Antifibrinolytic therapy in the management of the Kasabach Merritt syndrome.
The Kasabach Merritt syndrome consists of thrombocytopenia, microangiopathic hemolytic anemia, and a localized consumption coagulopathy that develops within the abnormal vascular channels of a hemangioma. In general, these patients demonstrate only mild abnormalities of screening clotting tests, but they can potentially develop life-threatening complications. We present a patient who developed a severe anemia that was refractory to erythrocyte transfusions. Treatment with epsilon-aminocaproic acid to inhibit fibrinolysis and cryoprecipitate to replenish his deficient circulating fibrinogen interrupted the cycle of his systemic coagulopathy and enabled us to transfuse him to a normal hematocrit.
Ortel
T L
TL
Department of Medicine, Duke University Medical Center, Durham, North Carolina 27710.
Onorato
J J
JJ
Bedrosian
C L
CL
Kaufman
R E
RE
D000328:Adult; D015119:Aminocaproic Acid / Q000627:therapeutic use; D000743:Anemia, Hemolytic / Q000097:blood / Q000150:complications* / Q000628:therapy; D000933:Antifibrinolytic Agents / Q000627:therapeutic use*; D001132:Arm; D001772:Blood Cell Count; D001803:Blood Transfusion; D004211:Disseminated Intravascular Coagulation / Q000097:blood / Q000150:complications* / Q000628:therapy; D006391:Hemangioma / Q000150:complications*; D006400:Hematocrit; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local; D012782:Shoulder; D013577:Syndrome; D013921:Thrombocytopenia / Q000150:complications*
D000933:Antifibrinolytic Agents; D015119:Aminocaproic Acid