卡波西型血管内皮瘤的临床治疗分析

Clinical treatment of Kaposiform hemangioendothelioma

目的探讨卡波西型血管内皮瘤(KHE)的临床治疗方法。方法回顾性分析2009年11月至2015年11月福建医科大学附属协和医院收治的11例KHE患儿的临床资料。患儿年龄为5 d至2岁,男8例,女3例。在早期5例患儿的治疗中,不管血小板计数是多少,常规应用糖皮质激素、普萘洛尔治疗,激素每日口服4.0~4.5 mg/kg,隔日1次,普萘洛尔每日最大剂量是2 mg/kg,分3次给药,每8 h 1次。在后期6例患儿的治疗中,以血小板危急值20×109/L为临界值,对于血小板高于危急值者,常规给予激素及普萘洛尔。而对于血小板低于危急值者,在服用激素及普萘洛尔的基础上,患儿口服西罗莫司,0.8 mg/m^2,平均每12 h给药1次。其中2例患儿治疗过程中曾加用长春新碱,剂量为0.05 mg/m^2。对治疗效果进行观察、随访。结果随访3~8年,11例中3例无血小板减少,激素和(或)普萘洛尔治疗,效果较好;1例血小板20×109/L的患儿,采用激素联合普萘洛尔治疗后瘤体消失;7例血小板低于危急值的患儿,其中5例使用激素、普萘洛尔联合西罗莫司治疗,效果良好,1例使用激素、普萘洛尔联合长春新碱治疗,并发脑出血死亡,1例使用激素联合普萘洛尔治疗后失访。结论以血小板危急值为临界值,高于此值者常规先口服糖皮质激素和普萘洛尔治疗;而低于此值者在服用激素和普萘洛尔的同时,口服西罗莫司治疗,可取得较好的效果。

Objective To discuss the clinical treatment methods of Kaposiform hemangioen-dothelioma(KHE).Methods Eleven children with KHE admitted to the Union Hospital of Fujian Medical University from November 2009 to November 2015 were retrospectively analysed.Children aged from 5 days to 2 years old,8 males and 3 females.In the treatment of cases 1-5,glucocorticoids and propranolol were routinely used for treatment regardless of the platelet count.The glucocorticoids was taken orally 4.0-4.5 mg/kg every other day,and the maximum daily dose of propranolol was 2 mg/kg given by three times a day.In the treatment of cases 6-11,the critical value of platelets number was 20×109/L.For those higher than the critical value,glucocorticoids and propranolol were routinely given.For those lower than the critical value,under the premise of taking glucocorticoids and propranolol,sirolimus was givenorally by the dose of 0.8 mg/m^2 twice a day.Two of the children had been treated with vincristine at a dose of 0.05 mg/m^2.The treatment effect was observed and followed up.Results Followed up for three to eight years.Three of eleven cases did not get thrombocytopenia,and the effect of glucocorticoids combined with propranolol treatment was good.One case’s platelets number was 20×109/L.The tumor dissappered after treated with glucocorticoids combined with propranolo.Seven cases with platelets was lower than the critical value.Five in seven cases were treated with glucocorticoids,propranolol and sirolimus.The effect was good.One case,treated with glucocorticoids,propranolol and vincristine,died of cerebral hemorrhage.Another one case was lost to follow-up after treatment with glucocorticoids and propranolol.Conclusions With the critical value of platelet criticality,patients above this value are routinely treated with glucocorticoid and propranolol first while those below this value are treated with sirolimus while taking glucocorticoids and propranolol.Through this method,better results can be achieved.