Budd-Chiari syndrome at the young woman with thrombophilia and myeloproliferative disease

Abstract

In the clinic of various diseases (especially hematological) Budd-Chiari syndrome occupies a signifi cant place. The occurrence of blood fl ow disorders in the portal vein system can be a manifestation and the only symptom that manifests itself in a number of diseases. Given the rapid development of portal hypertension, this syndrome requires rapid diagnosis and timely initiated therapeutic measures, as well as comprehensive research to identify the etiological cause of Budd-Chiari syndrome.Objectives: In this paper we demonstrated a clinical case of a young patient with Budd-Chiari syndrome, an unusual course and a combination of etiological factors.Materials and methods. Data from the medical history of the patient who was on treatment at the surgical department of the City Multi-profile Hospital № 2, Military-medical Academy St. Petersburg were used as materials.Result. The patient developed severe ascites 3 months after delivery, for which she was unsuccessfully examined by gynecologists to exclude ovarian tumors. Then, in the course of laboratory and instrumental studies, a final diagnosis was made of Budd-Chiari syndrom (thrombosis of the intrahepatic part of the inferior vena cava in the phase of partial recanalization against the background of chronic myeloproliferative disease and genetically determined thrombophilia.Conclusion: As this case shows, in the development of non-cirrhotic portal hypertension, it is necessary to exclude hepatic thrombosis, in the detection of which it is recommended to conduct a comprehensive examination to exclude all causes of this thrombosis (genetic analysis for thrombophylia, antiphospholipid syndrome, Jak-2 v617f for exclude polycythemia and myelofi brosis, examination for cancer pathology). Manifestation and the only symptom of thrombophilia and idiopathic myelofi brosis may be acute Budd-Chiari syndrome, which requires complex medical and surgical treatment, further observation and especially treatment of causal diseases.