Peixoto Vanda, Carneiro Ana, Trigo Fernanda, Vieira Mónica, Prudêncio Cristina
Chemical and Biomolecular Sciences, School of Health, Polytechnic Institute of Porto, 4200-072 Porto, Portugal.
Center for Translational Health and Medical Biotechnology Research (TBIO), Polytechnic Institute of Porto, 4200-465 Porto, Portugal.
Reports (MDPI). 2023 Sep 8;6(3):42. doi: 10.3390/reports6030042.
Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal, rare, complement-mediated hemolytic anemia. PNH can be associated with marrow failure and thrombophilia. We present a clinical report of splenic vein thrombosis in a patient with classic PNH. A 41-year-old male with classic PNH, naïve to complement inhibitor therapy, developed splenic vein thrombosis as a major adverse effect after vaccination protocol to prevent meningococcal disease. We also report anticoagulant and eculizumab treatment outcomes. In PNH patients, vaccination should be monitored to prevent major outcome events, like vaccine-induced thrombosis. Eculizumab proves effective for treating intravascular hemolysis and preventing more thrombotic events. The potential protective role of eculizumab on controlling complement activity and consequent inflammation may help the patient to not experience breakthrough hemolysis when infected with SARS-CoV-2. Extravascular hemolysis remains present, but new molecules are being studied to inhibit proximal complement and there is a good health prospective for PNH patients.
阵发性睡眠性血红蛋白尿(PNH)是一种克隆性、罕见的、补体介导的溶血性贫血。PNH可与骨髓衰竭和血栓形成倾向相关。我们报告一例典型PNH患者发生脾静脉血栓形成的临床病例。一名41岁的男性,患有典型PNH,未接受过补体抑制剂治疗,在接种预防脑膜炎球菌病的疫苗后,出现脾静脉血栓形成这一主要不良反应。我们还报告了抗凝和依库珠单抗的治疗结果。对于PNH患者,应监测疫苗接种情况以预防严重后果事件,如疫苗诱导的血栓形成。依库珠单抗被证明对治疗血管内溶血和预防更多血栓形成事件有效。依库珠单抗在控制补体活性及随之而来的炎症方面的潜在保护作用,可能有助于患者在感染严重急性呼吸综合征冠状病毒2(SARS-CoV-2)时不发生突破性溶血。血管外溶血仍然存在,但正在研究新的分子以抑制补体近端成分,PNH患者的健康前景良好。