Anestéziológia a intenzívna medicína 1/2022

Parturient with factor XII deficiency – a case report

Examination of coagulation state is a key factor for safe providing of neuraxial blockades. Parturients may present with coagulopathies specific for pregnancy or disorders occuring independently of pregnancy, where we count also deficiency of factor XII. Severe deficiency of this factor is a rare, inherited, mostly occasionally found disorder. Isolated markedly prolonged activated partial tromboplastine time is typical for this abnormality. In differential diagnosis intrinsic pathway factor deficiencies, which cause severe bleeding (hemophilias A, B, C, von Willebrand disease) need to be excluded. Factor XII deficiency is not associated with bleeding complication and these patients do not need any preprocedural hematological substitution. Factor XII plays a role in fibrinolysis as weak plasminogen activator, what suggests a higher occurance of trombotic events. Without precise medical history and correct laboratory diagnostic methods, there is a risk of unnecessary testing and worries of invasive procedures. We present a case report of a parturient with severe inherited factor XII deficiency, which was diagnosed after occasionally found prolonged activated partial tromboplastine time and subsequent complex hematological examination. We performed a safe uneventful epidural analgesia for spontaneous labour.

Keywords: factor XII, activated partial tromboplastine time, fibrinolysis, mixing study, trombosis, epidural analgesia