title: Hypercoagulable state as demonstrated by thromboelastometry in hemoglobin E/beta-thalassemia patients: Association with clinical severity and splenectomy status.
abstract: BACKGROUND
  Patients with hemoglobin E/beta-thalassemia disease (E/β) are at risk of thromboembolism. Rotational thromboelastometry (ROTEM®) can be used to determine a hypercoagulable state. The objective was to describe the hemostatic and thromboelastometric changes in pediatric patients with E/β with different clinical severity, in comparison with healthy children as controls.
  
  
  METHODS
  Fifty-three pediatric patients with E/β and 21 healthy children were enrolled. The clinical severity of E/β was categorized by using the clinical severity scores. All subjects were tested for complete blood count, protein C activity (PC), total protein S (PS), antithrombin (AT), D-dimer and fibrinogen (Fib) levels and thromboelastometry, measured by ROTEM®.
  
  
  RESULTS
  The levels of PC (65.7 vs 118.5%), PS (46.8 vs 78.4%), AT (95.7 vs 105.7%) and Fib (217 vs 294 mg/dL) were significantly lower, and the platelet count (PLT) was significantly higher in the patient group than the controls. The maximum clot firmness (MCF) of patients with moderate disease who were previously splenectomized (seven patients) and patients with severe disease (nine patients) were higher than patients who had intact spleen with moderate disease, patients with mild disease and controls (P<0.05). Only PLT had significant correlation with MCF (P<0.05).
  
  
  CONCLUSIONS
  Hypercoagulable state was demonstrated by ROTEM® in patients with E/β with severe disease and who were previously splenectomized. The hypercoagulable state was associated with the higher numbers of PLT rather than the decrease of PC, PS, and AT.
authors.affiliation: Department of Pediatrics, Chiang Mai University, Chiang Mai 50200, Thailand. Electronic address: rungrote.n@cmu.ac.th.
authors.forename: Rungrote
authors.initials: R
authors.lastname: Natesirinilkul
authors.affiliation: Department of Pediatrics, Chiang Mai University, Chiang Mai 50200, Thailand.
authors.forename: Pimlak
authors.initials: P
authors.lastname: Charoenkwan
authors.affiliation: Department of Internal Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.
authors.forename: Weerasak
authors.initials: W
authors.lastname: Nawarawong
authors.affiliation: Department of Anesthesia, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.
authors.forename: Settapong
authors.initials: S
authors.lastname: Boonsri
authors.affiliation: Department of Internal Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.
authors.forename: Pakinee
authors.initials: P
authors.lastname: Tantivate
authors.affiliation: Department of Pediatrics, Chiang Mai University, Chiang Mai 50200, Thailand.
authors.forename: Suwakon
authors.initials: S
authors.lastname: Wongjaikum
authors.affiliation: Department of Pediatrics, Chiang Mai University, Chiang Mai 50200, Thailand.
authors.forename: Suphara
authors.initials: S
authors.lastname: Manowong
authors.affiliation: Department of Pediatrics, Chiang Mai University, Chiang Mai 50200, Thailand.
authors.forename: Torpong
authors.initials: T
authors.lastname: Sanguansermsri
mesh_terms: D000293:Adolescent; D001777:Blood Coagulation; D002648:Child; D002675:Child, Preschool; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D006446:Hemoglobin E / Q000032:analysis*; D006801:Humans; D008297:Male; D010976:Platelet Count; D011486:Protein C / Q000032:analysis; D017293:Protein S / Q000032:analysis; D012307:Risk Factors; D013156:Splenectomy; D013916:Thrombelastography; D013922:Thrombocytosis / Q000097:blood / Q000150:complications*; D013923:Thromboembolism / Q000097:blood / Q000209:etiology*; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications*
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; D011486:Protein C; D017293:Protein S; C036309:fibrin fragment D; D006446:Hemoglobin E
keywords: Hemoglobin E/beta-thalassemia; Hypercoagulable state; Splenectomy; Thrombocytosis; Thromboelastometry