The purpose of the study is to identify the components of immunity, which might predict the development of multiple organ failure in patients after heart surgery.Material and methods. The study included 40 patients who were operated in cardiac surgery Department. The inclusion criteria were the presence of indications for cardiac surgery. The exclusion criteria were the presence of infective endocarditis in patients. Before the operation and after 24 hours we studied the blood level of leukocytes and lymphocytes. We analyzed the phenotype of immune cells using monoclonal antibodies, serum levels of procalcitonin and C3 and C4 complement components. All patients were evaluated for multiple organ disfunction (MOD) using the SOFA scale.The results showed that cardiac surgery leads to the development of MOD, statistically significant multidirectional changes in both quantitative and qualitative composition of all cells of the immune system, significant changes in the level of C3 and C4 components of the cascade of complement and plasma level of pro-calcitonin. ROC analysis was revealed that the relative content of monocytes is less than 7.1% of the number of leukocytes as well as the absolute content of monocytes with the CD14 + HLA-DR + phenotype less than 0.32_109/l in the preoperative period, and the C3 level of the complement component less than 0.52 g/l, as well as the maximum SOFA score in the postoperative period, were the best predictors of MOD after the procedures.Conclusion. The components of innate immunity make it possible to predict the complication of the cardiac surgery, earlier than the SOFA scale.

Introduction Paroxysmal nocturnal hemoglobinuria (PNH) is a rare and life-threatening hematopoietic stem cell disease caused by a partial or absolute deficiency of proteins linked to the cell surface membrane via a glycosylphosphatidyl-inositol anchor, which leads to complement-induced intravascular hemolysis mediated via the membrane attack complex. Multiparameter high-sensitivity flow cytometric measurement of PNH clones is the method of choice for the diagnosis of PNH, as recommended by the International Clinical Cytometry Society (ICCS). After publication of the ICCS guidelines, screening of patients considered at high risk of PNH was commenced in Russia. Data are presented on PNH clone size distribution across patients with relevant ICD-10 diagnostic codes (based on patients′ initial assumed diagnoses). Methods Patients were tested for the presence and size of PNH clones using high-sensitivity flow cytometry across nine laboratories. PNH clone evaluations were performed as described in the ICCS guidelines: CD59/CD235a monoclonal antibodies for RBC; CD45/CD15/CD24/FLAER for granulocytes and; CD45/CD64/CD14/FLAER or CD45/CD33/CD14/FLAER for monocytes. The sensitivity for PNH clone detection was 0.01%. Changes in PNH clone size were evaluated among patients who had follow-up studies after initial measurements. Results 1889 patients were assessed between October 2011 and June 2013 (Table 1). Suspected PNH and bone-marrow disorders (AA, MDS, cytopenia) were the most common reasons for PNH testing. The greatest proportions of patients with PNH clones were among those with of an initial assumed diagnosis of AA or PNH. Notably, around 40% of patients with an initial assumed diagnosis of PNH actually had no detectable PNH clones. Most patients with small clone sizes (< 1%) were in the AA, MDS and hemolytic anemia groups. Overall, mean clone sizes were slightly higher in monocytes (31.5%) than in granulocytes (30.1%) across the diagnostic categories. While there was generally a good correlation between clone size measurements in granulocytes and monocytes (linear regression r2 = 0.9851), 10% of PNH-positive patients had detectable clones only in one of these leucocyte populations (i.e. either in monocytes or in granulocytes, but not both). PNH clones in RBCs were generally lower than in granulocytes. Repeat clone size measurements were performed in 316 patients over a mean follow-up period of 7.8 months. In patients with initial clone sizes <50% the PNH clones tended to decrease over time, whereas in patients with initial clone sizes >50%, clones tended to increase. PNH clones were not changed at all in 98 patients at follow-up, among whom 48% were patients with AA. Conclusion These screening data confirm the utility of high-sensitivity flow cytometry testing in high-risk patient groups to ensure early and accurate diagnosis and to aid in the effective clinical management of patients. Disclosures: Babenko: Alexion: Research Funding. Sipol:Alexion: Research Funding. Borisov:Alexion: Employment. Naumova:Alexion: Research Funding. Boyakova:Alexion: Research Funding. Glazanova:Alexion: Research Funding. Chubukina:Alexion: Research Funding. Pronkina:Alexion: Research Funding. Popov:Alexion: Research Funding. Mustafin:Alexion: Research Funding. Fidarova:Alexion: Honoraria. Lisukov:Alexion: Honoraria. Kulagin:Alexion: Honoraria.

Адрес для переписки:Базарный Владимир Викторович ГБОУ ВПО «Уральский государственный медицинский университет» Минздрава РФ 620028, Россия, г. Екатеринбург, ул. Репина, 3. Тел.: 8 (912) 288-85-90. E-mail: vlad-bazarny@yandex.ru Address for correspondence : Bazarnyi Vladimir V. Ural State Medical University 620028, Russian Federation, Ekaterinburg, Repin str., 3. Phone: 7 (912) 288-85-90. E-mail: vlad-bazarny@yandex иммунология, 2015. Т. 17, № 1. С. 93-96. doi: 10.15789/1563-0625-2015-1-93-96 © Базарный В.В. и соавт., 2015 Immunologiya, 2015, Vol. 17, no. 1, pp. 93-96. doi: 10.15789/1563-0625-2015 Резюме. В исследовании 15 пациентов с аутоиммунными заболеваниями печени и 36 пациентов без аутоиммунной патологии установлена диагностическая информативность тестов определения антинуклеарных и антимитохондриальных аутоантител (АМА-М2), а также антител к асиалогли-копротеиновому рецептору (anti-ASGPR). На основе ROC анализа показано, что диагностическая чувствительность и диагностическая специфичность для АМА-М2 составили 73 и 100%, а для anti-ASGPR -только 60 и 77% соответственно. Следовательно, тест на anti-ASGPR при аутоиммунных заболеваниях печени не показал преимуществ перед уже существующими, и целесообразность его использования в клинической практике требует уточнения. Ключевые слова: аутоиммунный гепатит, первичный билиарный цирроз, аутоантитела CLINICAL DIAGNOSTIC VALUE OF AUTOANTIBODIES IN THE DIAGNOSIS OF AUTOIMMUNE LIVER DISEASESBazarnyi V.V., Mikhailova E.Yu., Partylova E.A. Ural State Medical University, Ekaterinburg, Russian FederationAbstract. We are studied the 15 patients with autoimmune liver diseases and 36 patients without autoimmune pathology found the diagnostic value of antinuclear and antimitochondrial autoantibodies (AMA-M2) tests, and antibodies to asialoglycoprotein receptor (anti-ASGPR). Based on the ROC analysis showed that the diagnostic sensitivity and diagnostic specificity of AMA-M2 was 73% and 100% and for anti-ASGPR -60% and 77%, respectively. Therefore, the test for anti-ASGPR in autoimmune diseases of the liver showed no advantages over standart tests, and its using in clinical practice requires clarification.

Essentialy, the diagnostics feature of myeloproliferative neoplasms (MPNs) is a genetic mutations. While bcr-abl translocation belongs to CML, Jak2V617F mutation is commonly associated with other MPNs. The recent decade has been clarified a data when a human blood presents bcr-abl translocation and Jak2V617F mutation both. This clinical case reports of bcr-abl translocation and Jak2V617F mutation when they were detected simultaneously by polymerase chain reaction (PCR) in a patient with myeloproliferative neoplasm, unclassifiable (MPN-U).