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Recurrent Venous Thromboembolism Is Common in Adults with Sickle Cell Disease
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A3762 - Recurrent Venous Thromboembolism Is Common in Adults with Sickle Cell Disease
Author Block: R. Strykowski1, B. Scarpato1, J. Patel1, R. T. Cohen1, S. Nouraie2, E. S. Klings1; 1Pulmonary, Boston University Medical Center, Boston, MA, United States, 2Medicine, PACCM, University of Pittsburgh, Pittsburgh, PA, United States.
Objective: Sickle cell disease (SCD) is a hypercoagulable state, yet the natural history of venous thromboembolism (VTE) is unknown. This leads to uncertainty concerning the optimal length of treatment for SCD patients with their first VTE. We hypothesized that recurrent VTEs are common in SCD and thus treatment strategies for first time VTE in the general population may be inadequate for these patients.
Methods: We performed a retrospective longitudinal chart review of all patients with SCD currently 18 years and older followed at Boston Medical Center between 2003-2017. We collected demographic data, SCD genotype, medical history, laboratory values, and echocardiography data, and ascertained if a VTE occurred. VTE was defined as a deep venous thrombosis (DVT), pulmonary embolism (PE) or both. For all patients with a VTE, we determined if the event was associated with thrombosis risk factors (“provoked”) or not (“unprovoked”). We recorded the anticoagulant prescribed and duration of therapy. The frequency of recurrent VTE was calculated and clinical data at each event were obtained. All data were analyzed using STATA software version 14.0.
Results: 233 individuals with SCD were included (69% with HbSS/HbSβ0 and 30.5% with HbSC/HbSβ+); 53% were female. 55/233 (23.6%) had a history of VTE, this was similar across Hb genotypes (p=0.70). 36% of VTE episodes were provoked. In 89%, anti-coagulation was prescribed. 8.2% received oral Factor Xa or thrombin inhibitors while heparin (unfractionated and/or low molecular weight) and/or warfarin was used in the others. 40.8% were treated for 6 months or less, 10.2% were treated 6 months - 1 year and 20.4% received treatment for > 1 year. 40% (22/55) of patients had a VTE recurrence. Of those with recurrence, both DVT and PE occurred in 55%, 27%, had a PE and 18% had a DVT. 54% (12/22) of the patients had 3 or more VTE events. 20% of those with a provoked VTE had recurrence, compared to a 36% recurrence risk among those whose first event was unprovoked.
Conclusions: Patients with SCD diagnosed with a VTE have a high risk of recurrence even in the absence of established thrombosis risk factors, suggesting an ongoing thrombotic risk attributable to their hemoglobinopathy. Our findings suggest that the current practice of 3-6 months of anti-coagulation for a first time VTE, based on the recommendations for the general population, is inadequate in SCD and stresses the need for prospective studies in this area.
Author Block: R. Strykowski1, B. Scarpato1, J. Patel1, R. T. Cohen1, S. Nouraie2, E. S. Klings1; 1Pulmonary, Boston University Medical Center, Boston, MA, United States, 2Medicine, PACCM, University of Pittsburgh, Pittsburgh, PA, United States.
Objective: Sickle cell disease (SCD) is a hypercoagulable state, yet the natural history of venous thromboembolism (VTE) is unknown. This leads to uncertainty concerning the optimal length of treatment for SCD patients with their first VTE. We hypothesized that recurrent VTEs are common in SCD and thus treatment strategies for first time VTE in the general population may be inadequate for these patients.
Methods: We performed a retrospective longitudinal chart review of all patients with SCD currently 18 years and older followed at Boston Medical Center between 2003-2017. We collected demographic data, SCD genotype, medical history, laboratory values, and echocardiography data, and ascertained if a VTE occurred. VTE was defined as a deep venous thrombosis (DVT), pulmonary embolism (PE) or both. For all patients with a VTE, we determined if the event was associated with thrombosis risk factors (“provoked”) or not (“unprovoked”). We recorded the anticoagulant prescribed and duration of therapy. The frequency of recurrent VTE was calculated and clinical data at each event were obtained. All data were analyzed using STATA software version 14.0.
Results: 233 individuals with SCD were included (69% with HbSS/HbSβ0 and 30.5% with HbSC/HbSβ+); 53% were female. 55/233 (23.6%) had a history of VTE, this was similar across Hb genotypes (p=0.70). 36% of VTE episodes were provoked. In 89%, anti-coagulation was prescribed. 8.2% received oral Factor Xa or thrombin inhibitors while heparin (unfractionated and/or low molecular weight) and/or warfarin was used in the others. 40.8% were treated for 6 months or less, 10.2% were treated 6 months - 1 year and 20.4% received treatment for > 1 year. 40% (22/55) of patients had a VTE recurrence. Of those with recurrence, both DVT and PE occurred in 55%, 27%, had a PE and 18% had a DVT. 54% (12/22) of the patients had 3 or more VTE events. 20% of those with a provoked VTE had recurrence, compared to a 36% recurrence risk among those whose first event was unprovoked.
Conclusions: Patients with SCD diagnosed with a VTE have a high risk of recurrence even in the absence of established thrombosis risk factors, suggesting an ongoing thrombotic risk attributable to their hemoglobinopathy. Our findings suggest that the current practice of 3-6 months of anti-coagulation for a first time VTE, based on the recommendations for the general population, is inadequate in SCD and stresses the need for prospective studies in this area.
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