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Kasabach-Merritt phenomenon: a report of 11 cases from a single institution
Naoko Yasui1, Katsuyoshi Koh, Motohiro Kato
1Departments of *Hematology/Oncology †Radiology ‡Pathology §Division of Plastic Surgery, Saitama Children's Medical Center, Magome, Saitama, Japan.
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.
