Coagulation Disorders Associated with Vascular Anomalies

Abstract

Coagulation disorders have been associated with vascular anomalies. Kasabach–Merritt phenomenon (KMP) describes thrombocytopenia and consumptive coagulopathy in the presence of a kaposiform hemangioendothelioma (KHE) or a tufted angioma (TA). A variant of KMP is associated with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). Localized intravascular coagulopathy (LIC) is encountered in 45 % of venous malformations, and it is characterized by high D-dimer level, low fibrinogen, and normal platelet count. All these patients are at risk of potential aggravation to disseminated intravascular coagulopathy (DIC). Clinicians and biologists must work in concert to diagnose the vascular anomaly and understand the pathophysiology of the coagulation disorder. Although the coagulation activation always generates thrombocytopenia and elevation of D-dimer levels, the targets of the treatment are different: tumor for KMP and consumption of the coagulation factors for LIC.