新鲜冰冻血浆治疗和预防遗传性血管性水肿急性发作.docVIP

新鲜冰冻血浆治疗和预防遗传性血管性水肿 汤蕊 张宏誉( 中国医学科学院 北京协和医学院 北京协和医院 变态反应科，北京 100730 [摘要]目的：探讨新鲜冰冻血浆（FFP）治疗遗传性血管性水肿（HAE）的适应症、安全性和有效性。 方法：回顾性分析北京协和医院3例FFP治疗HAE急性发作期和短期预防性治疗的病例。 结果：FFP可用于HAE急性发作期治疗和预防性治疗，对HAE-Ⅰ型和Ⅱ型患者均有效，适应症为喉水肿、腹痛等急性发作，和拔牙等手术操作的预防性治疗，本研究输注量为400ml，3例患者中2例患者治疗后缩短了发作时间，控制了发作症状，1例患者治疗后预防急性发作，其中1例次出现过敏反应，停止输注后缓解。Fresh frozen plasma for the treatment of hereditary angioedema TANG Rui ZHANG Hongyu Department of Allergy, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China [Abstract] Objective：To research patient records to determine whether FFP is effective and safety for the treatment of HAE and the indication of FFP. Methods：Retrospective analyze 3 patients with HAE in Peking Union medical college hospital who received FFP 6 times to determine if there was evidence that FFP was safe and effective for HAE patients. Results：3 case reports suggest that FFP is an effective prophylactic agent before surgery and for treatment of acute HAE attacks. FFP is effective for type Ⅰand type Ⅱ patients of HAE. Indication of FFP is acute attacks of laryngeal edema and severe abdominal pain, prevention of dental procedures and surgery. The dosage of FFP is 400ml in the study. The symptom of 2 patients alleviated in shorter period, and 1 patient did not appear symptom during dental extraction after FFP treatment. Only 1 patient appeared skin rash once, others had no adverse reaction. Conclusion：FFP seems to be safe and effective in preventing exacerbations of HAE before surgery and for acute attacks of HAE. [Key words] Hereditary angioedema; Fresh frozen plasma; Treatment 遗传性血管性水肿是一种罕见的常染色体显性遗传病，发病率约为1/50,000[1]，病因C1INH）的含量减低功能缺陷。其典型临床表现为反复发作、局限性、自限性皮肤及粘膜水肿。HAE主要分为两型：HAE-型也称为HAE普通型，约占HAE总人数的85%，为C1INH含量减低和功能缺陷。HAE-型也称为HAE变异型，约占HAE总人数的15%，C1INH的含量正常甚至增加，但C1INH的功能减低[]。 [2]、纳米滤过的C1INH[3]、重组C1INH[4]、激肽释放酶抑制剂[5]、缓激肽受体拮抗剂[6]，可用于急性发作期和预防性治疗，但我国尚无新鲜冰冻血浆（Fresh-frozen plasmaFFP）对HAE急性期治疗有效[]，本文例的。男性，13岁反复发作四肢、颜面、臀部水肿4年，数月至