How is protein S deficiency treated?

Updated: Jan 03, 2021
  • Author: Mohammad Muhsin Chisti, MD, FACP; Chief Editor: Perumal Thiagarajan, MD  more...
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Management of protein S deficiency takes place in the event of acute venous thromboembolism (VTE). Prophylaxis may be used in selected patients with asymptomatic carrier states without a thrombotic event.

Following an acute thrombosis, inital management is the same as for all acute VTE episodes, based on the severity of disease, co-morbidities, and hemodynamic stability. Main agents in the acute period include intravenous unfractionated heparin, low molecular weight heparin (LMWH), or a direct oral anticoagulant (DOAC).

The choice between a DOAC and a vitamin K antagonist (VKA) depends on factors such as patient preference, cost, and convenience. Historically, VKAs were the mainstay of treatment for VTE, including those caused by inherited thrombophilias. With the advent of DOACs, with their comparable efficacy as well as their safety profile, they are now increasingly used for VTE, including in patients with hereditary thrombophilias. 

In a prospective cohort study of patients with acute VTE diagnosed with inherited thrombophilias, DOACs had the same efficacy as heparin/VKAs and were shown to significantly reduce the 2-year VTE recurrence after anticoagulant discontinuation. DOACs did show an increased risk of clinically relevant non-major bleeding, while VKAs showed a slight increase in major bleeding. [35] A systematic review and meta-analysis conducted by Elsebaie et al also reported a low VTE recurrence and comparable rates of bleeding events between DOAC and VKA. [36]  These studies support the use of DOACs for acute VTE in the setting of inherited thrombophilias, including protein S deficiency.  

The question of whether to continue lifelong anticoagulation in patients with diagnosed protein S deficiency after their first thrombotic event is controversial. If the first thrombotic event was life threatening or occurred in multiple or unusual sites (eg, cerebral veins, mesenteric veins), most experts recommend lifelong therapy initially. If precipitated by a strong event (eg, trauma, surgery) and the thrombosis was not life threatening or involved multiple or unusual sites, some experts argue that these patients may have a lower risk of recurrence and deserve a trial without anticoagulation after 9 months.

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