BACKGROUND: Sickle cell disease is recognized as a hypercoagulable state; however, the frequency and characteristics of venous thromboembolism in sickle cell patients have not been well defined. The purpose of this study was to establish the prevalence and risk factors for venous thromboembolism in a large cohort of patients with sickle cell disease and determine the relationship between venous thromboembolism and mortality. METHODS: We performed a cross-sectional study of 404 sickle cell disease patients cared for at the Sickle Cell Center for Adults at Johns Hopkins. Demographic, sickle cell disease-specific comorbidity, and venous thromboembolism data were collected on all patients. RESULTS: One hundred one patients (25%) had a history of venous thromboembolism with a median age at diagnosis of 29.9 years. A history of non-catheter-related venous thromboembolism was found in 18.8% of patients. Sickle variant genotypes conferred a higher risk of non-catheter-related venous thromboembolism compared with sickle cell anemia genotypes (SS/Sβ(0)) (relative risk [RR] 1.77; 95% confidence interval [CI], 1.18-2.66). Tricuspid regurgitant jet velocity ≥2.5 m/s also was associated with non-catheter-related venous thromboembolism (RR 1.65; 95% CI, 1.12-2.45). Thirty patients (7.4%) died during the study period. Adjusting for all variables, non-catheter-related venous thromboembolism was independently correlated with death (RR 3.63; 95% CI, 1.66-7.92). CONCLUSION: Venous thromboembolism is common in adults with sickle cell disease. Sickle variant genotypes and tricuspid regurgitant jet velocity ≥2.5 m/s are associated with non-catheter-related venous thromboembolism. In addition, non-catheter-related venous thromboembolism appears to be an independent risk factor for death in our cohort. These results suggest that disease-specific prophylaxis and treatment strategies for venous thromboembolism should be investigated in sickle cell disease patients.
BACKGROUND: Sickle cell disease is recognized as a hypercoagulable state; however, the frequency and characteristics of venous thromboembolism in sickle cell patients have not been well defined. The purpose of this study was to establish the prevalence and risk factors for venous thromboembolism in a large cohort of patients with sickle cell disease and determine the relationship between venous thromboembolism and mortality. METHODS: We performed a cross-sectional study of 404 sickle cell disease patients cared for at the Sickle Cell Center for Adults at Johns Hopkins. Demographic, sickle cell disease-specific comorbidity, and venous thromboembolism data were collected on all patients. RESULTS: One hundred one patients (25%) had a history of venous thromboembolism with a median age at diagnosis of 29.9 years. A history of non-catheter-related venous thromboembolism was found in 18.8% of patients. Sickle variant genotypes conferred a higher risk of non-catheter-related venous thromboembolism compared with sickle cell anemia genotypes (SS/Sβ(0)) (relative risk [RR] 1.77; 95% confidence interval [CI], 1.18-2.66). Tricuspid regurgitant jet velocity ≥2.5 m/s also was associated with non-catheter-related venous thromboembolism (RR 1.65; 95% CI, 1.12-2.45). Thirty patients (7.4%) died during the study period. Adjusting for all variables, non-catheter-related venous thromboembolism was independently correlated with death (RR 3.63; 95% CI, 1.66-7.92). CONCLUSION:Venous thromboembolism is common in adults with sickle cell disease. Sickle variant genotypes and tricuspid regurgitant jet velocity ≥2.5 m/s are associated with non-catheter-related venous thromboembolism. In addition, non-catheter-related venous thromboembolism appears to be an independent risk factor for death in our cohort. These results suggest that disease-specific prophylaxis and treatment strategies for venous thromboembolism should be investigated in sickle cell disease patients.
Authors: Samir K Ballas; Susan Lieff; Lennette J Benjamin; Carlton D Dampier; Matthew M Heeney; Carolyn Hoppe; Cage S Johnson; Zora R Rogers; Kim Smith-Whitley; Winfred C Wang; Marilyn J Telen Journal: Am J Hematol Date: 2010-01 Impact factor: 10.047
Authors: Kenneth I Ataga; Charity G Moore; Cheryl A Hillery; Susan Jones; Herbert C Whinna; Dell Strayhorn; Cathy Sohier; Alan Hinderliter; Leslie V Parise; Eugene P Orringer Journal: Haematologica Date: 2008-01 Impact factor: 9.941
Authors: Willem M Lijfering; Jan-Leendert P Brouwer; Nic J G M Veeger; Ivan Bank; Michiel Coppens; Saskia Middeldorp; Karly Hamulyák; Martin H Prins; Harry R Büller; Jan van der Meer Journal: Blood Date: 2009-01-12 Impact factor: 22.113
Authors: Eduard J van Beers; Berthe L F van Eck-Smit; Melvin R Mac Gillavry; Charlotte F J van Tuijn; Joost W J van Esser; Dees P M Brandjes; Mies C Kappers-Klunne; Ashley J Duits; Bart J Biemond; John-John B Schnog Journal: Chest Date: 2008-01-15 Impact factor: 9.410
Authors: A R Folsom; W Tang; N S Roetker; A V Kshirsagar; V K Derebail; P L Lutsey; R Naik; J S Pankow; M L Grove; S Basu; N S Key; M Cushman Journal: J Thromb Haemost Date: 2014-12-11 Impact factor: 5.824
Authors: Mehdi Nouraie; Xu Zhang; Andrew Srisuwananukorn; Roberto F Machado; Victor R Gordeuk; Mark T Gladwin; Santosh Saraf Journal: Ann Am Thorac Soc Date: 2020-07
Authors: Megan Z Roberts; G Eric Gaskill; Julie Kanter-Washko; T Rogers Kyle; Brittany C Jones; Nicole M Bohm Journal: J Thromb Thrombolysis Date: 2018-05 Impact factor: 2.300