Surgical treatment of a patient with benign prostatic hyperplasia in a severe form of Hageman’s disease

Hageman’s disease is a rare genetic disorder of the hemostasis system characterized by a congenital deficiency of blood coagulation factor XII. The frequency of the disease in the general population is unknown, as it is asymptomatic under normal conditions, but it is considered to be about 1 case per 1 million population. The aim of the work is to present a clinical case of successful surgical treatment of a patient with prostate adenoma, with severe congenital deficiency of plasma coagulation factor XII. The patient underwent a postpartum adenomectomy for benign prostatic hyperplasia. Open access made it possible to successfully remove prostate adenoma despite the presence of severe deficiency of blood coagulation factor XII. To correct coagulopathy and compensate for factor XII deficiency, a transfusion of freshly frozen plasma in a volume of 10 ml/kg body weight (870 ml) was performed on the day of surgery and tranexam 1000 mg was administered intravenously. Preoperative correction of factor XII deficiency in the form of transfusion of freshly frozen plasma allowed during surgery and in the early postoperative period to fully compensate for factor XII deficiency and normalize APTT, which ensured adequate intraoperative hemostasis, surgical blood loss of no more than 200 ml. Immediately after surgery, enoxaparin was prescribed 0.4 ml subcutaneously per day for 7 days to prevent thromboembolic complications. The use of low molecular weight heparin eliminated the threat of postoperative thromboembolic complications. The course of the long-term postoperative period is without any special features.

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