Chapter: Coagulation disorders
Article: 3 of 6
Update: Feb 24, 2021
Author(s): Wohlgemuth, Walter A.
In patients with kaposiform hemangioendothelioma (KHE), tufted angioma (TA) and Kasabach-Merritt phenomenon or extensive venous malformation (VM) with localized intravascular coagulation (LIC), coagulation activation via the vascular anomaly may result in massive consumption of coagulation factors and platelets, leading to clinically manifest bleeding. This high consumption can far exceed the reproduction and replacement of necessary clotting factors and platelets, resulting in disseminated intravascular coagulation (DIC). This particularly manifests itself in subcutaneous local hematomas and bleeding even without adequate trauma. This is exacerbated in severe thrombocytopenia by the formation of petechial hemorrhages on the skin (petechiae) and/or mucous membranes (especially recurrent, severe gastrointestinal bleeding that can hardly be stopped).