title: Elevated hypercoagulability markers in hemoglobin SC disease.
abstract: Hemoglobin SC disease is a very prevalent hemoglobinopathy; however, very little is known about this condition specifically. There appears to be an increased risk of thromboembolic events in hemoglobin SC disease, but studies evaluating the hemostatic alterations are lacking. We describe the findings of a cross-sectional observational study evaluating coagulation activation markers in adult patients with hemoglobin SC, comparing them with those in sickle cell anemia patients and healthy controls. A total of 56 hemoglobin SC and 39 sickle cell anemia patients were included in the study, all in steady state, and 27 healthy controls. None of the patients was taking hydroxyurea. Hemoglobin SC patients had a significantly up-regulated relative expression of tissue factor, as well as elevations in thrombin-antithrombin complex and D-dimer, in comparison to controls (P<0.01). Hemoglobin SC patients had lower tissue factor expression, and thrombin-antithrombin complex and D-dimer levels when compared to sickle cell anemia patients (P<0.05). Markers of endothelial activation (soluble thrombomodulin and soluble vascular cell adhesion molecule-1) and inflammation (tumor necrosis factor-alpha) were both significantly elevated in hemoglobin SC patients when compared to controls, being as high as the levels seen in patients with sickle cell anemia. Overall, in hemoglobin SC patients, higher hemolytic activity and inflammation were associated with a more intense activation of coagulation, and hemostatic activation was associated with two very prevalent chronic complications seen in hemoglobin SC disease: retinopathy and osteonecrosis. In summary, our results demonstrate that hemoglobin SC patients have a hypercoagulable state, although this manifestation was not as intense as that seen in sickle cell anemia.
authors.affiliation: Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.
authors.forename: Marina P
authors.initials: MP
authors.lastname: Colella
authors.affiliation: Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.
authors.forename: Erich V
authors.initials: EV
authors.lastname: de Paula
authors.affiliation: Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.
authors.forename: João A
authors.initials: JA
authors.lastname: Machado-Neto
authors.affiliation: Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.
authors.forename: Nicola
authors.initials: N
authors.lastname: Conran
authors.affiliation: Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.
authors.forename: Joyce M
authors.initials: JM
authors.lastname: Annichino-Bizzacchi
authors.affiliation: Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.
authors.forename: Fernando F
authors.initials: FF
authors.lastname: Costa
authors.affiliation: Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.
authors.forename: Sara T
authors.initials: ST
authors.lastname: Olalla Saad
authors.affiliation: Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas Currently at the Department of Internal Medicine, University of São Paulo at Ribeirão Preto Medical School, São Paulo, Brazil ftraina@fmrp.usp.br fabiolatraina@gmail.com.
authors.forename: Fabiola
authors.initials: F
authors.lastname: Traina
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D001777:Blood Coagulation; D003430:Cross-Sectional Studies; D042783:Endothelial Cells / Q000378:metabolism; D005260:Female; D015870:Gene Expression; D006450:Hemoglobin SC Disease / Q000097:blood* / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D006461:Hemolysis; D006801:Humans; D018836:Inflammation Mediators; D007962:Leukocytes / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D019851:Thrombophilia / Q000097:blood*; D013925:Thromboplastin / Q000235:genetics
chemical_list: D015415:Biomarkers; D018836:Inflammation Mediators; D013925:Thromboplastin