Abstract

Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (CVADs) are commonly used for chronic transfusions and iron chelation in this population. CVADs are an additional venous thromboembolism (VTE) risk factor. The role of thromboprophylaxis in this setting is uncertain. The objectives are: (1) to determine whether thromboprophylaxis reduces VTE risk in SCD patients with CVAD and (2) to explore characteristics associated with VTE risk. We identified adults with SCD and CVAD intended for chronic use (≥3 months) at two comprehensive SCD centers. Thromboprophylaxis presence; type; intensity; and patient-, catheter-, and treatment-related VTE risk factors were recorded. Among 949 patients, 49 had a CVAD (25 without and 24 with VTE prophylaxis). Thromboprophylaxis type and intensity varied widely. Patients without thromboprophylaxis had higher VTE rates (rate ratio (RR) = 4.0 (95% confidence interval: 1.2–12.6), p = 0.02). Hydroxyurea was associated with lower VTE rates (RR = 20.5 (6.4–65.3), p < 0.001). PICC lines and Vortex and Xcela Power implantable devices were associated with higher rates compared with Port-a-Cath (RR = 5.8 (1.3–25.9), p = 0.02, and RR = 58.2 (15.0–225.0), p < 0.001, respectively). Thromboprophylaxis, hydroxyurea, and CVAD subtype were independently associated with VTE. The potentially protective role of thromboprophylaxis and hydroxyurea for VTE prevention in patients with SCD and CVAD merits further exploration.

Highlights

  • Sickle cell disease (SCD) is a high-risk inherited thrombophilia [1]

  • The objectives are: (1) to determine whether thromboprophylaxis reduces venous thromboembolism (VTE) risk in SCD patients with Central venous access devices (CVADs) and (2) to explore characteristics associated with VTE risk

  • We identified adults with SCD and CVAD intended for chronic use (≥3 months) at two comprehensive SCD centers

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Summary

Introduction

Sickle cell disease (SCD) is a high-risk inherited thrombophilia [1]. The cumulative incidence of venous thromboembolism (VTE) is reported to be 11% by age 40 with high rates of recurrence (>24% at 5 years) [5–7]. SCD patients often require central venous access devices (CVADs) for chronic transfusions and/or iron chelation. CVADs are known to be highly prothrombotic with a catheter-related thrombotic rate of 24% in SCD patients [8]. While it is well established that those with SCD and CVAD are at high risk for VTE, the role of pharmacological thromboprophylaxis for the prevention of VTE in this setting is uncertain due to a paucity of data. We hypothesized that thromboprophylaxis would reduce the VTE rate in adult SCD patients with CVAD. Our secondary objective was to explore additional potential VTE risk factors in this setting

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