How is venous thromboembolism in protein C deficiency treated?

Updated: Jan 04, 2019
  • Author: Shamudheen Rafiyath, MD; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

VTE in patients with protein C deficiency is managed in much the same way as it is for patients with VTE due to other causes. Because the risk of recurrent VTE in protein C–deficient patients may be as high as 60%, [22] long-term anticoagulation is often recommended, particularly following a spontaneous thromboembolic event.

In protein C deficiency, caution should be taken to reduce the risk of warfarin-induced skin necrosis while choosing the anticoagulant agent. Preventive measures include the use of an oral anticoagulant other than warfarin, use of warfarin with a lower starting dose, and longer duration of overlapping heparin or low molecular weight heparin (LMWH) administration.

Because of the availability of and familiarity with the use of direct oral anticoagulants (DOACs), the preference is to use DOACs for anticoagulation in patients with a typical VTE presentation. However, in massive pulmonary embolism and other severe clinical presentations such as hypoxemia/shock, or deep vein thrombosis with a proximal clot burden and in patients with concerns about adherence, the preference would be to administer heparin or an LMWH and then transition to warfarin, with the goal of keeping the international normalized ratio (INR) in the high end of the therapeutic range.


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