title: Thrombin activatable fibrinolysis inhibitor (TAFI): relationship to hemostatic alteration in patients with beta-thalassemia.
abstract: Profound hemostatic changes have been observed among thalassemic patients. Thrombin activatable fibrinolysis inhibitor (TAFI) is a newly discovered protein that potentially attenuates fibrinolysis. The authors aimed to investigate plasma level of TAFI in beta-thalassemia patients in relation to clinical severity and hemostatic alteration. Fifty-one thalassemic patients (mean age 10.79 +/- 5.59 years) (21 splenectomized thalassemia major patients, 18 nonsplenectomized thalassemia major patients, 12 nonsplenectomized thalassemia intermedia) were recruited from Pediatric Hematology Clinic, Ain Shams University; in addition, 32 healthy age- and sex-matched controls (10.31 +/- 5.58 years) were also included. In addition to clinical assessment, laboratory investigations included complete blood count (CBC), hemoglobin electrophoresis, prothrombin time (PT), activated partial thromboplastin time (PTT), liver function tests, viral hepatitis markers, serum ferritin, and plasma TAFI levels. Nine out of 51 patients (17.5%) suffered from bleeding manifestations mainly in the form of epistaxis; none of the studied patients had thromboembolism. Significant reduction in TAFI levels was shown in thalassemic patients compared to controls (P < .0001), in splenectomized compared to nonsplenectomized thalassemia group (P < .0001), and in thalassemia major compared to thalassemia intermedia group (P < .0001). Negative correlation was present between TAFI levels and both liver enzymes and serum ferritin levels (P < .05). Thalassemic patients suffering from bleeding showed lower mean TAFI levels compared to those not suffering from bleeding (P < .001). Marked reduction in TAFI levels was observed in thalassemic patients with splenectomy, altered liver functions, and poor chelation who therefore might be at a higher risk for altered hemostasis.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Galila M
authors.initials: GM
authors.lastname: Mokhtar
authors.forename: Randa M
authors.initials: RM
authors.lastname: Matter
authors.forename: H
authors.initials: H
authors.lastname: Shawki
authors.forename: Manal M
authors.initials: MM
authors.lastname: Abdel Aziz
mesh_terms: D000293:Adolescent; D025901:Carboxypeptidase B2 / Q000097:blood*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004796:Clinical Enzyme Tests; D004844:Epistaxis; D005260:Female; D005293:Ferritins / Q000097:blood; D006470:Hemorrhage; D006487:Hemostasis; D006801:Humans; D008099:Liver / Q000201:enzymology; D008111:Liver Function Tests; D008297:Male; D012720:Severity of Illness Index; D013156:Splenectomy; D017086:beta-Thalassemia / Q000097:blood* / Q000175:diagnosis
chemical_list: D005293:Ferritins; D025901:Carboxypeptidase B2