Clinical Treatment Of Kaposiform Hemangioendothelioma

Objective:To explore the clinical treatment of kaposiform hemangioendothelioma(KHE).Methods: To make a retrospective analysis of the clinical data of 11 patients with kaposiform hemangioendothelioma diagnosed in Union Hospital Affiliated to Fujian Medical University from 2009 to 2015,the data collection includes tumor size,platelet count and coagulation function at admission,bleeding tendency of patients,drug treatment plan after admission,tumor body changes and platelet changes after treatment,prognosis of patients after discharge,post-drug complication and other follow-up data,and preliminary exploration of clinical treatment options for KHE with platelet count above or below critical value.Results:Among the 11 patients,except that the propranolol was directly used for a mild children patient with platelet count and coagulation function,and small tumor.One year after taking the drug,the tumor disappeared,with remarkable effect.The remaining 10 cases were routinely treated with glucocorticoid combined with propranolol in the beginning.In the 3(3/10)patients with platelets above the critical value,all the routine uses of glucocorticoid alone(or not)combined with propranolol acquired a very good effect,the tumor becoming smaller,getting the opportunity for surgical resection,and even achieving a survival without the tumor.However,as for the other 7(7/10)patients with platelet below the critical value,3 patients(3 / 7)had received initial treatment with glucocorticoid and propranolol alone,of which 1 patient was lost to follow-up and 1 patient had too low platelet count,and cranial hemorrhage occurred after combined application of vincristine in the late stage.Intracranial hematoma caused severe cerebral palsy,and multiple patients with multiple organ failure died.And in one patient after treated with the combined application of vincristine chemotherapy,the platelet did not increase,and continued to be below the critical value.Therefore,the treatment plan was adjusted,where vincristine was abandoned while sirolimus was taken in combination,the count of patient's platelets gradually recovered to normal levels after one week.As for the remaining 4 patients(4/7),at the beginning of treatment,the combination of glucocorticoid,propranolol and sirolimus was used directly,it has obvious curative effect,and the count of patients' platelets gradually increased within 3-12 days,with the tumor becoming softer,smaller,as well as lighter than before in color.Conclusion: Taking the platelet critical value as the marginal value,and for patients above platelet critical value,glucocorticoid combined with propranolol will be routinely used firstly.And whether to add vincristine for chemotherapy according to the situation will be considered then.Yet for patients below platelet critical value,sirolimus will be used at the same time under the condition of treatment with glucocorticoid and propranolol.