文本框: KASABACH-MERRITT SYNDROME: CLINICAL COURSE OF FIFTEEN PATIENTS Terashima K, Nakamura M, Takashima Y, Amano K, Horikoshi Y, Mimaya J Shizuoka Children's Hospital, Shizuoka, Japan Objective: Reviewing fifteen patients, we evaluated the therapeutic options for Kasabach-Merritt syndrome (KMS) and attempted to identify the optimal combination of treatments. Methods: A retrospective analysis was performed on fifteen KMS patients. Hematologic data, size of hemangioma and clinical symptoms were evaluated in relation to the response to steroid, radiation, interferon alfa (IFN-α) and other treatments. Results: Hemangioma was diagnosed at birth in eleven of the 15 patients (9 boys and 6 girls) and thrombocytopenia was also diagnosed at birth in six. Airway obstruction was diagnosed in four, and three required intubation. Severe breeding occurred in two, and heart failure was diagnosed in three. Eleven patients were given steroid and radiation, and four of them were additionally given IFN-α. Three patients were irradiated only and one patient was given steroid only. Steroid therapy was initially effective in most cases but relapse was common. Ten of 14 patients irradiated responded, including patients given several courses of radiation for relapse. Two of four patients given IFN-αresponded, but it is difficult to evaluate the efficiency of IFN-α, since the duration of its administration was short. All fifteen patients are alive. Conclusion: Based on the fifteen cases reviewed, we recommend early initiation of radiation, in addition to steroid administration. When radiation fails, it is essential to try other possible methods of treatment immediately. 1938