Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Ann Med. 2023;55(2):2274510. doi: 10.1080/07853890.2023.2274510. Epub 2024 Jan 1.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease characterized by chronic complement-mediated hemolysis. The concentrated outbreak of coronavirus disease 2019 (COVID-19) in China after 6 December 2022, provided an opportunity to observe the disease course of PNH during an active Omicron infection epidemic.
Patients diagnosed with PNH at Peking Union Medical College Hospital (PUMCH) before 6 December 2022, were followed up until 10 April 2023. Clinical data related to coronavirus infection and hemolysis were recorded. Factors influencing the infection and severity rate of Omicron, as well as hemolysis provocation, were analyzed.
In total, 131 patients with PNH were included in this retrospective analysis; 87.8% were infected with Omicron. Among them, 15.7% met the criteria for severity, and 1 patient died (0.87%). No protective factors were identified against Omicron infections. However, patients with severe Omicron infection ( = 18) had a lower vaccination rate than those with non-severe infection ( = 97; = 0.015). Among those infected ( = 115) with Omicron, there was a significant increase in lactate dehydrogenase (LDH) levels compared with those in the uninfected group ( = 16, = 0.000). Patients with severe infections ( = 18) had even higher LDH increase rates than those without severe infections ( = 97; = 0.002). 10 (37.0%) patients treated with complement inhibitors developed breakthrough hemolysis (BTH). Patients treated with complement inhibitors ( = 27) exhibited less severe hemolysis than treatment-naïve patients ( = 104; = 0.003).
Omicron infection exacerbates hemolytic attacks in patients with PNH. Vaccination helps mitigate the severity of Omicron infection, and using complement inhibitors reduces hemolysis exacerbation.
阵发性睡眠性血红蛋白尿症（PNH）是一种罕见的获得性疾病，其特征为慢性补体介导的溶血。2022 年 12 月 6 日后，中国新冠疫情的集中爆发为观察 PNH 在奥密克戎流行期间的疾病过程提供了机会。
我们对 2022 年 12 月 6 日前在北京协和医院（PUMCH）确诊的 PNH 患者进行了随访，随访时间截至 2023 年 4 月 10 日。记录与新冠病毒感染和溶血相关的临床数据。分析影响奥密克戎感染率和严重程度的因素以及溶血诱因。
本回顾性分析共纳入 131 例 PNH 患者，87.8%感染了奥密克戎。其中，15.7%符合重症标准，1 例死亡（0.87%）。未发现针对奥密克戎感染的保护因素。然而，重症感染患者（n=18）的疫苗接种率低于非重症感染患者（n=97；=0.015）。在感染的 115 例患者（n=115）中，与未感染者相比，乳酸脱氢酶（LDH）水平显著升高（n=16，=0.000）。重症感染患者（n=18）的 LDH 升高率甚至高于非重症感染患者（n=97；=0.002）。10（37.0%）例接受补体抑制剂治疗的患者发生突破性溶血（BTH）。与未接受补体抑制剂治疗的患者相比，接受补体抑制剂治疗的患者（n=27）溶血程度较轻（n=104；=0.003）。
奥密克戎感染会加重 PNH 患者的溶血发作。接种疫苗有助于减轻奥密克戎感染的严重程度，使用补体抑制剂可减少溶血加重。
