Kasabach — Merritt syndrome: coagulo — analytical observations

Studies are presented on a 20-year-old man with congenital giant hemangioma occupying the whole left side of the body, with alterations in the clotting system characteristic of a consumption-coagulopathy with “defensive” fibrinolysis. There were also changes in red cell morphology, consistent with “traumatic hemolytic anemia”; the platelet and red cell half-time survival were shortened to 1.8 and 15 days, respectively. Total blood volume was 10.3 liters. Following treatment with continuous IV heparin, abnormalities of the clotting mechanism were corrected including a rise in platelets from 46,000 to 330,000/mm3 fibrinogen from45 to 300 mg% and factor VIII from 20 to 120% Hemolytic parameters also improved as demonstrated by a fall in reticulocyte count and concentration of free hemoglobin in plasma. Heparin administration restored the hemostatic defenses and permitted extensive surgical intervention. After healing by first intention, heparin was discontinued and the consumption-coagulopathy with “defensive” fibrinolysis reappeared.