Porro F, Cattaneo M
Divisione di Medicina d'Urgenza, IRCCS Ospedale Maggiore di Milano.
Ann Ital Med Int. 2000 Oct-Dec;15(4):291-5.
Meningitis and meningococcal sepsis are emergency conditions associated with high mortality. The outcome is worsened by the onset of disseminated intravascular coagulation. This may present, particularly in children, with the clinical picture of purpura fulminans, characterized by extensive necrotic-hemorrhagic skin lesions, ischemia of the extremities and multiorgan failure. It has been observed that depletion of coagulation inhibitors, particularly protein C, plays a key role in the development of this severe complication. We describe the case of a woman who presented in the Emergency Room with signs of meningitis, drowsiness, hypotension and petechie. Bacterioscopic examination of the cerebrospinal fluid evidenced characteristic gram-negative diplococci. Laboratory data disclosed initial disseminated intravascular coagulation with low levels of proteins C and S. Following intravenous infusion of antibiotics, fluids and fresh frozen plasma, the patient's condition rapidly improved. However, multiple skin lesions appeared on her fingers, toes and heels. It is likely that the infusion of coagulation inhibitors contained in fresh frozen plasma, prevented evolution to full-blown purpura fulminans. The first choice treatment for purpura fulminans in meningococcal sepsis is infusion of protein C concentrate, which is not, however, currently available on the market.
脑膜炎和脑膜炎球菌败血症属于急症,死亡率很高。弥散性血管内凝血的发生会使病情恶化。这种情况,尤其是在儿童中,可能表现为暴发性紫癜的临床症状,其特征为广泛的坏死性出血性皮肤病变、肢体缺血和多器官功能衰竭。据观察,凝血抑制剂尤其是蛋白C的消耗在这种严重并发症的发展中起关键作用。我们描述了一名女性病例,她因脑膜炎、嗜睡、低血压和瘀点症状被送往急诊室。脑脊液的细菌学检查发现了特征性革兰氏阴性双球菌。实验室数据显示最初存在弥散性血管内凝血,蛋白C和S水平较低。静脉输注抗生素、液体和新鲜冰冻血浆后,患者病情迅速改善。然而,她的手指、脚趾和脚跟出现了多处皮肤病变。很可能是新鲜冰冻血浆中所含凝血抑制剂的输注阻止了病情发展为全面的暴发性紫癜。脑膜炎球菌败血症中暴发性紫癜的首选治疗方法是输注蛋白C浓缩物,但目前市场上尚无此类产品。