Atypical hemolytic uremic syndrome (aHUS) is a systemic disease, a type of thrombotic microangiopathy (TMA). It is based on uncontrolled activation of the alternative complement pathway of a hereditary or acquired nature, leading to generalized thrombosis in the microvasculature. Chronic activation of the alternative complement pathway leads to the damage of endothelial cells, erythrocytes and platelets and, as a result, to thrombotic microangiopathy and systemic multiorgan damage. Currently, in roughly half of the cases, it is impossible to identify aHUS triggers. Fresh frozen plasma (FFP) is used as first-line drug to reverse the symptoms. It helps to eliminate the deficiency of self-proteins – complement factor H and complement factor I (CFH and CFI), membrane cofactor protein (MCP), and stable and labile proteins – factors of hemostasis, and to stop thrombosis in the microvasculature. FFP administration is a preparatory step before anticomplementary therapy. Disease prognosis is always serious and is associated with severe complications and high mortality. At least 6 % of patients develop multiple organ failure with generalized TMA, injury of the central nervous system, gastrointestinal tract, lungs, and kidneys. The paper describes a clinical case of a patient with aHUS.

This analysis presents literature data, derived from open authoritative medical sources, dealing with current methods for earliest diagnosis of cardiotoxic complications, especially in the period of their subclinical manifestations. Opportunities and difficulties of diagnosing these complications are studied using the methods suggested, at the stage of sub-clinical myocardial dysfunction. The analysis results are presented in the form of a comparison chart covering positive aspects as well as the challenges occurring in real clinical practice. The current imaging methods showing the heart tissue condition and myocardial competence, characterize the relevant parameters more accurately. In addition to that, they enable to detect minimal changes as compared with standard 3D-echocardiography with the analysis of left ventricular ejection fraction. Therefore, many more new methods for studying cardiotoxicity should be implemented in real clinical practice.

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Введение. В процессе изучения особенностей течения онкогематологических заболеваний, в том числе неходжкинских лимфом (НХЛ), врачи-онкологи и гематологи нередко выявляют повышенный уровень антифосфолипидных антител (аФЛ) и неоднозначную их роль в прогнозировании осложнений. Цель исследования: определить клиническую значимость исходно повышенного уровня аФЛ в отношении риска возникновения тромботических осложнений у пациентов с диагнозом «индолентная НХЛ» в ходе противоопухолевой иммунохимиотерапии. Материалы и методы. На базе медицинских учреждений г. Самары было проведено ретроспективное когортное наблюдательное исследование 51 пациента с диагнозом «индолентная НХЛ». Больные были распределены в 2 группы: в основную группу вошли 28 больных с исходно повышенным уровнем аФЛ (в 1,3–1,5 раза больше верхней границы нормальных значений), в контрольную – 23 пациента с показателями, соответствующими референсным значениям. Пациентов обследовали в 3 визита: до лечения (V1), после всех курсов иммунохимиотерапии (V2) и через 6 мес (V3). Проанализированы клинико-демографические данные, анамнез тромботических осложнений, отслежен в динамике уровень аФЛ – антитела к β2-гликопротеину 1 (англ. anti-β2-glycoprotein 1 antibodies, anti-β2-GP1), волчаночный антикоагулянт (англ. lupus anticoagulant, LA), антитела к кардиолипину (англ. anti-cardiolipin antibodies, аСL). Результаты. Несмотря на закономерное снижение уровня аФЛ в процессе противоопухолевой терапии (с 126,5 ± 11,6 до 71,00 ± 6,14 Ед/мл для anti-β2-GP1; с 1,00 ± 0,21 до 0,75 ± 0,11 усл. ед. для LA; с 16,00 ± 0,97 до 7,40 ± 0,87 Ед/мл для aCL), в основной группе пациентов выявлена прямая взаимосвязь между исходно повышенным титром аФЛ и повышенной вероятностью наступления тромботических событий. Заключение. Определение титра аФЛ у пациентов с индолентными НХЛ до начала полихимиотерапии даст возможность специалистам онкогематологического профиля более точно стратифицировать больных по группам риска возникновения ранних и отсроченных тромботических осложнений. Предложенный подход в дополнение к общепринятой диагностической тактике позволит оптимизировать ведение пациентов с целью снижения количества нежелательных явлений. Introduction. While studying the features of hematologic malignancies, including non-Hodgkin’s lymphomas (NHL), oncologists and hematologists often reveal an increased level of antiphospholipid antibodies (aPА) and mention their controversial role in predicting complications. Aim: to define the prognostic significance of the initially elevated aPА level in relation to the risk of thrombotic complications in patients with indolent NHL during antitumor immunochemotherapy. Materials and Мethods. A retrospective cohort observational study of 51 patients with indolent NHL was conducted in Samara Clinics. The patients were divided into 2 groups: the main group included 28 patients with initially elevated aPA level (1.3–1.5 times higher than the upper limit of normal values), the control group included 23 patients with normal aPA values. Patients were examined at 3 visits: before treatment (V1), after all courses of immunochemotherapy (V2) and after 6 months (V3). Clinical and demographic data, a history of thrombotic complications analyzed; aPA level was monitored over time: anti-β2-glycoprotein 1 antibodies (anti-β2-GP1), lupus anticoagulant (LA), anti-cardiolipin antibodies (аСL). Results. Despite the predictable decreasing of aPL level during antitumor therapy (from 126.5 ± 11.6 to 71.00 ± 6.14 U/ml for anti-β2-GP1; from 1.00 ± 0.21 to 0.75 ± 0.11 units for LA; from 16.00 ± 0.97 to 7.40 ± 0.87 GPL/ml for aCL), a direct correlation was found between the initially elevated aPA titer and an increased probability of thrombotic events in the main group of patients. Conclusion. Determining aPA titer in patients with indolent NHL before polychemotherapy allows oncohematologists to more accurately stratify patients according the risk groups of early and late thrombotic complications. In addition to the generally accepted diagnostic strategies, suggested approach will optimize patient management aimed at reducing the number of adverse events.