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Refractory Kasabach–Merritt phenomenon successfully treated with sirolimus, and a mini‐review of the published work
Author(s) -
Wang Zuopeng,
Li Kai,
Dong Kuiran,
Xiao Xianmin,
Zheng Shan
Publication year - 2015
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/1346-8138.12797
Subject(s) - sirolimus , medicine , refractory (planetary science) , consumptive coagulopathy , vincristine , coagulopathy , surgery , chemotherapy , cyclophosphamide , physics , astrobiology
Kasabach–Merritt phenomenon ( KMP ) is a rare and life‐threatening disease involving a vascular tumor combined with severe consumptive coagulopathy. We present for the first time a case of KMP with the vascular tumor involving two anatomical sites; the patient failed to respond to steroids and vincristine. Following sirolimus therapy at a dose of 0.8 mg/m 2 twice daily, the lesions shrank and the platelet count improved and remained normal 4 months after initial therapy. Current treatments for KMP are not particularly effective. Sirolimus at 0.8 mg/m 2 per dose, administrated twice daily, appears to be a safe and effective management option. It appears to be an interesting therapeutic option in refractory KMP , but the time to response is variable.