Prothrombin complex concentrate such as therapy and prophylaxis in factor X-deficient patient (Friuli variant)

Factor X (FX) deficiency is a serious, rare bleeding disorder, with 1 in 500 000 affected people. Hemorrhages, hematuria, epistaxis, and other bleeding complications are frequent.

Now, we report a case of a well-known 77-year-old FX-deficient patient (Friuli variant, level <1%, mutation Pro(343)→Ser, exon VIII) with hypertension, chronic obstructive pulmonary disease (COPD), and chronic gastritis, admitted many times to hospital due to surgical complications after aortic abdominal aneurysm (AAA) repair. Use of prothrombin complex concentrate (PCC) such as hemostatic therapy during surgeries and prophylaxis after discharge is shown in this article. Three consecutive surgeries were considered. First, endoleak postendoprosthesis; second, AAA breakage; and third, planned surgery, a new endovascular prosthesis positioning and femur-femoral bypass. No adverse events due to PCC were found by local physicians.

We discuss the methods commonly used in the treatment and prophylaxis of patients with FX deficiency to reduce hemorrhagic risk and to improve their quality of life.

Waiting for specific therapeutic options for FX deficiency, currently, the best treatment is represented by PCC. Its correct use permits an improvement in life quality and a reduction in bleeding frequency in FX-deficient patients.