Objective: To improve the understanding of the rare clinical presentation and management of purpura fulminans (PF) in patients with paroxysmal nocturnal haemoglobinuria (PNH). Methods: A case of PF occurring in PNH is reported, while the related literature review is conducted. Results: A 49-year-old male patient suffered from one-week history of fever, greenish-brown colour urine, multiple well demarcated and painful purpura of the head and neck. He had been reported to have two thromboembolic events during the 22-year course of PNH. Skin biopsy displayed classic PF features. Laboratory testing showed a high PNH clone, intravascular hemolysis and coagulation system changes. After sufficient anticoagulation and short course of glucocorticoid therapy, the clinical conditions were improved correspondingly. During a follow-up period of 6 month, there was no recurrence of thrombosis. Conclusion: PF should be considered in PNH patients with unexplained, quickly developed painful purpura. Extensive work-up should be performed to find out other potential thrombophilic risk factors after diagnosis of PF. Early diagnosis, adequate anticoagulation therapy and control hemolysis were essential to PF treatment occurring in PNH. The survival of patients and the qualities of life can be improved. The PNH clone detection is needed to evaluate the status of procoagulation and predict the risk of recurrent thrombosis.
目的： 提高对于阵发性睡眠性血红蛋白尿症（PNH）合并暴发性紫癜这一罕见部位血栓并发症的认识并探讨对此类急重症的诊治。 方法： 报道一例PNH患者合并暴发性紫癜的临床诊治过程，并对相关文献进行复习。 结果： 患者，男，49岁，诊断PNH 22年，曾发生下肢静脉及门静脉血栓，因门静脉血栓迁延继发门脉高压、胃底静脉曲张、血小板减少，本次主要表现为前驱感染后多处皮肤大片痛性紫黑色皮疹、尿色加深，经皮肤活检病理诊断为暴发性紫癜，检测提示PNH克隆高负荷、血管内溶血加重，经依诺肝素6 000 U每12 h 1次抗凝、头孢美唑抗感染及短程泼尼松控制溶血等治疗后，皮肤梗死得以控制好转，随访6个月病情稳定，无再次血栓事件发生。 结论： PNH患者如发生原因不明的急性痛性紫黑色皮疹，需高度警惕暴发性紫癜，同时应积极寻找其他易栓因素并评估体内凝血状态。早期诊断并给予充分的抗凝、控制溶血是PNH伴发暴发性紫癜的治疗关键，也有助于改善患者的预后。在临床诊疗中也应重视PNH克隆负荷检测，有助于评估患者易栓状态、预测血栓发生风险并调整抗凝策略。.
Keywords: Paroxysmal nocturnal hemoglobinuria; Purpura fulminans; Thrombosis.