摘要
目的总结气压法治疗小儿Kasabach—Merritt现象（KMP）的疗效和安全性。方法回顾性分析1997年10月至2008年5月接受气压法治疗的17例KMP患儿的临床资料。17例中男11例，女6例；年龄3d～3岁，中位年龄3．5个月；病变位于躯干8例，下肢5例，上肢4例；瘤体最大直径均〉8cm；血小板均〈100×10^9／L，血红蛋白均〈110g／L，血清纤维蛋白原均〈2．0g／L。气压法治疗应用自行设计的“气压式血管瘤治疗器”（专利号：ZL97232266．3）。瘤体局部取材活检或手术切除组织病理检查以明确KMP的病理性质。结果2例患儿分别接受4和6个月的气压法治疗后瘤体消失，血液指标恢复正常，随访11个月和5年无复发，疗效评定为治愈；11例接受4～6个月气压法治疗，随访6～24个月病情继续好转，未再治疗，疗效评定为显效；2例接受6个月气压法治疗后随访5个月和2年，病情无进展，疗效评定为好转；2例接受气压法治疗6个月无效，改行手术切除治疗。组织病理学检查证实17例KMP中卡波西形血管内皮瘤14例，丛状血管瘤3例。结论气压法对四肢、躯干部位的KMP疗效确切，无明显不良反应。
Objective To summarize the efficacy and safety of employing pneumatic compression therapy in infants with Kasabach-Merritt phenomenon （KMP）. Methods Seventeen patients with KMP （ 11 males, 6 females） were treated with pneumatic compression therapy from October 1997 to May 2008. And their clinical characteristics, course of treatment and clinical and laboratory data were retrospectively analyzed. Among 17 patients, 8 cases were located in trunk, 5 in lower extremities and 4 in upper extremities. The diameters of lesions exceeded 8 cm in all patients. The platelet count was all 〈 100×10^9/L while hemoglobin 〈 110 g/L and fibrinogen 〈 2. 0 g/L. The self-designed device for pneumatic compression henmangioma therapy was employed （ Patent No： ZL97232266.3 ）. Biopsy and exairesis were performed from the local lesions with KMP in order to determine the pathological features. Results Two patients were cured after pneumatic compression therapy for 4 and 6 months respectively, and their lesions disappeared, blood parameters became normal and remained relapse-free after a 5/11-year follow-up. Eleven patients were effective after pneumatic compression therapy for 4 - 6 months, and improvement was demonstrated after 6 - 24 months follow-up without any treatment. Two patients showed improvement after pneumatic compression therapy for 6 months after a follow-up for 5 months or 2 years without progression. After a 6-month pneumatic compression therapy, 2 ineffective patients underwent surgical resection. There were 14 cases of kaposiform hemangioendothelioma （ KHE ） and 3 cases of tufted hemangioma （ TA ）. Conclusion Pneumatic compression therapy has definite curative effects for KMP lesions in extremities and trunk and its side effects are fewer.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2009年第26期1830-1833,共4页
National Medical Journal of China
关键词
血管内皮瘤
贫血
溶血性
血小板减少
气体压缩疗法
Hemangioendothelioma
Anemia, hemolytic
Thrombocytopenia
Pneumatic compression therapy
