Splanchnic vein thrombosis: Clinical manifestations, risk factors, management, and outcomes.

BACKGROUND: Clinical manifestations and optimal management strategies in patients with splanchnic vein thrombosis (SVT) are not well characterized. METHODS: We conducted a retrospective cohort study including all newly diagnosed SVT evaluated between January 2007 and December 2018. Efficacy outcome was thrombosis resolution, and safety outcomes included death and occurrence of bleeding. RESULTS: We included 155 patients with a mean age of 56.2 (18-87). Local risk factors were present in 118 (76.1%) patients and 30 (19.4%) had only systemic/thrombophilia. Local risk factors included abdominal cancers (31%), surgery (20.6%) and liver cirrhosis (19.4%). Thrombophilia screening was conducted in approximately 50% of patients. Factor V Leiden or Prothrombin G20210A mutations were observed in 7.1% of patients whereas 14.4% were JAK2V617F mutation positive. Most common manifestations at onset were abdominal pain (56.1%), whereas 44.6% were incidentally found. Portal vein thrombosis was observed more in primary cases (91.9% vs. 69.5%, p = 0.012). Anticoagulation was used in 93.5% cases. Indefinite anticoagulation was used more frequently in primary SVT (62.2% vs. 41.5%, p = 0.045). Thrombosis resolution and bleeding complications among primary (without local risk factors) and secondary (with local risk factors) SVT were observed in 48.5%, 65%, 8.1%, and 11.9%, respectively with no difference when comparing patients treated with direct oral anticoagulants or warfarin and/or low molecular weight heparin (58% vs. 62%, p = 0.326, 9% vs. 12%, p = 0.518). CONCLUSIONS: In this cohort anticoagulation resulted in partial or complete thrombosis resolution in a significant proportion of patients with an acceptable bleeding risk regardless local risk factors or type of anticoagulant.

Desmopressin responsiveness at a capped dose of 15  µg in type 1 von Willebrand disease and mild hemophilia A.

Desmopressin (DDAVP) is commonly used in the treatment of patients with type 1 von Willebrand disease (VWD) and mild hemophilia A. A patient's responsiveness to DDAVP based on a 0.3  µg/kg dose determines future therapeutic efficacy of the drug. The aim of the study was to determine whether a capped dose of 15 µg subcutaneous DDAVP is able to achieve the same level of DDAVP responsiveness as previously reported. This is a retrospective chart review of patients from 1995 to 2013 in adults and children with type 1 VWD and hemophilia A weighing more than 50 kg. Levels of factor VIII, ristocetin cofactor, and von Willebrand factor antigen were measured before and after 1 h of administration of 15 µg of DDAVP. In patients with type 1 VWD, the complete response rate was 82.5% with a partial response rate of 12.5% and 5% nonresponders. In patients with mild hemophilia A, the complete response rate was 53.8% with a partial response rate of 38.5% and 7.7% nonresponders. These results using a capped 15-µg dose of DDAVP are similar to previously published reports using the 0.3-µg/kg dose.