Portal vein thrombosis in a patient with severe hemophilia B: A challenging balanced management.

Key Clinical Message: The increased life expectancy in patients with hemophilia (PwH) over the last years has raised the incidence of comorbidities, including thromboembolic events. Thromboembolic events are rare in PwH and most of them occur in the presence of exogenous risk factors. There is still scarce scientific evidence on the optimal antithrombotic treatment and management approach in this population. Abstract: In the hemophilic population thromboembolic events are rare. Most of them are often multifactorial and occur in the presence of both exogenous (orthopedic surgery, intensive replacement therapy, use of central venous catheters…) and endogenous (cardiovascular diseases) risk factors. We describe the case of a 43-year-old patient with severe hemophilia B (sHB) receiving prophylaxis with eftrenonacog alfa (rFIXFc) and antithrombotic treatment due to portal vein thrombosis. The patient was treated with extended half- life factor IX (EHL-FIX) prophylaxis maintaining higher trough levels to avoid new bleeding episodes associated to the underlying disease and the use of antithrombotic therapy with low molecular weight heparin. EHL-FIX concentrates allow prolonged intervals between intravenous infusions and higher hemostatic protection thanks to increased factor trough levels. This current case report provides clinical evidence in antithrombotic management in a patient with severe hemophilia B.

Mortality and bleeding complications of COVID-19 critically ill patients with venous thromboembolism.

BACKGROUND: Venous thromboembolism (VTE) disease in COVID-19 patients is a remarkable issue, especially its relationship with bleeding events and mortality. The objective of this study was to describe the outcomes of critically ill patients with COVID-19, hospitalized in the Intensive Care Unit (ICU), in relationship with VTE during their stay. METHODS: This is a prospective cohort study of critically ill COVID-19 patients in two hospitals that underwent a venous ultrasound at the beginning of follow-up of both lower limbs in April 2020. In case of clinical suspicion of new VTE during the 30-day follow-up, additional ultrasound or thoracic CT were performed. Global VTE frequency, major bleeding events and survival were collected, and their predictors were studied. RESULTS: We included 230 patients. After 30 days of follow-up, there were 95 VTE events in 86 patients (37.4%). Thirteen patients (5.7%) developed major bleeding complications and 42 patients (18.3%) died. None of the comorbidities or previous treatments were related with bleeding events. D-Dimer at admission was significantly related with VTE development and mortality. Independent predictors of mortality in the regression model were older age (>66 years), D-Dimer at admission (>1.500 ng/mL) and low lymphocyte count (<0.45×109/L) with an AUC in the ROC curve of 0.81 (95% CI: 0.73-0.89). Patients presenting these three conditions presented a mortality of 100% in the predictive model. CONCLUSIONS: VTE frequency in ICU COVID-19 patients is high and risk of major bleeding is low. Comorbidities and laboratory parameters of admission in these patients can be a useful tool to predict mortality.