气压法治疗小儿Kasabach-Merritt现象17例分析

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3 d～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>8 cm;血小板均<100×109/L,血红蛋白均<110 g/L,血清纤维蛋白原均<2.0 g/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.

Clinical analysis of 17 eases of pneumatic compression therapy in infants with Kasabach-Merritt phenomenon

Objective To summarize the efficacy and safety of employing pneumatic compression therapy in infants with Kasabaeh-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 eases 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 × 109/Lwhile hemoglobin < 110 g/L and fibrinogen < 2. 0 g/L. The serf-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 lesiorts in extremities and trunk and its side effects are fewer.