Refractory Kasabach-Merritt phenomenon successfully treated with sirolimus, and a mini-review of the published work.

Abstract

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.

• Authors: Zuopeng Wang, Kai Li, Kuiran Dong, Xianmin Xiao, Shan Zheng
• Journal: The Journal of dermatology (J Dermatol) Vol. 42 Issue 4 Pg. 401-4 (Apr 2015) ISSN: 1346-8138 [Electronic] England
• PMID: 25728547 (Publication Type: Case Reports, Journal Article, Review)
• Copyright: © 2015 Japanese Dermatological Association.

Chemical References

• MTOR protein, human
• TOR Serine-Threonine Kinases
• Sirolimus

Topics

• Humans
• Infant
• Kasabach-Merritt Syndrome (blood, drug therapy, pathology)
• Male
• Platelet Count
• Sirolimus (administration & dosage, therapeutic use)
• TOR Serine-Threonine Kinases (antagonists & inhibitors)