检查指标升高前来就诊。他的病史包括常见变异型免疫缺陷和慢性肾病。该患者有黄疸、肝脏病理检查显示再生结节以及免疫球蛋白水平低下。腹部增强磁共振成像显示肝脏呈肝硬化表现、有一个5×3厘米的病灶以及边界不清的结节，这些结节强化减弱。计算机断层扫描引导下的肝活检显示有肝紫癜、局灶性结节性增生及纤维化。未发现其肝脏疾病的其他病因。原发性免疫缺陷患者发生肝紫癜的病因仍不清楚。需要进一步研究以了解其潜在机制。
