title: Increased prevalence of heparin-induced thrombocytopenia in patients with Budd-Chiari syndrome: a retrospective analysis.
abstract: BACKGROUND
  Budd-Chiari syndrome (BCS) is a rare, potentially fatal disease characterized by hepatic venous outflow tract obstruction. Heparin-induced thrombocytopenia (HIT) is an immune-mediated complication of heparin therapy, with mortality approaching 10%. The reported prevalence of HIT in adults is 0.2-5.2%. Expert consensus through case reports is the only existing evidence of HIT in BCS. To our knowledge, this is the first study to formalize this anecdotal evidence.
  
  
  METHODS
  A retrospective analysis was carried out of patients presenting at a tertiary liver centre with acute liver failure because of BCS or BCS as the primary indication for liver transplantation between 2000 and 2013. The prevalence of HIT in the study group was compared with the highest reported prevalence in adult medical patients receiving heparin (5.2%). Mortality, length of stay and liver transplantation rates were also studied.
  
  
  RESULTS
  Of 32 BCS patients, 9 (28.1%) developed HIT, significantly higher than the previously reported prevalence of HIT in medical patients (5.2%) (P<0.0001). There was no difference in mortality (P=0.66), length of stay (P=0.58) and liver transplantation rate (P=0.39) between HIT-positive and HIT-negative patients.
  
  
  CONCLUSIONS
  The prevalence of HIT (28.1%) in our cohort of BCS patients is significantly higher than that in the general population (0.2-5.2%). Although this study was not powered to detect outcome differences, as heparin is the mainstay of acute BCS treatment, this represents a significant risk. We recommend a high index of suspicion for HIT in patients with BCS and thrombocytopenia, an appropriate HIT-testing strategy and consideration of direct thrombin inhibitors.
authors.affiliation: Institute of Liver Studies, King's College Hospital, Denmark Hill, London, UK.
authors.forename: Sameer
authors.initials: S
authors.lastname: Zaman
authors.forename: Stefan
authors.initials: S
authors.lastname: Wiebe
authors.forename: William
authors.initials: W
authors.lastname: Bernal
authors.forename: Julia
authors.initials: J
authors.lastname: Wendon
authors.forename: Julia
authors.initials: J
authors.lastname: Czuprynska
authors.forename: Georg
authors.initials: G
authors.lastname: Auzinger
mesh_terms: D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000009:adverse effects*; D006502:Budd-Chiari Syndrome / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality; D005260:Female; D006493:Heparin / Q000009:adverse effects*; D006801:Humans; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D016031:Liver Transplantation; D008131:London / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D062606:Tertiary Care Centers; D013921:Thrombocytopenia / Q000097:blood / Q000139:chemically induced* / Q000175:diagnosis / Q000401:mortality; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D006493:Heparin