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Observational Study
. 2014 Dec;12(12):2010-6.
doi: 10.1111/jth.12744. Epub 2014 Oct 18.

Venous thromboembolism incidence in the Cooperative Study of Sickle Cell Disease

R P Naik  1 , M B Streiff, C Haywood Jr, J B Segal, S Lanzkron
Affiliations
Observational Study

Venous thromboembolism incidence in the Cooperative Study of Sickle Cell Disease

R P Naik et al. J Thromb Haemost. 2014 Dec.

Abstract

Background: Venous thromboembolism (VTE) has been recently recognized as a complication of sickle cell disease (SCD); however, the incidence of VTE in SCD is unknown.

Objectives: The primary objective of this study was to determine the incidence of first VTE, including pulmonary embolism (PE) and deep vein thrombosis (DVT), among SCD patients age ≥ 15 years. We also evaluated genotypic differences in VTE risk and determined the relationship between VTE and mortality.

Patients/methods: In this retrospective cohort study, we used data from the Cooperative Study of Sickle Cell Disease (CSSCD) to calculate incidence rates for first VTE. We used Cox proportional hazard models to estimate hazard ratios (HRs) for time to VTE by genotype and time to death by VTE status.

Results: We included 1523 SCD patients aged ≥ 15 years with 8862 years of follow-up in this analysis. The incidence rate for first VTE was 5.2 events/1000 person-years (95% confidence interval [CI] 3.8-6.9) with a cumulative incidence of 11.3% (95% CI 8.3-15.3) by age 40 years. Individuals with the SS/Sβ(0) -thalassemia genotype had the highest rate of VTE (7.6 events/1000 person-years [95% CI 5.3-10.6]). The incidence of PE exceeded that of isolated DVT (3.6 [95% CI 2.5-5.1] events/1000 person-years vs. 1.6 [95% CI 0.9-2.7] events/1000 person-years), although this difference was not statistically significant. SCD patients with VTE had a higher mortality rate (adjusted HR 2.32 [95% CI 1.20-4.46]) than those without VTE.

Conclusions: Patients with SCD are at substantial risk for VTE, and individuals with VTE are at higher risk of death than those without VTE.

Keywords: mortality; pulmonary embolism; sickle cell disease; venous thromboembolism; venous thrombosis.

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Conflict of interest statement

Conflicts of Interest

B. Streiff reports personal fees from Boehringer-Inglheim, Daiichi-Sankyo, Janssen Healthcare, Sanofi-Aventis and Pfizer; and grants from Portola, outside the submitted work.

Figures

Figure 1
Figure 1
Cumulative Event Functions for First Venous Thromboembolism (VTE) in SCD patients, by Genotype
Figure 2
Figure 2
Cumulative Event Functions for Pulmonary Embolism (PE) and Isolated Deep Venous Thrombosis (DVT) in SCD patients
Figure 2
Figure 2
Cumulative Event Functions for Pulmonary Embolism (PE) and Isolated Deep Venous Thrombosis (DVT) in SCD patients
Figure 3
Figure 3
Overall Survival Curves in SCD patients, by Venous Thromboembolism Status
See this image and copyright information in PMC

References

    1. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med. 1997;337:762–769. - PubMed
    1. Tam DA. Protein C and protein S activity in sickle cell disease and stroke. J Child Neurol. 1997;12:19–21. - PubMed
    1. Ataga KI, Moore CG, Hillery CA, Jones S, Whinna HC, Strayhorn D, Sohier C, Hinderliter A, Parise LV, Orringer EP. Coagulation activation and inflammation in sickle cell disease-associated pulmonary hypertension. Haematologica. 2008;93:20–26. - PubMed
    1. Villagra J, Shiva S, Hunter LA, Machado RF, Gladwin MT, Kato GJ. Platelet activation in patients with sickle disease, hemolysis-associated pulmonary hypertension, and nitric oxide scavenging by cell-free hemoglobin. Blood. 2007;110:2166–2172. - PMC - PubMed
    1. Mekontso Dessap A, Deux JF, Abidi N, Lavenu-Bombled C, Melica G, Renaud B, Godeau B, Adnot S, Brochard L, Brun-Buisson C, Galacteros F, Rahmouni A, Habibi A, Maitre B. Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 2011;184:1022–1029. - PubMed

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