BACKGROUND. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal hematologic disease manifesting among others in hemolytic anemia. Due to predominantly intravascular hemolysis, iron metabolism in PNH patients is characterized by a number of features. Pathogenetic treatment with complement C5-inhibitors modifies the course of hemolysis and results in iron metabolic changes. AIM. To characterize the serum parameters of iron metabolism in PNH patients and to evaluate T2* MRIs of the liver and kidneys. MATERIALS & METHODS. Serum parameters of iron metabolism measured in 97 analyses and 99 T2* MRIs of the liver and kidneys were assessed in 82 PNH patients treated at the National Research Center for Hematology from 2018 to 2024. Patients were 21–76 years of age (median 39 years); there were 38 women and 44 men. RESULTS. The analysis focused on the data from 78 PNH patients. Among non-recipients of C5-inhibitors, iron overload in liver tissue was identified in 10/38 (26.3 %) patients. In C5-inhibitor recipients, iron overload in liver tissue was found significantly more often, i.e. in 28/40 (70 %) patients. Iron overload in liver tissue was more pronounced in patients with suboptimal hematologic response to PNH therapy. A renal T2* MRI was performed in 71 patients. In all non-recipients of C5-inhibitors (n = 34), iron overload in renal tissue was detected. In C5-inhibitor recipients, iron overload in renal tissue occurred significantly less often, i.e. in 21/37 (57 %) patients. CONCLUSION. Iron overload in renal cortex was identified in all PNH patients who were not treated with C5-inhibitors. Iron overload in liver tissue was associated with blood transfusions and suboptimal hematologic response to pathogenetic PNH therapy. In evaluating iron overload in liver and renal tissues, laboratory methods for assessing iron metabolism in PNH patients have lower sensitivity compared to T2* MRI. In PNH patients, the achievement of optimal response to C5-inhibitor treatment is associated with a decrease in severity of iron overload in renal tissue and no hepatic hemosiderosis.

Introduction. As a result of portal hypertension (sequestration of platelets in an enlarged spleen) and liver failure (decreased production of thrombopoietin in the liver) in liver cirrhosis, thrombocytopenia develops, which is associated with the risk of periprocedural/perioperative bleeding complications. There are still unresolved questions regarding risk stratification of bleeding complications, the prognostic role of thrombocytopenia, as well as the need for treatment of thrombocytopenia and its methods.Materials and methods. The Russian Scientific Liver Society selected a panel of experts in the field of therapeutic and surgical hepatology, hematology, transfusion medicine to make reasoned statements and recommendations on the issue of treatment of thrombocytopenia before elective surgery / invasive procedures in patients with liver cirrhosis.Results. Relevant clinical issues were determined based on the PICO principle (patient or population, intervention, comparison, outcome). The Delphi panel made five questions and gave reasoned answers, framed as ‘clinical practice recommendations and statements’ with evidence-based comments. The questions and statements were based on the results of search and critical analysis of medical literature using keywords in English- and Russian-language databases. The formulated questions could be combined into four categories: bleeding risk stratification, the prognostic value of thrombocytopenia, the necessity and methods of thrombocytopenia drug correction, and bleeding risk reduction.Conclusions. The results of experts' work are directly related to high-quality management of patients with liver cirrhosis and thrombocytopenia, who have scheduled invasive procedures/surgery. Thus, this recommendations and statements can be used in clinical practice.

The paper presents the experience of successful treating a patient with diuretic-resistant ascites associated with Budd– Chiari syndrome. The authors provide a clinical observation of deceased donor liver transplantation to a patient with Budd–Chiari syndrome type 1, total hepatic vein occlusion, and diuretic-resistant ascites after portacaval shunt surgery. The post-transplantation period is observed as stable, with no signs of liver failure; ascites is reduced. The first experience of surgical treatment of a patient with Budd–Chiari syndrome, as well as the first experience of liver transplantation after portacaval shunting is appeared to be successful.

OBJECTIVE:To determine the features of preoperative hemostatic therapy and laboratory control in patients with severe and inhibitory forms of hemophilia A under preventive treatment by FVIII.MATERIAL AND METHODS:Four patients with severe and inhibitory forms of hemophilia A underwent surgery between 2021 and 2022. All patients received Emicizumab (the first monoclonal drug for «non-factor» therapy of hemophilia) for prevention of specific hemorrhagic signs of hemophilia.RESULTS:Surgical intervention under preventive Emicizumab therapy was essential. Additional hemostatic therapy was not carried out or performed in reduced mode. There were no hemorrhagic, thrombotic or other complications. Thus, the so-called «non-factor» therapy is one of the variants for uncontrollable hemostasis in patients with severe and inhibitory forms of hemophilia.CONCLUSION:Preventive injection of Emicizumab ensures certain buffer of hemostasis system and stable lower limit of coagulation potential. This is the result of stable concentration of Emicizumab when used in any of the registered forms regardless of age and other individual characteristics. The risk of acute severe hemorrhage is excluded, while the probability of thrombosis is not increased. Indeed, FVIII has higher affinity than Emicizumab and displaces Emicizumab from coagulation cascade that does not result summation of total coagulation potential.

Seven of the country’s leading experts in the field of therapeutic and surgical hepatology answer questions on this issue: how relevant is thrombocytopenia in patients with CLD for clinical practice, what is the average proportion of patients who need correction of thrombocytopenia before scheduled and emergency surgical/invasive interventions and how is thrombocytopenia correction performed. All experts are unanimous in the opinion that thrombocytopenia is most common at the stage of cirrhosis. Further, the experts discussed the issues of the total number of patients with CLD, the proportion of patients with thrombocytopenia at all, the proportion of patients with thrombocytopenia in need of surgical interventions, the experts were asked to provide schemes for correcting thrombocytopenia, which they use in their clinical practice. The focus of the experts’ attention was on the discussion of patients who have a platelet level of less than 50 000/µl and need scheduled surgical interventions / invasive procedures. All experts unanimously stratify these interventions according to the risk of hemorrhagic complications: high – more than 1.5%, low – less than 1.5%. The most common and available method for the correction of thrombocytopenia at present is platelet transfusion. Details of all this information, as well as statistical data, can be found in this article.

ОБОСНОВАНИЕ Наличие ингибиторов к факторам свертывания крови осложняет течение заболевания у 15—32% больных гемофилией. Любое оперативное вмешательство у пациентов этой группы сопряжено с высоким риском развития тяжелых геморрагических осложнений как во время операции, так и в послеоперационном периоде, что требует обеспечения надежного гемостаза и четкого лабораторного контроля эффективности проводимой гемостатической терапии. ЦЕЛЬ ИССЛЕДОВАНИЯ Обозначить проблемы обеспечения гемостаза при выполнении хирургического вмешательства у больных ингибиторной формой гемофилии. МАТЕРИАЛ И МЕТОДЫ Осуществлен анализ 5 хирургических вмешательств у больных с ингибиторной формой гемофилии. Все операции проведены в ФГБУ НМИЦ гематологии в 2016—2020 гг. Медиана возраста составила 46 лет. Все хирургические вмешательства выполнены на органах брюшной полости: 4 операции с использованием лапароскопического доступа (1 холецистэктомия, 2 герниопластики, 1 уретеролитотомия) и 1 «открытое» оперативное вмешательство — герниопластика. Гемостатическая терапия осуществлялась препаратами антиингибиторного коагулянтного комплекса и rFVIIa. РЕЗУЛЬТАТЫ Осложнения в послеоперационном периоде зафиксированы у 4 больных: у 3 были геморрагические осложнения, у 1 — тромботические. Геморрагические осложнения наблюдались в 1-е сутки после операции и требовали коррекции гемостатической терапии. Решающим фактором критического тромбоза селезеночной артерии одного из пациентов явилось повышение коагуляционного потенциала крови при введении rFVIIa на фоне истощения фибринолитической системы (удлинение XIIa-зависимого фибринолиза с 25 до 75 мин) и снижения концентрации антитромбина III, участвующего в инактивации FVIIa, до 81%. ЗАКЛЮЧЕНИЕ Применение стандартных протоколов гемостатической терапии не обеспечивает надежного и безопасного гемостаза при ингибиторной форме гемофилии. При назначении гемостатической терапии больным гемофилией необходимо учитывать индивидуальные характеристики каждого конкретного пациента: компенсаторные механизмы свертывающей системы, сочетанную патологию, изменение клинической ситуации. Для персонализированного подбора схемы гемостатической терапии и контроля системы гемостаза в реальных физиологических условиях необходимо до хирургического вмешательства и на протяжении периоперационного периода оценивать результаты всех возможных гемостазиологических тестов, как общепринятых, так и интегральных, для снижения риска развития геморрагических и тромботических осложнений.

Ten patients with cirrhosis and portal hypertension successfully underwent balloon-occluded retrograde transvenous obliteration (BRTO) of gastric varices. As an example, the paper presents a clinical observation demonstrating the use of a BRTO endovascular procedure. A spontaneous functioning gastrorenal shunt was distinctly observed during multidetector computed tomography in a patient with Child-Pugh А cirrhosis of alimentary-toxic and viral etiology, as well as portal hypertension and associated gastric varices (Type 2, Grade 3) with frequent recurrent bleeding. The presence of a shunt with no indications for TIPS procedure, as well as the inexpediency of a shunt surgery (taking positive immunoblot into account), enabled the formulation of indications for BRTO. This factor ensured regression of gastric varices, as well as eliminated the threat of recurrent gastric bleeding.

Ph-negative myeloproliferative disorders such as polycythemia vera (PV), essential thrombocythemia, and primary myelofibrosis are rare clonal hematopoietic diseases that typically occur at age over 50. Only 10–15% of PV cases are diagnosed during the reproductive years, most commonly manifested as splanchnic venous thrombosis (SVT). Here, we demonstrate a case of a 6-weeks pregnant woman with a history of stillbirth and miscarriage presented with SVT. A terminal ileal resection and right hemicolectomy were performed, as well as the appointment of low molecular weight heparin in the postoperative period was initiated that allowed the patient to continue the pregnancy and deliver a child. The JAK2 V617F mutation was detected and masked PV was diagnosed according to bone marrow findings. In this article, we discuss the etiology of SVT in adults and effect of JAK2 V617F mutation on pregnancy outcomes as well as summarize current knowledge about pregnancy outcomes in PV.

КЛИНИЧЕСКАЯ И ЭКСПЕРИМЕНТАЛЬНАЯ ХИРУРГИЯ■ РЕКОНСТРУКТИВНАЯ И МИКРОСОСУДИСТАЯ ХИРУРГИЯ Цель -ретроспективный анализ результатов и оптимизация технологии портокавального шунтирования у больных с внепеченочной портальной гипертензией (ВПГ).Материал и методы.Проанализированы результаты портокавального шунтирования у 407 больных с ВПГ с 1975 по 2020 г.Медиана возраста составила 26,5 лет, 216 женщин, 191 мужчина.Показаниями к хирургическому лечению во всех наблюдениях были рецидивы гастроэзофагеальных кровотечений или эндоскопические признаки угрозы их возникновения наряду с невозможностью эндоскопического лечения.Результаты.Выполнение портокавального шунтирования у данной категории больных представляет значительную сложность из-за малого диаметра анастомозируемых сосудов портальной системы, часто встречающегося тромбоза, а также постфлебитических изменений сосудов (склероз и значительное истончение сосудистой стенки).Внедрение элементов микрохирургической техники и использование Н-образных анастомозов с аутовенозным или синтетическим графтом позволили увеличить выполнимость операции портокавального шунтирования с 10-15 до >50%.Наиболее частыми и значимыми осложнениями раннего послеоперационного периода были гастроэзофагеальные кровотечения, чаще всего связанные с нарушением проходимости сосудистого анастомоза, и хилоперитонеум, обусловленный пересечением расширенных лимфатических коллекторов и применением коагуляции для предотвращения лимфореи.Послеоперационная летальность составила 1,50%.Наилучшие результаты получены при выполнении портокавальных анастомозов «бок в бок» с магистральными сосудами портальной системы.Выполнимость спленоренальных анастомозов составила 17,0%, а мезентерикокавальных -27,8% общего числа выполненных шунтов.Наиболее часто выполнимым (40,6%) и достаточно эффективным был мезентерикокавальный анастомоз Н-типа, однако стеноз и тромбоз этого вида шунта возникали в 4,76 раза чаще, чем при формировании соустья «бок в бок».В отдаленном периоде основными осложнениями были рецидивы гастроэзофагеальных кровотечений (8,60%), связанные с тромбозом (5,20%) или со стенозом шунта (3,40%).Шунтовая энцефалопатия отмечена в 1,60% наблюдений.Летальность в отдаленном периоде, связанная с осложнениями портальной гипертензии и последствиями хирургических вмешательств, составила 3,40%.Заключение.При наличии любого сосуда портальной системы, пригодного для создания анастомоза, портокавальное шунтирование является операцией выбора у больных с ВПГ и угрозой кровотечения из варикозно расширенных вен пищевода и желудка.Отдаленные результаты этих операций свидетельствуют о высокой эффективности в отношении профилактики пищеводно-желудочных кровотечений.В большинстве наблюдений операции портокавального шунтирования у больных с ВПГ позволяют обеспечить их полную медико-социальную реабилитацию на длительный период.

Hemostasis disorders in cirrhosis / severe liver disease are complex, as they affect both pro- and anticoagulant factors, as well as pro- and antifibrinolytic components. Most of the tests that are used in clinical practice to assess coagulation do not take into account the compensatory capabilities of this system as a whole. This document provides guidance for the optimal application and interpretation of global screening tests in the assessment of hemostasis in cirrhosis/severe liver disease, analysis of risk factor of spontaneous and perioperative hemorrhagic complications in these patients, and possible ways to correct them. Thrombocytopenia is one of the most common hematological abnormalities in cirrhosis/ severe liver disease. The frequency of thrombocytopenia in such patients at the stage of liver cirrhosis is 70%, at the pre-cirrhotic stage – 6%. The latest scientific data on the use of thrombopoietin receptor agonists in chronic liver diseases are presented: which patients are the best candidates for a such of therapy. The rationality and limitations in prescribing blood components to patients with liver cirrhosis/severe liver diseases are described. Emphasis is placed on the fact that a number of hemorrhagic complications develop for other non-coagulopathic reasons: decompensated portal hypertension, traumatization of a varicose vein with trophic wall disorders, etc. Data are presented on the use of viscoelastic tests to optimize the management of patients with liver cirrhosis, which simultaneously assess the four main known components of the hemostasis system: the coagulation cascade, platelets, anticoagulant mechanisms and the fibrinolysis system. These tests allow optimization of transfusion of blood components in such patients and should be further studied. Research in this very complex area of hepatology is ongoing and must continue.

Aim. Current clinical recommendations are intended to supply gastroenterologists, physicians and general practitioners with modern methods for the diagnosis and treatment of liver cirrhosis and fibrosis.Key points. Liver fibrosis develops with connective tissue accumulation in liver in the outcome of various chronic diseases, including alcohol misuse, viral hepatitises, autoimmune and more rare hereditary liver diseases. Liver cirrhosis is the final stage of most chronic diffuse liver diseases. The recommendations present current opinions on pathogenesis of liver fibrosis and cirrhosis, principles of diagnosis, treatment and prevention of their main complications: hepatic encephalopathy, oesophageal and gastric variceal bleeding, acute kidney injury/hepatorenal syndrome, infectious complications (i.a. spontaneous bacterial peritonitis), hyponatraemia, pulmonary complications, etc.Conclusion. Timely diagnosis and adequate therapy in cirrhosis can prevent life-threatening complications and improve the patients’ prognosis and quality of life.

This study demonstrates a successful clinical outcome in a female patient with severe refractory ascites associated with a complex combined form of extrahepatic portal hypertension. The study emphasizes the need and particular importance of comprehensive diagnostics and assessment of the angioarchitectonics in the portal vein system, thorough study of the porto–hepatic circulation and central hemodynamics, as well as of other major homeokinesis disorders. Understanding the pathogenesis of the disease allows for the development of a therapeutic plan with a strictly personalized approach toward the selection and staging of various surgical interventions.

Objective. To optimize the management strategies in pregnant women with portal hypertension (PH) of different genesis. Patients and methods. Forty-four women with PH aged 19 to 44 years were examined and divided into two groups depending on the pathogenesis of PH. Group I (n = 24) included women with extrahepatic PH, group II (n = 20) – women with hepatic PH. Esophagogastroduodenoscopy, ultrasound, doppler ultrasound, fetal cardiotocography, and clinical and biochemical laboratory examinations were used as research methods. Results. Thrombophilia, anemia of varying severity, and liver cirrhosis have an impact on the development of pre-eclampsia, threatened preterm labor, placental insufficiency, and low birth weight in newborns. PH during pregnancy is a risk factor for bleeding from esophageal varices. Careful monitoring of patients using instrumental methods and management of pregnancy by obstetrician-gynecologists and surgeons create conditions for favorable perinatal outcomes. Conclusion. The risk of bleeding from esophageal varices during pregnancy, regardless of gestational age, and in the postpartum period is significantly more common in patients in group I compared to group II; the method of prevention is anticoagulant therapy. Key words: pregnancy, placental insufficiency, portal hypertension, preterm birth, cirrhosis

Clinical case demonstrates successful treatment of a pregnant woman, who had multiple giant splenic artery aneurysms with a high risk of spontaneous rupture. Pregnancy proceeded along with hereditary thrombophilia, thrombosis and cavernous transformation of the vena cava, extrahepatic portal hypertension, esophageal and gastric varices II–III. The surgical and obstetric tactics, surgical intervention, appropriate salvage and multidisciplinary pregnancy follow-up ensured a successful Cesarean delivery at 38 weeks, saving the life of the child and mother.

ОБОСНОВАНИЕ Кровотечения портального генеза в стационаре являются самой частой причиной смерти пациентов с циррозом печени. В связи с этим вопрос о выборе сроков и способах их остановки является чрезвычайно актуальным. ЦЕЛЬ ИССЛЕДОВАНИЯ Сравнительный анализ эффективности и безопасности эндоскопического лигирования как способа экстренного гемостаза первой линии и эндоскопического лигирования, выполненного в срочном порядке после предварительного компрессионного гемостаза зондом Сенгстакена—Блэкмора у пациентов с кровотечением портального генеза. МАТЕРИАЛ И МЕТОДЫ В статье проанализированы результаты лечения 57 пациентов с острыми кровотечениями из варикозно-расширенных вен пищевода и желудка. Отличия в сравниваемых группах заключались в выборе сроков лигирования и использования предварительной компрессионной терапии. РЕЗУЛЬТАТЫ Полученные результаты свидетельствуют об идентичности методов эндоскопического лигирования у пациентов в экстренном и срочном порядке независимо от применения предварительного компрессионного гемостаза. ЗАКЛЮЧЕНИЕ Эндоскопическое лигирование как способ экстренного гемостаза первой линии является не менее эффективным и безопасным, чем эндоскопическое лигирование, выполненное в срочном порядке после использования временного компрессионного гемостаза зондом Сенгстакена—Блэкмора и сопровождается статистически значимым уменьшением длительности пребывания в стационаре пациентов с кровотечениями портального генеза.