title: [Thrombotic microangiopathy after extracorporeal circulation: important differential diagnosis].
abstract: Thrombotic microangiopathies are characterized by platelet activation, endothelial damage, hemolysis and microvascular occlusion. This group of diseases is primary represented by thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Patients present with microangiopathic hemolytic anemia and thrombocytopenia as well as occlusion-related organ ischemia to a variable degree. A deficiency of the metalloprotease ADAMTS-13 is a major risk for acute disease manifestation as this is a regulator of unusually large von Willebrand factor (vWF) multimers, which are extremely adhesive and secreted by endothelial cells. In classical TTP an ADAMTS-13 activity below 5% is specific, whereas in other forms of thrombotic microangiopathies activity of ADAMTS-13 ranges from very low to normal. Symptoms of different forms of thrombotic microangiopathy are frequently overlapping and a clear classification according to clinical criteria is often difficult. Due to a high mortality, particularly of TTP, immediate diagnosis and therapy are essential. In this article two cases of thombotic microangiopathy after cardiac surgery are reported. After exclusion of TTP and HUS as well as other etiologies of thrombotic microangiopathy a relationship between the use of extracorporeal circulation and the pathogenesis of thrombotic microangiopathy is assumed.
authors.affiliation: Departement Anästhesie und Intensivmedizin, Universitätsspital Basel, Schweiz. schmidtT@uhbs.ch
authors.forename: T
authors.initials: T
authors.lastname: Schmidt
authors.forename: D A
authors.initials: DA
authors.lastname: Tsakiris
authors.forename: M
authors.initials: M
authors.lastname: Grapow
authors.forename: M
authors.initials: M
authors.lastname: Siegemund
mesh_terms: D051722:ADAM Proteins / Q000235:genetics; D000071120:ADAMTS13 Protein; D000369:Aged, 80 and over; D001018:Aortic Diseases / Q000150:complications / Q000601:surgery; D001241:Aspirin / Q000627:therapeutic use; D003128:Coma / Q000209:etiology; D003422:Critical Care; D003937:Diagnosis, Differential; D005112:Extracorporeal Circulation / Q000009:adverse effects*; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000209:etiology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000150:complications / Q000601:surgery; D016491:Peripheral Vascular Diseases / Q000175:diagnosis* / Q000209:etiology* / Q000235:genetics; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011183:Postoperative Complications / Q000235:genetics / Q000628:therapy; D011696:Purpura, Thrombocytopenic / Q000209:etiology / Q000628:therapy; D012307:Risk Factors; D013927:Thrombosis / Q000209:etiology*; D014841:von Willebrand Factor / Q000235:genetics
chemical_list: D010975:Platelet Aggregation Inhibitors; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human; D001241:Aspirin