期刊
JOURNAL OF DERMATOLOGY
卷 42, 期 4, 页码 401-404出版社
WILEY-BLACKWELL
DOI: 10.1111/1346-8138.12797
关键词
case report; Kasabach-Merritt phenomenon; mini-review; sirolimus; treatment
类别
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 0.8mg/m(2) twice daily, the lesions shrank and the platelet count improved and remained normal 4months after initial therapy. Current treatments for KMP are not particularly effective. Sirolimus at 0.8mg/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.
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