Journal
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
Volume 35, Issue 7, Pages 554-558Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MPH.0b013e318281558e
Keywords
Kasabach-Merritt phenomenon; chemotherapy; vascular tumor
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Background:Kasabach-Merritt phenomenon (KMP) is a rare condition and optimal treatments have not yet been established, especially for cases that are unresponsive to first-line therapy. We retrospectively reviewed 11 KMP cases treated over the past 13 years in our institute.Observations:With the exception of 1 case, steroids were administered as the first-line therapy. Eight cases required second-line or third-line therapy. The effective salvage therapies include interferon (n=1), radiotherapy (n=1), and chemotherapy (n=5). One case continues to depend upon chemotherapy. Three refractory cases were therapy dependent over 1 year of age, whereas 8 were treated effectively by 6 months of age.Conclusions:Chemotherapy seems to be the most effective therapy for steroid-resistant KMP cases.
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