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

BACKGROUND: 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. CASE REPORT: 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. DISCUSSION: 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. CONCLUSION: 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.

Retinal vein occlusion: evaluation of "classic" and "emerging" risk factors and treatment.

Retinal vein occlusion (RVO) is the second most common retinal vein disease and an important cause of blindness and visual morbidity. Systemic risk factors are commonly associated with RVO, while unclear it is the role of the thrombophilic and coagulation disorders. To evaluate "classic" and "emerging" risk factors, and to establish a good treatment for RVO. Fifty patients, 31 males and 19 females, with RVO were selected for our study. RVO patients were divided into two groups: those with central retinal vein occlusion (CRVO) and those with branch retinal vein occlusion (BRVO). All patients were subjected to an anamnestic investigation and were tested for thrombophilia, coagulation disorders and hyperlipidemia. Treatment and prophylaxis were evaluated. We have named "classic" the systemic risk factors associated with RVO and "emerging" those risk factors, haemostasis related, not clearly associated with RVO. RVO occurs more commonly in patients aged over 50. "Emerging" risk factors were more frequent in CRVO, "classic" in BRVO. Hyperhomocysteinemia is the most common "emerging" risk factor related to RVO. 71.4% of tested patients had hypercholesterolemia. Treatment with LMWH would appear to be safe and effective, but the small number of patients considered not allow us a definitive evaluation of its efficacy. Although our study has shown the correlation between RVO and the "emerging" risk factors, more studies are necessary to better know the real role of thrombophilic and coagulation disorders in this disease and to determine a specific protocol for the treatment and prophylaxis of RVO.