Increased incidence of VTE in sickle cell disease patients: risk factors, recurrence and impact on mortality

Abstract

Previous reports show increased incidence of venous thromboembolism [VTE, deep-vein thrombosis (DVT) and pulmonary embolus (PE)] in sickle cell disease (SCD) patients but did not account for frequency of hospitalization. We determined the incidence of VTE in a SCD cohort versus matched controls. For SCD patients, risk factors for incident VTE, recurrence and the impact on mortality were also determined. Among 6237 patients with SCD, 696 patients (11·2%) developed incident-VTE: 358 (51·6%) had PE (±DVT); 179 (25·7%) had lower-extremity DVT only and 158 (22·7%) had upper-extremity DVT. By 40 years of age, the cumulative incidence of VTE was 17·1% for severe SCD patients (hospitalized ≥3 times a year) versus 8·0% for the matched asthma controls. Amongst SCD patients, women (Hazard ratio [HR] = 1·22; 95% confidence interval [CI]: 1·05-1·43) and those with severe disease (HR = 2·86; 95% CI: 2·42-3·37) had an increased risk of VTE. Five-year recurrence was 36·8% in patients with severe SCD. VTE was associated with increased risk of death (HR = 2·88, 95% CI: 2·35-3·52). In this population-based study, the incidence of VTE was higher in SCD patients than matched controls and was associated with increased mortality. The high incidence of recurrent VTE in patients with severe SCD suggests that extended anticoagulation may be indicated.

Osteonecrosis of the femoral head in sickle cell disease: prevalence, comorbidities, and surgical outcomes in California

“…Despite recently reported increased VTE incidence in our SCD cohort, 31 there were no admissions for VTE within 30 days of discharge from hip replacement surgery. This is in contrast to 0.2% of readmissions for pulmonary embolism in older non-SCD adults undergoing hip replacement surgery in the United States and 0.3% rates of readmission for pulmonary embolism in older non-SCD patients in Canada.…”

“…31 We searched for specific ICD-9-CM codes for SCD found in any of the 25 diagnosis fields (supplemental Table 1). To be included in this analysis, subjects were required to have a high probability of SCD, which we defined as (1) $2 separate admissions with an SCD code in the principal (first position) diagnosis, or (2) $1 admission with an SCD code in the principal position and an SCD code in a secondary position on $2 additional admissions.…”

Prevalence and risk factors for venous thromboembolism in children with sickle cell disease: an administrative database study

“…Ann Brunson and colleagues retrospectively evaluated 6237 patients with SCD using the Patient Discharge Database for the State of California. 32 By 40 years of age, the cumulative incidence of VTE was 12.5% (95% CI, 11.5-13.6). Additionally, VTE was associated with a 2.9-fold increased HR of death.…”

Cancer specific survival in patients with sickle cell disease

“…As previously described, the SCD cohort was identified using longitudinal records from the California Patient Discharge Data (PDD) and the Emergency Department Utilization (EDU) databases from the Office of Statewide Health Planning and Development (OSHPD) (Adesina et al , ; Brunson et al , ,b). Since July 1990, the State of California has required that non‐Federal hospitals report up to 25 diagnoses and up to 21 procedures associated with each hospitalisation, coded using the International Classification of Disease, Ninth Revision, Clinical Modification (ICD‐9‐CM) up to 2014.…”

“…Sickle cell disease (SCD), the most common inherited blood disorder in the United States, is associated with a number of serious complications, including vaso‐occlusive crisis, thrombosis, stroke, acute chest syndrome and osteonecrosis of the femoral head (Platt et al , ; Powars et al , ; Adesina et al , ; Brunson et al , 2017a). We and others have found an increased incidence of certain cancers among SCD patients compared to general or hospitalized populations (Seminog et al , ; Brunson et al , 2017b).…”