Publisher: John Wiley & Sons Inc
E-ISSN: 1346-8138|42|4|401-404
ISSN: 0385-2407
Source: THE JOURNAL OF DERMATOLOGY, Vol.42, Iss.4, 2015-04, pp. : 401-404
Disclaimer: Any content in publications that violate the sovereignty, the constitution or regulations of the PRC is not accepted or approved by CNPIEC.
Abstract
AbstractKasabach–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/m2 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/m2 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.
Related content
The effects of sirolimus on Kasabach–Merritt phenomenon coagulopathy
BRITISH JOURNAL OF DERMATOLOGY, Vol. 178, Iss. 2, 2018-02 ,pp. :
Recent advances in the pathobiology and management of Kasabach–Merritt phenomenon
BRITISH JOURNAL OF HAEMATOLOGY, Vol. 7-1048, Iss. 1, 2015-10 ,pp. :
Recent advances in the pathobiology and management of Kasabach–Merritt phenomenon
BRITISH JOURNAL OF HAEMATOLOGY, Vol. 171, Iss. 1, 2015-10 ,pp. :
Kasabach–Merritt syndrome, kaposiform haemangioendothelioma and platelet blockade
BRITISH JOURNAL OF HAEMATOLOGY, Vol. 171, Iss. 1, 2015-10 ,pp. :