Kasabach-Merritt Phenomenon

• DOI: 10.1097/mph.0b013e318281558e
• Publication Date: 2013-02-06T15:16:45Z
• Authors: Naoko Yasui; Katsuyoshi Koh; Motohiro Kato; Myoung-ja Park; Daisuke Tomizawa; Koichi Oshima; Naoki Uchisaka; Yoshihiro Gocho; Ayumu Arakawa; Masafumi Seki; Eiji Oguma; Hiroshi Kishimoto; Shoji Watanabe; Akira Kikuchi; Ryoji Hanada
• Keywords: Male; Infant, Newborn; Infant; Kasabach-Merritt Syndrome; Combined Modality Therapy; 3. Good health; 03 medical and health sciences; Treatment Outcome; 0302 clinical medicine; Humans; Female; Retrospective Studies

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.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.Chemotherapy seems to be the most effective therapy for steroid-resistant KMP cases.