How is NPF in protein C deficiency treated?

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

Like WISN, NPF is a medical emergency that requires rapid normalization of plasma protein C activity. Although fresh frozen plasma has been used as a source of exogenous protein C in the treatment of NPF, frequent dosing is required to maintain adequate plasma levels, thereby limiting its usefulness in this setting. Highly purified protein C concentrate represents an attractive alternative that does not subject patients to the high volume and protein load of fresh frozen plasma. [72, 73, 74]

After treatment of the acute phase of NPF, patients are transitioned to anticoagulation therapy, which they must remain on indefinitely. Warfarin may be used in this setting, provided that exogenous protein C is administered during its initiation in order to avoid the development of WISN. [75] For patients with breakthrough thrombosis despite anticoagulation, protein C concentrate may be infused at home. A subcutaneous formulation of protein C requiring administration every 3 days has been used successfully in this context. [76]

Living donor liver transplantations have been successfully performed in NPF, resulting in a permanent cure. [77]


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