Abstract
Objective To investigate clinical features and therapeutic methods for infants with Kasabach-Merritt syndrome (KMS) . Methods From January 2013 to December 2016, eight cases of infants with KMS were admitted to the Department of Pediatrics, Zhujiang Hospital of Southern Medical University. The medical history, clinical manifestations, results of laboratory tests, treatment methods and curative effect of eight cases were recorded by retrospective analysis method and the prognosis of the infants were followed up. Results Among the eight cases, the lesion location of angioma was three cases in submandibular, two cases in lower limbs, and three cases in internal organs (one case in liver, one case in pancreas, one case in mesentery). Angioma of six cases became shrunken and soft, and the color of the tumor became light after receiving drug therapy, which were glucocorticoid, α-2b interferon, rapamycin and vincristine single or combination therapy. Among them, mesenteric and pancreas angioma patients were treated with operation respectively because of life-threatening hemorrhage and giant hemangioma oppresses. The platelet was fully restored to normal in 4-30 days. The time of fully recovery of blood coagulation function was 2-50 days. The time of tumor completely disappeared was 1-2 years. Two cases due to economic reasons gave up treatment and died. Through two years follow-up, six cases were allcured. And the height, weight and organ function of the six cases were good. Conclusions Infants with KMS are in a critical condition and progress rapidly. Glucocorticoid, vincristine and rapamycin combined treatment can quickly control bleeding and improve prognosis. For the infants with critical bleeding and
vital organs oppressed by huge angioma, surgery can quickly reduce tumor size so as to relieve bleeding.
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