Department of Hematology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, No.1 Shuaifuyuan, Beijing 100730, China.
J Infect Chemother. 2020 May;26(5):502-505. doi: 10.1016/j.jiac.2019.12.012. Epub 2020 Jan 23.
Chronic active Epstein-Barr virus (EBV) infection (CAEBV) is a high-mortality form of EBV infection. However, chronic hypoxemia is rare in these patients. We herein reported a case of severe hypoxemia due to intrapulmonary shunting in CAEBV. A 17-year-old girl presented with fever, dyspnea, cyanosis, and hepatosplenomegaly. Laboratory tests showed mild liver dysfunction and high copy numbers of EBV-DNA in the peripheral blood. A left supratrochlear lymph node biopsy showed infiltration of highly proliferative T lymphocytes with positive EBV encoded small RNA by in situ hybridization. Technetium-99m-labeled macroaggregated albumin and contrast-enhanced echocardiography confirmed the existence of intrapulmonary shunting, which was probably related to hepatopulmonary syndrome. The final diagnosis was CAEBV with intrapulmonary shunting. The patient was treated with cyclosporine A, etoposide, and dexamethasone. Finally, the patient died of respiratory failure. Intrapulmonary shunting is a rare complication of CAEBV. Early recognition and exploring the cause of hypoxemia should be highlighted in patients with CAEBV.
慢性活动性 EBV 感染（CAEBV）是一种 EBV 感染的高死亡率形式。然而，这些患者中慢性低氧血症很少见。我们在此报告一例因 CAEBV 导致的肺内分流引起的严重低氧血症。一名 17 岁女孩出现发热、呼吸困难、发绀和肝脾肿大。实验室检查显示轻度肝功能障碍和外周血 EBV-DNA 高拷贝数。左滑车上淋巴结活检显示高度增生的 T 淋巴细胞浸润，原位杂交显示 EBV 编码的小 RNA 阳性。锝-99m 标记的巨聚合白蛋白和对比增强超声心动图证实存在肺内分流，可能与肝肺综合征有关。最终诊断为 CAEBV 合并肺内分流。患者接受环孢素 A、依托泊苷和地塞米松治疗。最终，患者死于呼吸衰竭。肺内分流是 CAEBV 的罕见并发症。在 CAEBV 患者中，应早期识别并探讨低氧血症的原因。
