[Kasabach-Merritt syndrome in children]

Kasabach-Merritt syndrome is a combination of thrombocytopenia, intravascular coagulation, and a rapid increase in the size of an angioma. Anemia and disseminated intravascular coagulation may develop. This infrequent syndrome is severe and may be life-threatening. Pathophysiologic mechanisms underlying the condition are incompletely understood and, consequently, many different treatments are used, including systemic corticosteroids, compression, embolization, antifibrinolytic agents, platelet aggregation inhibitors, irradiation, and others. From findings in eight personal cases, the authors review clinical and biological features, pathophysiologic hypotheses and therapeutic strategies.