title: Intravascular hemolysis, vascular endothelial cell activation and thrombophilia in splenectomized patients with hemoglobin E/β-thalassemia disease.
abstract: The relationship between asplenia and thrombophilia in β-thalassemia disease patients is not yet completely understood. One hundred and ten adult hemoglobin (Hb) E/β-thalassemia (E/β-Thal) disease outpatients, dichotomized according to the presence or absence of the spleen, were prospectively studied for evidence of intravascular hemolysis (IVH) and vascular endothelial cell (EC) activation. Biomarkers of IVH (serum cell-free Hb), EC [soluble E-selectin (sE-selectin) and soluble vascular cell adhesion molecule 1 (sVCAM-1)], platelet and EC [soluble P-selectin (sP-selectin)], inflammation [high-sensitivity C-reactive protein (hs-CRP)], and coagulation [thrombin-antithrombin complexes (TAT)] activation, as well as other selected blood tests were determined. The 61 splenectomized patients had a more severe hemolytic disease and higher levels of cell-free Hb and ferritin (p = 0.003), sE-selectin, sP-selectin, hs-CRP, and TAT (p < 0.05). However, serum levels of sVCAM-1 were not different between the two groups. The findings suggested IVH and EC activation. Together with chronic iron overload and chronic low-grade inflammation activation, the findings extend our understanding of the mechanism of thrombophilia in splenectomized E/β-Thal disease patients.
authors: Division of Hematology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors: Vichai
authors: V
authors: Atichartakarn
authors: Suporn
authors: S
authors: Chuncharunee
authors: Napaporn
authors: N
authors: Archararit
authors: Umaporn
authors: U
authors: Udomsubpayakul
authors: Katcharin
authors: K
authors: Aryurachai
mesh_terms: D000328:Adult; D000990:Antithrombin III; D015415:Biomarkers / Q000097:blood; D002097:C-Reactive Protein / Q000378:metabolism; D019040:E-Selectin / Q000097:blood; D042783:Endothelial Cells / Q000473:pathology; D005260:Female; D006446:Hemoglobin E / Q000378:metabolism*; D006461:Hemolysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D019007:P-Selectin / Q000097:blood; D010447:Peptide Hydrolases / Q000097:blood; D011183:Postoperative Complications / Q000209:etiology; D011446:Prospective Studies; D013156:Splenectomy / Q000009:adverse effects*; D019851:Thrombophilia / Q000097:blood* / Q000209:etiology* / Q000473:pathology; D019010:Vascular Cell Adhesion Molecule-1 / Q000097:blood; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood* / Q000601:surgery*
chemical_list: D015415:Biomarkers; D019040:E-Selectin; D019007:P-Selectin; C546936:SELE protein, human; D019010:Vascular Cell Adhesion Molecule-1; C046193:antithrombin III-protease complex; D000990:Antithrombin III; D002097:C-Reactive Protein; D006446:Hemoglobin E; D010447:Peptide Hydrolases