title: Thromboelastographic characterization of the activated clotting system in children with sickle cell trait or sickle cell disease.
abstract: BACKGROUND
  Recent epidemiological evidence suggests sickle cell disease (SCD) and sickle cell trait (SCT) is a risk factor for venous thromboembolism. The increased in-vivo markers of thrombin generation support the notion that such patients are in a chronic hypercoagulable state. In an attempt to better understand the underlying mechanism, global hemostatic assays including thrombin generation assay (TGA) and thromboelastography (TEG) have been utilized by several groups, but thus far, have shown inconsistent results either due to small sample size or technical differences.
  
  
  OBJECTIVE
  Global hemostatic characterization of children with SCD or SCT by using TGA and modified TEG methods.
  
  
  METHODS
  In this pilot study, we obtained TGA, TEG and other hemostatic data on specimens from 13 patients with SCD, 14 with SCT and 12 race-matched healthy controls (NC).
  
  
  RESULTS
  R time and K time with modified TEG methods were significantly shorter in SCD when compared to SCT and NC. Alpha and MA did not show any significant differences between the groups. There was no difference seen between SCT and NC. TGA profiles did not show any difference between the three groups. As expected the in-vivo markers of thrombin generation and activation of fibrinolysis including D dimer and thrombin-antithrombin complexes were significantly higher in SCD subjects as compared to SCT and NC.
  
  
  CONCLUSIONS
  The modified TEG methods are able to detect the activated coagulation system for the SCD population but a larger and more homogenous SCT cohort needs to be studied for more conclusive results.
authors.affiliation: Division of Transfusion Medicine and Coagulation, Baylor College of Medicine, Houston, TX, United States. Electronic address: shveta85g@gmail.com.
authors.forename: Shveta
authors.initials: S
authors.lastname: Gupta
authors.affiliation: Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, CA, United States.
authors.forename: Roxana
authors.initials: R
authors.lastname: Carmona
authors.affiliation: Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, CA, United States.
authors.forename: Jemily
authors.initials: J
authors.lastname: Malvar
authors.affiliation: Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, CA, United States; University of Southern California, Los Angeles, CA, United States.
authors.forename: Guy
authors.initials: G
authors.lastname: Young
mesh_terms: D000293:Adolescent; D000328:Adult; D000990:Antithrombin III; D001777:Blood Coagulation; D002648:Child; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D006450:Hemoglobin SC Disease / Q000097:blood* / Q000150:complications; D006801:Humans; D008297:Male; D010447:Peptide Hydrolases / Q000097:blood; D010865:Pilot Projects; D012805:Sickle Cell Trait / Q000097:blood* / Q000150:complications; D013916:Thrombelastography; D013917:Thrombin / Q000032:analysis; D019851:Thrombophilia / Q000097:blood* / Q000209:etiology; D055815:Young Adult
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; C046193:antithrombin III-protease complex; C036309:fibrin fragment D; D000990:Antithrombin III; D010447:Peptide Hydrolases; D013917:Thrombin
keywords: Children; Coagulation; Sickle cell disease; Sickle cell trait; Thrombin generation assay; Thromboelastography