CHRONIC DEFIBRINATION SYNDROME DUE TO A GIANT HEMANGIOMA ASSOCIATED WITH MICROANGIOPATHIC HEMOLYTIC ANEMIA

An eight year old boy with the Kasabach-Merritt syndrome was studied with respect to consumption coagulopathy, which was demonstrated by simultaneous coagulation studies performed on venous and hemangioma blood. Venous blood samples showed only thrombocytopenia, moderate fibrinogenopenia and factor IX deficiency; whereas hemangioma blood samples revealed prolonged recalcification, one-stage prothrombin, cephalin-Stypven,® thrombin, calcified thrombin, P and P and partial thromboplastin times. There was a plasma defect in the thromboplastin generation test. Besides thrombocytopenia and a more pronounced fibrinogenopenia, deficiencies of factors V, VIII and IX and an increase in the fibrinolytic activity were found. The factor IX deficiency was considered to be a coincidental finding. The patient also presented signs of microangiopathic hemolytic anemia, thought to be related to the giant hemangioma. © 1969.