Division of Gastroenterology, Department of Internal Medicine, St. Marianna University School of Medicine, Yokohama City Seibu Hospital, Yokohama, Kanagawa, Japan.
Division of Hematology and Oncology, Department of Internal Medicine, St. Marianna University School of Medicine, Yokohama City Seibu Hospital, Yokohama, Kanagawa, Japan.
Am J Case Rep. 2022 Feb 20;23:e934003. doi: 10.12659/AJCR.934003.
BACKGROUND Common variable immunodeficiency (CVID) is a rare disease. Infectious mononucleosis-like symptoms due to Epstein-Barr virus reactivation in adulthood are also rare. Here, we aimed to report a case of Epstein-Barr virus reactivation presenting with relapsing infectious mononucleosis-like symptoms with liver failure in common variable immunodeficiency with chronic hepatitis B virus infection. CASE REPORT A 36-year-old Japanese woman with chronic hepatitis B virus infection developed relapsing fever, lymphadenopathy with marked splenomegaly, and ascites 6 months after treatment with propagermanium, a nonspecific immune modulator, and subsequent treatment with entecavir and pegylated interferon sequential therapy. Although the hepatitis B virus load was controlled, Epstein-Barr virus deoxyribose nucleic acid was detected in her serum. Seven months later, her symptoms improved following corticosteroid treatment. Prior to sequential therapy, she developed pneumonia 4 times in 2 months and exhibited consistent hypoimmunoglobulinemia before corticosteroid treatment. Further examinations showed low amounts of switched memory B cells, and absence or barely detectable levels of isohemagglutinins. Subsequently, she was diagnosed with common variable immunodeficiency. CONCLUSIONS Epstein-Barr virus reactivation with relapsing infectious mononucleosis-like symptoms can occur following immune modulation therapy in patients with common variable immunodeficiency, and this can affect the patient's primary disease. Therefore, immunoglobulin screening along with the consideration of CVID in all patients is required before immune modulation therapy is planned.
普通变异性免疫缺陷症（CVID）是一种罕见疾病。成年人中由于 EBV 再激活而导致的传染性单核细胞增多症样症状也较为罕见。在此，我们旨在报告一例 CVID 合并慢性乙型肝炎病毒（HBV）感染患者，在使用非特异性免疫调节剂丙种球蛋白后，出现 EBV 再激活并伴有反复传染性单核细胞增多症样症状和肝功能衰竭。
一名 36 岁日本女性，在使用丙种球蛋白和随后的恩替卡韦及聚乙二醇干扰素序贯治疗治疗慢性 HBV 感染后 6 个月，出现反复发热、淋巴结病伴明显脾肿大和腹水。尽管乙型肝炎病毒载量得到控制，但她的血清中检测到 EBV 脱氧核糖核酸。7 个月后，她的症状在接受皮质类固醇治疗后改善。在序贯治疗之前，她在 2 个月内发生了 4 次肺炎，在皮质类固醇治疗前表现出持续的低免疫球蛋白血症。进一步检查显示，转换记忆 B 细胞数量减少，同种异型凝集素缺失或几乎检测不到。随后，她被诊断为 CVID。
在 CVID 患者中，免疫调节治疗后可发生 EBV 再激活伴反复传染性单核细胞增多症样症状，这可能影响患者的原发性疾病。因此，在计划免疫调节治疗之前，需要对所有患者进行免疫球蛋白筛查并考虑 CVID 的可能性。
