Kaposiform hemangioendothelioma with satellitosis and Kasabach-Merritt phenomenon

[Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon].

Kasabach-Merritt phenomenon is a serious coagulopathy associated with kaposiform hemangioendothelioma (KHE), tufted angioma, and possibly other vascular neoplasms. KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia. We retrospectively reviewed 10 cases of KHE without Kasabach-Merritt phenomenon. The tumors app...

Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with embolization and vincristine in two newborns.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor and has a high mortality in newborns when associated with Kasabach-Merritt syndrome (KMS). In two newborns with KHE and severe KMS refractory to medical treatment, emergency embolization led to clinical improvement in the acute neonatal setting by reducing tumor volume, increasing the platelet count, and improving other clotting par...

Kaposiform Hemangioendothelioma in a Newborn with Kasabach-Merritt Syndrome

Sirolimus for treatment of kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon

KHE: kaposiform hemangioendothelioma KMP: Kasabach-Merritt phenomenon INTRODUCTION Kaposiform hemangioendothelioma (KHE) was first described by Zuckerberg et al in 1992. KHE is defined as a rare, locally aggressive infiltrative vascular neoplasm that typically occurs during infancy and childhood. KHE generally originates on the skin as a distinctive cutaneous lesion with ill-defined borders, la...

The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that KMP is not caused by infantile hemangiomas. Man...