bluuebunny/crossref_metadata_2025_split huggingface dataset:
PB2390 THE ROLE OF T REGULATORY CELLS IN THE PATHOGENS AND IMMUNITY OF BETA‐THALASSEMIA MAJOR PATIENTS
Authors: Adly A. , Ismail E. , Saleh M. , Hamed G. 
Published: June 2019

Background:The basis of the pathogenesis of the immunodeficiency in thalassemia is iron overload and allogenic stimulation caused by multiple blood transfusions. T regulatory cells (Tregs) are a component of the immune system that constitutes a key mechanism in maintaining peripheral self‐tolerance through the control of auto‐reactive lymphocyte activation.Aim:To determine the frequency of Tregs in children and adolescents with β‐thalassemia major and assessed their relation to the clinical and laboratory characteristics of patients including transfusion therapy, iron overload and alloimmunization.Methods:Sixty β‐TM patients studied focusing on transfusion history and chelation therapy. Hematological and biochemical data including markers of hemolysis and serum ferritin were assessed. Patients’ records were analyzed for the presence of alloantibodies. Assessment of T regulatory cells was done using flow cytometry. Patients were compared with 30 healthy controls.Results:The percentages of CD4+ T lymphocytes and Tregs were significantly reduced in β‐TM patients compared with the control group (p < 0.05). Patients with patients with splenectomy, heart disease and pulmonary hypertension risk had lower levels of these cells than those without. The percentage of Tregs was decreased among patients with serum ferritin ≥2500 μg/L compared with patients below this cutoff and in patients with history of alloimmune antibodies. There were significant negative correlations between Tregs and each of indirect bilirubin and ferritin levels while they were positively correlated with WBCs counts and hemoglobin.Summary/Conclusion:Conclusion: Children and adolescents with β‐TM may encounter a state of immune dysfunction. Low frequency of Tregs in β‐TM patients may contribute to the formation of alloantibodies. Alterations in Tregs are more evident in relation to iron overload. It is important to assess the suppressive activity of these cells in standard proliferation assays to examine whether their function is disrupted in β‐TM together with the alteration in their frequency or not.
PB2406 THE INFLUENCE OF XMNI AND BCL11A GENE MODIFIERS ON HEMOGLOBIN F LEVELS IN EGYPTIAN β‐THALASSEMIA MAJOR PATIENTS
Authors: Nasreldin E. , galal S. , atwa M. , abdellatif H. , idris N. , ezzat G. , gaber M. , dahpy M. , raffat D. 
Published: June 2019

Background:β‐thalassemia major (β‐TM) is an autosomal recessive disorder in which β‐globin chain synthesis is severely decreased or absent which lead to blood transfusion dependency with its catastrophic complications. Polymorphisms at position ‐158 of XmnI‐HBG2 on chromosome 11 and BCL11A site on chromosome 2p16 might be associated with elevated hemoglobin F (HbF) expression which may,in turn,improve β‐thalassemia severityAims:This study aims to explore the modifying effects of XmnI and BCL11A loci on HbF levels in Egyptian β‐thalassemia patientsMethods:A prospective case‐control study of 70 multi‐transfused β‐thalassemia major patients and 22 controls was conducted in Paediatric hematology unit of Assiut university hospital from January 2016 till April 2017. PCR‐RFLP was used for detection of single nucleotide polymorphisms at XmnI and BCL11A site loci.Results:XmnI Polymorphism detected in 9 of 70 patients and associated with higher mean HbF levels (53.48%) than patients without polymorphism (meanHbF level was 42.23%)(P‐Value = 0.035).The frequency of CT heterozygousgenotype was 8(11.4%), TT homozygous genotype was (1.4%) while the wild genotype CC detected in 61 (87.1%) of the cases. While BCL11A Polymorphism detected in 21 of 70 patients had no effect on either Hb or HbF levels (P‐Value = 0.26). The TT genotype frequency was 49 (70%) and TC heterozygous genotype was detected in 21 (30 %) of patients. The CC genotype was absent.Summary/Conclusion:The presence of XmnI polymorphism rather than BCL11A polymorphism has a modifying effect on HbF production and increased Hb levels between β‐TM patients and so, it helps in identifying patients’ benefits from HU therapy.image
PB2412 ADULT THALASSEMIA PATIENTS OF PEDIATRIC HEMATOLOGY CLINIC: A SINGLE CENTER EXPERENCE IN TURKEY
Authors: Karakas Z. , Ocak S. , Bilici M. , Yılmaz Y. , Tuna R. , Karaman S. , Tugcu D. , Unuvar A. 
Published: June 2019

Background:Advances in understanding and treatments in transfusion dependent thalassemia (TDT) improved the clinical outcomes. Disease‐related complications seem to be the challenging problem in aging patients with thalassemia who now survive longer than in the past.Aims:Here we aimed to describe clinical features of adult thalassemia patients followed on the pediatric hematology clinic.Methods:All the patients with TDT and older than 25‐years were reviewed retrospectively. Demogrophical, cilinical features, status of iron overload and treatment and disease related complications were recorded.Results:A total of 69 patients were evaluated. Thirty three were female and 36 were male. Mean age was 32.9 years (range 25‐53 years). Mean duration of follow‐up and iron chelation therapy were 31,2 years (range 14‐50 years), and 25 years (range 7‐ 31 years), respectively.Fortyfour patients had splenectomy. Mean ferritin levels was found to be 1645 ng/ml (range between 361‐8600 ng/ml). Cardiac iron status were evaluated in 44 patients and cardiac T2∗was normal (>20 msec) in 40 patients, moderate and severe iron overload was shown in 1 and 3 patients, respectively. 1 patients had moderate. Liver iron status was normal (<2 mg) in 16 out of 53 patients with liver T2∗ MRI. Liver iron overload was found to be mild (2‐5 mg) in 11 patients, moderate (5‐10 mg) in 10 patients and severe (>10 mg) in 16 patients. A strong positive correleation was found between serum ferritin levels and liver iron overload (p < 0.001). There was no significant association between cardiac iron overload and serum ferritin levels. Also a slight association was shown cardiac and liver iron overload (p = 0.03).Summary/Conclusion:In our country the adult TDT patients are still on follow‐up in pediatric hematology clinics based on the experience of the pediatric hematologists on those patients. Although these older patients were constituted a new cohort, in this study the clinical features and status of iron overload in older TDT patients were found to be acceptable and similar to younger TDT's regarding despite long period of regular transfusion and histroical features of the treatment.
PB2416 LABORATORY INVESTIGATION OF HEMOGLOBINOPATHIES IN HUNGARY
Authors: Mezei Z. A. , Bereczky Z. , Nagy E. , Antal‐Szalmás P. , Kappelmayer J. 
Published: June 2019

Background:About 7% of the world population is affected by genetic defects of the hemoglobin molecule, making hemoglobinopathies the number one monogenic disease. Hemoglobinopathies were most common historically in the Mediterranean region and in African and south East Asian populations, but with the increase in migration from the twentieth century they are present worldwide. In Hungary the most common hemoglobinopathy is beta thalassemia, but is not endemic. In the majority of cases, the patients carrying the disease have little or no clinical symptoms. Routine laboratory investigations show a microcytic anemia, but to confirm the diagnosis further laboratory investigations are needed.Aims:To investigate the frequency of the different beta thalassemia mutations in Hungary and to present an algorithm for the investigation of microcytic anemias.Methods:In the period 2005‐2018, overall 3666 patients were investigated with the possible diagnosis of beta thalassemia. In all cases venepuncture was performed, and EDTA anticoagulated blood was collected from the antebrachial vein. Hemoglobin analysis was carried out with a high‐performance liquid chromatography method on a Variant II analyzer (Bio‐Rad, Hercules, CA, USA), and HbA2 and HbF levels were recorded. From 1704 samples DNA was isolated and Sanger sequencing of the beta globin gene was performed on an ABI Prism 310 Genetic analyzer (Applied Biosystems, Foster City, CA, USA).Results:In 1470 cases (40%), we found elevated HbA2 and/or HbF levels. In 1704 cases Sanger sequencing was performed and in 804 patients we found a mutation in in the beta globin gene as a cause of beta thalassemia. In 4 cases, a mutation leading to hemoglobin variants with increased O2 affinity were identified. Janus Kinase 2 (JAK 2) gene exon 12 mutations were not present in these patients with altered O2 affinity, but elevated hemoglobin and erythrocyte counts were observed, in accordance with the genetic results.Summary/Conclusion:We suggest an algorithm for the investigation of microcytic anemias in regions where the disease is not endemic, along with a few examples from our laboratory. Hemoglobin analysis is important in disorders where beta thalassemia might be suspected like hemolytic anemia, drug induced anemia and hydrops foetalis.
PB2420 EFFICIENCY OF DUAL ANTIPLATELET THERAPY AFTER PERCUTANEOUS CORONARY INTERVENTION
Authors: Matvienko O. , Smirnova O. , Gelzer I. , Tarkovskaya L. , Golovina O. , Papayan L. 
Published: June 2019

Background:Percutaneous coronary intervention (PCI) is the treatment metod of ischemic heart disease. Platelet hyperactivity is associated with stent thrombosis so this patients need antiplatelet therapy. Such therapy usually includes clopidogrel and aspirin. It might be useful to assess total procoagulant activity using a global coagulation test in patients on dual antiplatelet therapy (DAPT)Aims:To estimate the plasma coagulation potential level in patients with a different response to antiplatelet therapy.Methods:This research included 46 patients after the successful coronary stent placement. All of them were given 75 mg clopidogrel and 150 mg aspirin per day. The antiplatelet therapy efficiency was assessed with light transmission aggregometry performed with a Hronolog 4‐channel platelet aggregometer using the adenosine diphosphate (ADP, 5 μM) and collagen (2 μg/ml) as agonists. Maximal platelet aggregation (MA, %) was determined. Thrombin generation was measured in platelet‐rich plasma by Calibrated Automated Thrombinogram Assay method (CAT) using reagent containing recombinant tissue factor (Trombinoscope BV, The Netherlands) as a trigger. Following parameters were derived: endogenous thrombin potential (ETP, nM‐min), peak thrombin (Peak, nM), and rate of thrombin generation (R, nM/min). STATISTICA 6.0 package was used in data analysis. Results are presented as median with 95% confidence intervals, p < 0.05 was considered statistically significant.Results:The results of aggregometry are presented in table. 30 (65%) patients (group №1) had a significant decrease in aggregation response only to collagen compared with controls, the response to ADP was not considerably lower. 16 (35%) patients (group №2) had a significant decrease in aggregation response to both collagen and ADP. The results of platelet aggregation suggest that the patients in the group №2 were more sensitive to antiplatelet therapy. There was a significant decrease in ETP, Peak and R in the group №2 compared with group №1 (ETP:Мe‐1636,5, CI: 1168,6‐1919,9 vs. Me‐1785,0, CI: 1415,7‐2576,2, p < 0,05; Peak: Me‐112,8, CI: 72,3‐138,9 vs. Me‐134,7, CI: 80,6‐180,2, p < 0,001; R: Me‐9,2, CI: 4,1‐13,3 vs. Me‐ 12,4, CI: 4,5‐21,2, p < 0,01).Summary/Conclusion: Patients with a better sensitivity to DAPT had lower plasma coagulation potential. It might be associated with the lower risk of stent thrombosis in patients with good response to DAPT.. Individual assessment of plasma coagulation potential using thrombin generation test could be useful for prediction of the thrombosis risk in the stent in patients after PCI. More research is clearly needed.image
PB2421 INVESTIGATION OF FIBRIN MONOMER AND VON WILLEBRAND FACTOR LEVELS IN CHILDREN AFTER CARDIAC SURGERY WITH CARDIOPULMONARY BYPASS
Authors: Lastovka V. , Gordeeva O. 
Published: June 2019

Background:Cardiopulmonary bypass (CPB) is associated with thrombotic complications. Occurrence of thrombosis after CPB is 11% which takes the third place between CPB‐associated complications. Current data demonstrates the impotance of researching of changes in haemostatic system in paediatric patients after CPB. Provided below data is an intermediate result of our research.Aims:Evaluate the dynamics of vWF and FM in children undergoing cardiac surgery.Methods:39 patients in age up to 11 months 29 days (median age ‐ 5,5 months, youngest age – 2 days after birth, oldest – 11 months 29 days), who underwent cardiac surgery with CPB to treat congenital heart diseases, were enrolled in this study. All patients were divided into two groups: 1st‐without thrombosis, 2nd‐with thrombotic complications. Von Willebrand factor and Fibrin‐monomer (FM) plasma levels were assessed in three points: before surgery, 24‐hours after surgery and 72‐hours after surgery. Thrombotic cases were proved by doppler ultrasound or MRI.Results:Thrombotic complications were diagnosed in 7 children (18%). Between all thrombotic complications ischemic strokes were diagnosed in 57% (4 cases), arterial thrombosis in 28 % (2 cases), intracardiac thrombus in 14% (1 case). In group with thrombotic complications FM‐mean values in points 1, 2 and 3 respectively were 78,33; 37,15 and 79,44 mcg/ml, meanwhile in group without thrombosis ‐ 8,21, 13,78 и 8,72 mcg/ml. PC‐mean values in 1st – group were‐ 104, 292 and 170%, in the 2‐nd group 192, 362 and 301% respectively in the points 1,2 and 3. Statistically significant differences between groups in 3d point (p < 0,05) and correlation between vWF and FM (r = 0,92, p < 0.05) were detected.Summary/Conclusion:CPB causes hypercoagulation with increasing of vWF and FM levels.Moreover, assotiated with high risk of thrombotic complications at the first 3 days after cardiac surgery. Further studies to investigate prognostic value of FM and vWF as markers of thrombotic complications and possibility of prevention and treatment of these complication in children undergoing cardiac surgery with CPB.
PB2422 INHERITED THROMBOPHILIA BY WOMEN WITH THROMBOSIS AND REPRODUCTIVE FAILURES IN ANAMNESIS
Authors: Kiseleva A. , Butina E. , Poponina E. , Paramonov I. 
Published: June 2019

Background:Thrombosis or thrombotic syndrome is a pathological condition caused by the formation of blood clots in the arteries, veins and cavities of a heart ‐ blood clots. As known. pregnancy is a period of increased risk of thrombotic complications due to hypercoagulation, venous stasis and damage to the vessel wall (Virchow triad). Hereditary predisposition to thrombophilia is a risk factor for thrombosis during pregnancy, affecting its course.Aims:To assess the distribution of polymorphic genes associated with thrombophilia and prothrombotic conditions (F2, F5, F7, F13, FGB, ITGA2, ITGB3, PAI‐1) by women with thrombosis and reproductive failures in anamnesis.Methods:Typing of thrombophilia and prothrombotic conditions genes was carried out by polymerase chain reaction in real time on the detecting amplifier DT‐96 (DNA Technology, Russia). The criterion χ2 was used for statistical data processing. Polymorphism of genes was studied in 123 women: 39 ‐ women with thrombosis of the upper and lower extremities, pulmonary embolism and reproductive failures (from 1 to 5 missed abortions), 84 ‐ healthy women without pathology of pregnancy (control group).Results:The following frequency of occurrence of polymorphic alleles: F2, F5, F7, F13, FGB, ITGA2, ITGB3, PAI‐1 in the studied group and the control group was established.The presence of ”non‐mutant“ alleles: F2 ‐ 92.3% and 98.8% (p = 0.059); F5 ‐ 94.9% and 95.2% (p = 0.931); F7 ‐ 97.4% and 73.8% (p = 0.002); F13 ‐ 59.0% and 50.0% (p = 0.354); FGB ‐ 53.8% and 48.8% (p = 0.604); ITGA2 ‐ 51.3 and 44.6% (p = 0.489); ITGB3 ‐ 61.5% and 82.1% (p = 0.014); PAI‐1 ‐ 15.4% and 16.7% (p = 0.885).The presence of ”mutant“ heterozygous alleles: F2 ‐ 7.7% and 1.2% (p = 0.059); F5 ‐ 5.1% and 3.6% (p = 0.685); F7 ‐ 2.6% and 25.0% (p = 0.003); F13 ‐ 28.2% and 41.7% (p = 0.152); FGB ‐ 43.6% and 44.0% (p = 0.963); ITGA2 ‐ 38.5 and 47.0% (p = 0.372); ITGB3 ‐ 33.3% and 16.7% (p = 0.038); PAI‐1 ‐ 46.2% and 52.4% (p = 0.52).Based on the results of the study, it was established that the T/C polymorphism of the ITGB3 gene is more common by women with thrombosis and reproductive failure, and T/T polymorphism ‐ by women from the control group. The heterozygous form of the F7 gene was less frequently detected by women from the studied group than in the control group (p <0.05). The non‐mutant form of the F7 gene was found significantly more often in women from the studied group than from the control group (p <0.05).Summary/Conclusion:The presence of the T/C polymorphism of the ITGB3 gene and the absence of the G/A polymorphism of the F7 gene are associated with reproductive failure by women with thrombosis in anamnesis.
PB2446 A STUDY OF TRANSFUSION RELATED ADVERSE EVENTS IN FAYOUM UNIVERSITY HOSPITALS, EGYPT: HEMOVIGILENCE INITIATIVE
Authors: Ezzat G. , Abdelrazik A. , Elshafei S. , Abbas M. Mansour , El Hassan S. Abou 
Published: June 2019

Background:Hemovigilance is an adverse reaction monitoring system to improve the safety and quality of blood transfusion. Identification of the adverse reactions and their risk factors would help in taking appropriate steps to reduce their incidence.Aims:To identify the transfusion related adverse events and their rates for benchmarking purposes in Fayoum University Hospitals (FUH) for implementation of the hemovigilance system.Methods:The study was conducted in FUH, from May 2016 to April 2017. All patients receiving transfusion therapy and all blood donors were included. All the adverse events related to transfusion of blood and blood products were recorded.Results:A total of 8687 blood components were issued to 3292 recipients from 8300 donors. Ninety Nine donor reactions were reported, with a rate of (1.19%), vasovagal attacks being the most common complication (91.8 %). Thirty Six (1.09 %) recipients adverse reactions were reported to the blood bank. The most common reaction observed in recipients was allergic reaction (47.2%) followed by febrile non‐ hemolytic transfusion reaction (36.1%).Summary/Conclusion:Data related to adverse transfusion reactions in developing countries for benchmarking purposes are rare in literature. The use of transfusion related adverse reactions to build the hemovigilance system facilitates its development and allows areas requiring improvement to be tracked and identified.
PB2449 RED CELL ALLOIMMUNIZATION IN MULTITRANSFUSED ONCOLOGY PATIENTS
Authors: Mahesar A. 
Published: June 2019

Background:Blood transfusion is common nowadays in management of various diseases especially oncology patients. Blood grouping and cross matching is done in routine practice before transfusion against Major blood groups (i.e., ABO & Rh blood groups). Alloantibodies can be produced against Minor blood groups. These Alloantibodies can cause acute or delayed hemolytic transfusion reactions. These hemolytic reactions cause anemia as well as other related complications. Oncology patients can produce alloantibodies because of multiple transfusions from different donors. Pre transfusion antibody screening and identification in oncology patients can help them from unnecessary time required for identification of proper cross matched donor. Alloantibody identification can prevent the patients from complications related to allogenic blood transfusion. This study is designed to determine the frequency of alloantibodies in multitransfused oncology in Pakistani population.Aims:The objectives of the study is to determine the frequency and types of RBC alloimmunization in multitransfused oncology patients at a teaching hospital in Lahore, Pakistan.Methods:A total of 145 cases fulfilling the inclusion and exclusion were selected by non probability purposive sampling. Blood sample was taken and Plasma was separated with centrifugation. Routine blood testing for ABO and Rh grouping was done. The patient's plasma that was positive on initial screening for alloimmunization was further tested with Antibody Identification Panel.Results:The frequency of Red cell alloimmunization is 5.5% (8/145). The alloantibodies for Rh were 6 (4.13%) and one patient positive each for Kell and Duffy having frequency of 0.68 % each. Alloantibodies are found to be Anti‐D four (4) and each one (1) Anti‐ C, Anti‐E, Anti‐K and Anti FyaSummary/Conclusion:Allo‐antibodies are produced against Minor blood groups in multitransfused oncology patients. Alloantibodies against Rh system were most frequent.
PB2450 DYE‐LIGAND CHROMATOGRAPHY AS AN ALTERNATIVE METHOD FOR OBTAINING THE FACTORS VIII/VON WILLEBRAND COMPLEX
Authors: Shurko N. , Danysh T. , Novak V. 
Published: June 2019

Background:A new dye‐ligand chromatographic procedure has been developed to produce a highly purified factors VIII/von Willebrand complex (FVIII/vWF) concentrate from plasma cryoprecipitate.Factor VIII (FVIII) and von Willebrand factor (vWF) circulate in the plasma as a non‐covalent protein complex FVIII/vWF. The levels of factor VIII in vivo are coordinately regulated with levels of vWF.Willebrand's disease together with hemophilia A are the most common heredity diseases of the hemostasis system. The concentrate of FVIII/vWF complex is used for the treatment of these diseases.The main method of obtaining of plasma concentrates of FVIII/vWF complex remains the method of ion‐exchange chromatography. With the use of ion exchange chromatography it is not possible to maintain the initial ratio because the elution of these two factors from the ion exchanger occurs at different ionic strength values. That is, the ion‐exchange chromatography method does not allow for the absolute yield of vWF together with FVIII.Aims:investigate the ratio of FVIII/vWF complex at various stages of the technology opurification of antihemophilic concentrate.Methods:Cryoprecipitation, ion‐exchange chromatography on DEAE‐Sepharose FAST FLOW; dye‐ligand affinity chromatography.Results:We development of a new method to obtain purified preparation of the complex FVIII/vWF, that inclused cryoprecipitation, ion‐exchange chromatography on DEAE‐Sepharose FAST FLOW and dye‐ligand affinity chromatography.The degree purification of FVIII was 95.3 times on the stage of ion‐exchange chromatography. The ratio FVIII/vWF has changed approximately twice (from 0.78 to 1.75) (Table 1). We used a buffer NaCl with an ionic strength of 0.3 M for co‐elution of FVIII and vWF. However, part of the vWF was eluted with the first fractions (0.15 M NaCl).The research demonstrated that the process of purification of the FVIII and vWF with dye‐ligand is due to the phenomenon of the negative affinity adsorption. This property we used in the technology of obtaining of FVIII/vWF complex. This property provides the output level of the relationship this provides the output level of the relationship (Table 1).Summary/Conclusion: It was established that at the stage of dye‐ligand chromatography, the loss from the initial activity of FVIII is practically absent and the original ratio of FVIII/vWF is saved.image