Introduction Budd Chiari Syndrome (BCS) is associated with an underlying pro-coagulant haematological disorder in up to 87% of cases. Following liver transplantation (LT), anticoagulation with heparin is commonplace in order to prevent thrombotic complications. Heparin induced thrombocytopaenia (HIT) is a rare but life threatening complication of heparin therapy, where the thrombotic risk is 30 times that of control populations. HIT associated thrombosis has a reported incidence of between 0.5 and 3% in patients on un-fractionated heparin. We reviewed all our patients with BCS who underwent LT to assess the incidence and clinical significance of HIT.
Results In total, 36 patients underwent LT for BCS between 1995 and 2008. An underlying pro-coagulant disorder was identified in 22 patients (myeloproliferative disorder (MPD) n=17, Protein C Deficiency n=2, Behcet's n=2 and lupus anti-coagulant n=1). One third of patients were thrombocytopenic (platelets <150 cells/μl) prior to LT. All patients had received un-fractionated heparin prior to and following LT. Thrombocytopaenia occurred in 85% of patients within the first 10 post operative days (median 49 cells/μl, range 7–292 cells/μl). A diagnosis of HIT was made when patients had a platelet count of <150 cells/μl and circulating PF4-heparin antibodies were identified. The overall incidence of HIT was 22% (8/36 patients), however in patients with a MPD the incidence was 41% (7/17). Furthermore a MPD was present in 7/8 (88%) patients who developed HIT vs 10/28 (36%) patients who did not develop HIT (p=0.016). An acute post operative thrombotic complication occurred in 50% (4/8) of patients who developed HIT compared to 4% (1/28) of patients that didn't (p=0.005). Bleeding complications occurred in 38% (3/8) of patients that developed HIT compared to 14% (4/28) who didn't (p=0.16). In hospital mortality was 38% in those patients that developed HIT compared to 7% in those who did not develop HIT (p=0.06). The fall in platelet count was not statistically different between those patients who did and did not develop HIT. In a cohort of patients (n=30) transplanted for hepatic artery thrombosis, who had also received un-fractionated heparin pre and post LT, HIT occurred in 1/30 (3%).
Conclusion We have demonstrated that HIT is a common complication of patients treated with un-fractionated heparin following LT for BCS. Moreover, the presence of an underlying MPD is a strong predisposing factor for its development. In patients that develop HIT the thrombosis rate, bleeding complication rate and pre-hospital discharge mortality are all increased. The diagnosis requires a high index of suspicion due to the frequency of thrombocytopaenia following LT. Due to the exceptionally high incidence of HIT in patients with MPD, these patients may benefit from standardised treatment with lepirudin following LT.
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