Background: Venous thromboembolism (VTE) risk is well recognised in adults with Sickle cell disease (SCD). This risk is poorly understood in children and adolescents with SCD, although it is acknowledged that central venous access devices (CVAD) are a major risk factor. VTE risk increases as children reach adolescence and young adulthood, as does the risk of haemorrhagic stroke. Many patients have CVAD to facilitate exchange transfusion for cerebral vasculopathy: a risk factor for clot and haemorrhagic stroke.Aim: We reviewed our cohort of SCD patients with Port-a-Cath’s (Port) to define the risk of VTE.Methods: Records for all patients with SCD (0-23 years) followed at Children’s Health Ireland over a 15-year period (01/01/2010-01/01/2015) were reviewed. (table 1)We obtained data on: SCD genotype, SCD treatment, Port insertions, hospital admissions; VTE diagnosis and treatment, Age, Gender, Body mass index (BMI) and other VTE risk factors (eg. sex, age at time of VTE insertion, sickle cell genotype, double/single lumen Port-a-cath, type of blood transfusion programme, mean age of Port-a-cath, hospital admission rates, hydroxyurea therapy and others).Results: There were 550 SCD patients; 180 on blood transfusion programmes. 29 patients had Ports; 6 developed VTE (20.6%).All VTE patients were over 15 years old (15.89-20.94) and had double-lumen ports for an exchange transfusion programme for cerebral vascular abnormalities. 83% (5/6) of affected patients were male with an average haematocrit (HCT) of 32%, vs. 30.5% (7/23) males, respectively, HCT of 27% in the non-affected cohort. 5/6 (83%) had an acute infection within 3 months of VTE diagnosis.All patients received a 8-12 week course of anti-coagulant. At median follow-up (F/U) of 34 months (range 11.5-52) there has been no VTE recurrence.Conclusion: The current literature for VTE in Paediatric SCD is limited. Our data demonstrates that VTE was related to acute infection and there has been no recurrence off anti-coagulation therapy, suggesting a role for cytokines, neutrophils and upregulation of endothelial adhesion molecules in thrombus formation. (table 2)We suggest use of 6 weeks of prophylactic anticoagulation following acute infections in patients over 14 years of age.
