title: Sickle cell disease is associated with iron mediated hypercoagulability.
abstract: Sickle cell disease (SCD) is associated with a significant hypercoagulable state and several hemostatic anomalies have been identified in this disease state. Of interest, SCD patients can become iron overloaded after transfusion, and iron can enhance fibrinogen as a substrate for thrombin, resulting in thrombi that commence coagulation quickly and form rapidly. We hypothesized that SCD patients would display hypercoagulable plasma coagulation kinetics and an iron enhancement of coagulation. After obtaining IRB approval, we assessed coagulation kinetics and iron enhancement with viscoelastic methods in archived, citrated plasma obtained from ambulatory or hospitalized SCD patients (n = 20). All SCD patients had plasmatic hypercoagulability, and 65 % were positive for iron enhancement of coagulation. In conclusion, continuing investigation correlating such viscoelastic data with clinical symptoms may provide insight into the role played by iron in the setting of SCD, including complications such as vaso-occlusive crisis.
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, Duke University, Durham, NC, USA.
authors.forename: Nirmish
authors.initials: N
authors.lastname: Shah
authors.forename: Ian J
authors.initials: IJ
authors.lastname: Welsby
authors.forename: Martha A
authors.initials: MA
authors.lastname: Fielder
authors.forename: Wayne K
authors.initials: WK
authors.lastname: Jacobsen
authors.forename: Vance G
authors.initials: VG
authors.lastname: Nielsen
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications; D001777:Blood Coagulation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007501:Iron / Q000097:blood*; D019190:Iron Overload / Q000097:blood / Q000209:etiology; D008297:Male; D019851:Thrombophilia / Q000097:blood / Q000209:etiology
chemical_list: D007501:Iron