Kasabach-Merritt phenomenon is characterized by a normal to slightly prolonged prothrombin time and partial thromboplastin time, severe thrombocytopenia, hypofibrinogenemia, and elevated D-dimer, and affects up to 71% of patients with KHE.1,3 When unrecognized or not treated promptly, KMP can cause severe bleeding, disseminated intravascular coagulation (DIC), shock, and multi organ failure. This evidence concerns the gene F2 and Hypofibrinogenemia.