title: Venous thromboembolism in adults with sickle cell disease: a serious and under-recognized complication.
abstract: BACKGROUND
  Sickle cell disease is recognized as a hypercoagulable state; however, the frequency and characteristics of venous thromboembolism in sickle cell patients have not been well defined. The purpose of this study was to establish the prevalence and risk factors for venous thromboembolism in a large cohort of patients with sickle cell disease and determine the relationship between venous thromboembolism and mortality.
  
  
  METHODS
  We performed a cross-sectional study of 404 sickle cell disease patients cared for at the Sickle Cell Center for Adults at Johns Hopkins. Demographic, sickle cell disease-specific comorbidity, and venous thromboembolism data were collected on all patients.
  
  
  RESULTS
  One hundred one patients (25%) had a history of venous thromboembolism with a median age at diagnosis of 29.9 years. A history of non-catheter-related venous thromboembolism was found in 18.8% of patients. Sickle variant genotypes conferred a higher risk of non-catheter-related venous thromboembolism compared with sickle cell anemia genotypes (SS/Sβ(0)) (relative risk [RR] 1.77; 95% confidence interval [CI], 1.18-2.66). Tricuspid regurgitant jet velocity ≥2.5 m/s also was associated with non-catheter-related venous thromboembolism (RR 1.65; 95% CI, 1.12-2.45). Thirty patients (7.4%) died during the study period. Adjusting for all variables, non-catheter-related venous thromboembolism was independently correlated with death (RR 3.63; 95% CI, 1.66-7.92).
  
  
  CONCLUSIONS
  Venous thromboembolism is common in adults with sickle cell disease. Sickle variant genotypes and tricuspid regurgitant jet velocity ≥2.5 m/s are associated with non-catheter-related venous thromboembolism. In addition, non-catheter-related venous thromboembolism appears to be an independent risk factor for death in our cohort. These results suggest that disease-specific prophylaxis and treatment strategies for venous thromboembolism should be investigated in sickle cell disease patients.
authors.affiliation: Department of Medicine, Division of Hematology, Johns Hopkins University, Baltimore, MD 21205, USA. rakhi@jhmi.edu
authors.forename: Rakhi P
authors.initials: RP
authors.lastname: Naik
authors.forename: Michael B
authors.initials: MB
authors.lastname: Streiff
authors.forename: Carlton
authors.initials: C
authors.lastname: Haywood
authors.forename: Julie A
authors.initials: JA
authors.lastname: Nelson
authors.forename: Sophie
authors.initials: S
authors.lastname: Lanzkron
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000401:mortality; D015897:Comorbidity; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D054556:Venous Thromboembolism / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality; D055815:Young Adult