RADIATION THERAPY FOR KASABACH-MERRITT SYNDROME

ANALYSIS OF UNFAVORABLE FACTORS IN 5 CHILDREN

Abstract

Purpose: During the past 10 years, five infants with Kasabach-Merritt syndrome (K-M) received radiation therapy were reported. We investigated whether radiation therapy for K-M was useful and what the unfavorable factors of K-M were.
Material and treatment: During the past 10 years, we have treated five infants with K-M. The syndrome occurred at ages ranging from birth to 4 months. The incidence of female to male ratio was 3: 2. Among 5 cases, the site of hemangioma was as follows; shoulder, anterior chest wall, lower abdominal wall, face and neck and inguinal site. All 5 cases received medication to control the coagulopathy including prednisone and blood transfusion at first. Because the platelet count and the bleeding tendency did not improve in any case, these cases received radiation therapy.Total dose ranged from 5 to 10Gy and fraction-size ranged from 0.5 to 1.75Gy. Irradiation session was 2 or 3 times per week.
Results: In 5 cases, 4 cases showed cure of bleeding tendency and disappearance of tumor, andsur vived. In these 4 cases, normalization of platelet count was obtained at the early phase of dose ranging from 3 to 5 Gy by radiation therapy. In the remaining case, bleeding tendency was improved at the late phase by initial radiation therapy, however, after that immediately relapsed. In this case, the salvage radiation therapy was not effective and she died from airway obstruction. This case was of neonatal age and had bulky neck tumor.
Conclusion: We recognized that radiation therapy was effective for K-M.
A serious case was of neonatal age and had bulky neck tumor. It was estimated that the unfavorable factors of K-M were neonatal case and bulky neck tumor case.