title: Coagulation contact phase factors and inhibitors in beta-thalassemia major children.
abstract: Selected hemostatic parameters of 23 children affected by beta-thalassemia major were studied and compared to an age- and sex-matched group. Plasma prekallikrein level was reduced in all patients, splenectomized or not. In splenectomized patients, platelet count and in vitro platelet aggregability were significantly increased and Protein C was slightly increased. The activated partial thromboplastin time was prolonged and the normotest reduced. Finally, a reduction in the plasma levels of fibrinogen and of vitamin K-dependent proteins, including the antithrombotic Protein C, was observed in nonsplenectomized patients. Our data indicate that the hemostatic system in patients with thalassemia major may be altered. The relationship between these laboratory changes and clinical manifestations remains to be established.
authors.affiliation: Institute of Clinical and Preventive Pediatrics, University of Bari, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Schettini
authors.forename: D
authors.initials: D
authors.lastname: De Mattia
authors.forename: G
authors.initials: G
authors.lastname: Arcamone
authors.forename: V
authors.initials: V
authors.lastname: Sabato
authors.forename: M
authors.initials: M
authors.lastname: Altomare
authors.forename: M G
authors.initials: MG
authors.lastname: Burattini
authors.forename: F
authors.initials: F
authors.lastname: Fedele
authors.forename: A
authors.initials: A
authors.lastname: Compagnone
authors.forename: G
authors.initials: G
authors.lastname: Ciavarella
mesh_terms: D000293:Adolescent; D001778:Blood Coagulation Disorders / Q000209:etiology*; D002648:Child; D003131:Combined Modality Therapy; D003676:Deferoxamine / Q000627:therapeutic use; D006801:Humans; D010974:Platelet Aggregation; D010976:Platelet Count; D011288:Prekallikrein / Q000032:analysis; D011486:Protein C / Q000032:analysis; D013156:Splenectomy; D013789:Thalassemia / Q000097:blood / Q000150:complications* / Q000628:therapy
chemical_list: D011486:Protein C; D011288:Prekallikrein; D003676:Deferoxamine