ТРОМБОЗ ГЛУБОКИХ ВЕН У ПАЦИЕНТКИ С БОЛЕЗНЬЮ ВИЛЛЕБРАНДА

Objective: to identify the possible factors of thrombogenic risk and ways of its prevention in patients with von Willebrand disease. Case description. Patient X., 42 years old, who suffers from von Willebrand disease type 3 with 5-years of age. Asked on reception to the traumatologist in the polyclinic of the Regional Hospital with pain in the left hip joint. Recommended planned operative treatment in the Altai Regional Clinical Hospital. Preoperative preparation included the infusion of concentrate of von Willebrand factor and coagulation factor VIII. Operation – cement total arthroplasty of the left hip joint. In the postoperative period analgesic treatment, elastic compression of the lower extremities, iron supplements, also conducted infusion of concentrate of von Willebrand factor and coagulation factor VIII for 20 days and thromboprophylactic with dabigatran. On the 3rd day after the operation the patient revealed deep vein thrombosis of the femoral segment (floating clot). Results. The patient was operated for emergency indications in the Department of endovascular surgery – installation of venous cava filter “Volan”. Dabigatran is cancelled, appointed clexane for 3 months. In our clinical example the patient lacked risk factors of pulmonary embolism as obesity, age, smoking, prolonged immobilization, estrogen therapy. Overdose of factor VIII were not observed – the level of factor did not exceed 135 % on transfusions. At the same time, the patient was found polymorphisms in the genes ITGA2, FGB, MTHFR, MTR – heterozygote, MTRR – mutant homozygote, which may indicate the genetic factors of thrombogenic risk. Also a significant risk factor was massive surgical intervention (total hip replacement). Despite preventive measures (elastic compression, thromboprophylactic dabigatran, early activation) we cannot to avoid thrombotic complications. Conclusion. This article presents a case demonstrating a thrombotic complication in patients with von Willebrand disease type 3 in the postoperative period of total hip arthroplasty. It is shown that despite preventive measures, she developed a very rare von Willebrand disease the phenomenon of deep vein thrombosis, bleeding was not observed. Pulmonary embolism was avoided by the installation of cava filter.