Schellongowski Peter, Bauer Edith, Holzinger Ulrike, Staudinger Thomas, Frass Michael, Laczika Klaus, Locker Gottfried J, Quehenberger Peter, Rabitsch Werner, Schenk Peter, Knöbl Paul
Department of Medicine I, Intensive Care Unit 13i2, Medical University of Vienna, Vienna, Austria.
Vox Sang. 2006 May;90(4):294-301. doi: 10.1111/j.1423-0410.2006.00760.x.
The aim of this study was to document the effects of supplementation with a plasma-derived protein C concentrate in adult patients with infectious purpura fulminans.
We report the effect of the administration of a human protein C concentrate (Ceprotin, Baxter, Vienna, Austria) in eight adult patients with purpura fulminans. Five patients received the concentrate as level-adjusted continuous infusion (10 U/kg/h, target protein C activity 100%) and three patients received the concentrate as bolus infusions (100 U/kg every 6 h) in addition to standard sepsis therapy. Heparin, fresh-frozen plasma, antithrombin- and fibrinogen concentrates, low-dose rtPA, and platelet transfusions were given when appropriate.
Six patients had overt disseminated intravascular coagulation: platelets, 19 g/l; fibrinogen, 60 mg/dl; antithrombin, 47%; prothrombin time, 32%; activated partial thromboplastin time (APTT), 88 s; d-dimer, 66 microg/ml; protein C activity, 29% (medians). Five patients had septic shock, six renal failure and four respiratory failure. Patients received between 5000 and 77,000 U of protein C concentrate over 2.5 days (median); the protein C activity increased to 184% (median) and coagulopathy resolved within 3 days in seven of the eight patients. Six patients survived, one died early from fulminant sepsis, and one died after 14 days from candida sepsis.
Our data suggest that treatment with a plasma-derived protein C zymogen concentrate might be a useful support in adult patients with purpura fulminans.
本研究旨在记录补充血浆源性蛋白C浓缩物对成年感染性暴发性紫癜患者的影响。
我们报告了人蛋白C浓缩物(Ceprotin,百特公司,奥地利维也纳)对8例成年暴发性紫癜患者的治疗效果。5例患者接受浓缩物按水平调整的持续输注(10 U/kg/h,目标蛋白C活性100%),3例患者除接受标准脓毒症治疗外,还接受浓缩物大剂量输注(每6小时100 U/kg)。在适当的时候给予肝素、新鲜冰冻血浆、抗凝血酶和纤维蛋白原浓缩物、低剂量rtPA以及血小板输注。
6例患者出现明显的弥散性血管内凝血:血小板计数为19 g/l;纤维蛋白原为60 mg/dl;抗凝血酶为47%;凝血酶原时间为32%;活化部分凝血活酶时间(APTT)为88秒;D-二聚体为66 μg/ml;蛋白C活性为29%(中位数)。5例患者出现感染性休克,6例出现肾衰竭,4例出现呼吸衰竭。患者在2.5天内接受了5000至77,000 U的蛋白C浓缩物(中位数);蛋白C活性增加至184%(中位数),8例患者中有7例的凝血病在3天内得到缓解。6例患者存活,1例因暴发性脓毒症早期死亡,1例在念珠菌性脓毒症14天后死亡。
我们的数据表明,血浆源性蛋白C酶原浓缩物治疗可能对成年暴发性紫癜患者有有益的支持作用。