ДЕФИЦИТ XII ФАКТОРА У ДЕТЕЙ: КОАГУЛОПАТИЯ ИЛИ ЛАБОРАТОРНАЯ НАХОДКА?

Abstract

Factor XII deficiency is a non-hemorrhagic coagulopathy resulting in prolonged in vitro clotting time. At the same time, in our country there are cases when patients with factor XII deficiency receive preventive fresh frozen plasma before surgical treatment. Objective of the study: to analyze the manifestations of hemorrhagic and thrombotic syndrome in children with factor XII deficiency. Materials and methods of research: records in the medical history of patients with factor XII deficiency observed in our center on the basis of a consultative department and a short-stay hospital from January 2016 to October 2021 were retrospectively analyzed for hemorrhagic and thrombotic manifestations in the anamnesis, the type of surgical interventions performed, the need for hemostatic support. Study design: monocenter cohort, retrospective and observational, approved by the ethics committee of the Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The studies were carried out after the signing of the informed consent by the legal representatives of the child. Results: from January 2016 to October 2021 in the Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology (Moscow), 92 patients with factor XII deficiency were observed in the consultative department and the short-stay department of the hospital. The gender distribution was as follows: boys – 51 (55%), girls – 41 (45%). The median age at the time of diagnosis was 8 years (1st quartile for the 5-year period, 3rd quartile for the 10-year period). Of these, 22 patients underwent various surgical interventions. None of the patients had episodes of peri- and postoperative bleeding, none of the patients had episodes of thrombotic complications. There was no statistically significant association between the activity of factor XII and the severity of hemorrhagic syndrome according to the PBQ scale (Kendall's rank correlation coefficient 0.04, p=0.6). Conclusion: Factor XII deficiency is most likely a laboratory finding and does not increase the incidence of bleeding or thrombosis and therefore does not require routine drug therapy.