bluuebunny/crossref_metadata_2025_split huggingface dataset:
PB2318 DOUBLE HETEROZYGOSITY S/C AT HEMATOLOGY DEPARTMENT ANNABA HOSPITAL ‐ALGERIA
Authors: Djenouni A. , Grifi F. 
Published: June 2019

Background:Major sickle cell disorders account for 95% of the hemoglobinopathies supported at our service; double S/C heterozygosity is infrequent and represents only 6,4%.Aims:The objective of our work is to determine the prevalence and characteristics of acute and chronic complications that occurred in our patients during their follow‐up.Methods:This is a retrospective, descriptive study of 99 patients with double S / C heterozygosity and followed regularly since recruitment until December 2017.The patients benefit from a regular follow‐up, every 04 months with an annual report aimed at detecting the chronic complications.Results:The average age of patients at the time of diagnosis is 21 years, with extremes of [02‐49 years].The current average age of the cohort is 33 years, with extremes of [13‐66 years]The majority of our patients come from Algerian‐Tunisian border area (79%).During this monitoring, several variously associated complications were collected:The acute complications are dominated by vaso‐occlusive crises in various locations, with exceptionnellent‐required hospitalization;During follow‐up, 32.09% of our patients developed various chronic complications:Summary/Conclusion:Despite of an acute symptomatology low noise from the S/C, chronic complications are frequent, imposing the implementation of a multidisciplinary management strategy at our level.image
PB2359 THE ROLE OF AUTOLOGOUS STEM CELL TRANSPLANTATION IN INCREASING THE EFFICIENCY OF TREATMENT IN PATIENTS WITH MULTIPLE MYELOMA
Authors: Garifullin A. , Voloshin S. , Chubukina Z. , Kuzyaeva A. , Sсhmidt A. , Bessmeltsev S. 
Published: June 2019

Background:Multiple myeloma remains an incurable disease despite the emergence of new drugs. The search of optimal therapeutic strategy to increase life expectancy and remission in patients with this disease is continues. This is facilitated to the achievement of the maximum possible response and eradication of residual tumor load. Existing recommendations for the treatment of multiple myeloma approve the use of autologous stem cell transplantation (autoSCT) to improve disease control.Aims:To determine the impact of autologous stem cell transplantation on efficiency of treatment and tumor load in patients with multiple myeloma.Methods:The study included 89 patients with MM (median age 58 years, male/female – 1.12:1). The induction therapy with Bortezomib‐based regimens (VD, CVD, VMP, PAD) was used in 62/89 (69.6%) patients, Immunomodulator‐based regimens (Thal + D, RD, VRD, PomD) – in 22/89 (24.8%), chemotherapy – in 4/89 (5.6%). High dose therapy (Mel200) and autoSCT is carried out in 64/89 (71.9%) patients. The efficiency of therapy was evaluated according to IMWG criteria. Tumor load was determined by multicolor flow cytometry (5‐colors): 79 patients – after 4‐6 courses of induction therapy, 39 patients – after autoSCT; 29 patients – tumor load was performed in dynamics (before and after autoSCT).Results:The sex, age, the variant induction antimyeloma therapy did not affect on response rate and tumor load. CR was achieved in 28/89 (31.4%) before AutoSCT and 50/89 (56.2%) after AutoSCT (p > .05). However, high dose therapy (Mel200) and AutoSCT has increased the possibility of achieving complete response in dynamics: before AutoSCT in 5/29 (17.2%), after AutoSCT – in 13/29 (44.8%) (p<.05).
Tumor load after 4‐6 courses of induction therapy (n = 79) was 0.28 ± 0.34 for CR + sCR, 1.65 ± 2.66 for VGPR, 2.10 ± 2.53 for PR.Tumor load after HDT (Mel200) and AutoSCT (n = 39) was 0.06 ± 0.07 for CR + sCR, 0.15 ± 0.46 for VGPR, 0.17 ± 0.22 for PR.The effect of autoSCT on PFS in 39 patients. The median PFS was 97 months in the group “tumor load after autoSCT <0.01%”, 70 months – in the group “tumor load without autoSCT <0.01%”, 50 months – in the group “tumor load after autoSCT >0.01%” and 25 months – in the group “tumor load without autoSCT >0.01%”(p = .006).
Examination of tumor load in the dynamics in 29 patients showed that autoSCT reduces the level of tumor load (before autoSCT = 2.27 ± 0.20, after autoSCT – 0.14 ± 0.01). Tumor load <0.01% after autoSCT was detected more often than before autoSCT (14/29 and 5/29 patients, respectively)(p<.05).
Summary/Conclusion:AutoSCT increase the efficiency of therapy, reduce tumor load and increase the duration of progression free survival in patients with multiple myeloma.image
PB2369 THE METHOD OF STORAGE OF AUTOLOGOUS PERIPHERAL BLOOD HEMATOPOIETIC STEM CELLS
Authors: Rysev G. , Kuzyaeva A. , Voloshin S. , Shuvaev V. , Schmidt A. , Garifullin A. , Kuvshinov A. , Mikhaleva M. , Linnikov S. 
Published: June 2019

Background:High‐dose chemotherapy (HDCT) followed by autologous transplantation of hematopoietic stem cells (auto‐HSCT) is an important component of treatment in multiple myeloma (MM). There is a standard method of controlled cryopreservation of HSC suspension. We found that the storage of native HSC suspension with temperature fluctuations from +3 °C to +5 °C for 5 days does not significantly affect the content of CD34+ cells in the product, the index 7AAD– (7‐AAD (7‐aminoactinomycin – D) is a fluorescent marker that penetrates damaged cell membranes and binds to double‐stranded DNA. Through 7AAD does not penetrate intact membranes, so living cells are not stained 7AAD with flow cytometry), and colony‐forming ability (CFA) of HSC, as well as the recovery time of hematopoiesis in MM patients after auto‐HSCT.Aims:To evaluate the effectiveness and safety of the method of storage of non‐cryopreserved peripheral blood stem cells.Methods:26 patients with MM were included in this study(male/female ratio 1.36:1). All the patients get standard immunochemotherapy programs and were in remission at the time of auto‐HSCT. Patients were divided into two groups depending on the method of stem cell storage: group 1 – non‐cryopreserved (n = 10), group 2 – cryopreserved (standard) (n = 16). An effectivity and safety were evaluated in such parameters as the number of CD34+ and 7AAD‐ cells, CFA after apheresis and before reinfusion of HSC. Also we evaluated the number of platelet concentrate transfusions, the timing of engraftment of granulocytic and megakaryocytic blood sprouts, the length of hospital stay after auto‐HSCT, the number of complications.Results:The results are presented in the comparison table of evaluated parameters.Summary/Conclusion:Thus, the proposed method of storage of HSC is not inferior to the traditional method with cryopreservation on such parameters as CD34+, 7AAD–, CFA, the number of platelet concentrate transfusions, terms of hematopoiesis restoration, length of hospital stay after HSCT, the number of complications.image
PB2380 PROGNOSTIC EVALUATION OF MULTIPLE MYELOMA PATIENTS SUBMITTED TO UP‐FRONT AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION FROM A SINGLE BRAZILIAN CENTER
Authors: Vivas Y. , Hungria V. , Santos K. , Chaoubah A. , Bustamante‐Teixeira M. , Vivas L. , Costa L. , Neto A. 
Published: June 2019

Background:Although autologous hematopoietic stem cell transplantation (AHSCT) has been performed in the context of multiple myeloma since the end of the 90's, response to therapy and patient survival are not homogeneous between different patients.Aims:In order to understand the factors involved in therapy response degree and survival on AHSCT performed as first‐line therapy, we conducted a retrospective analysis of myeloma multiple patients from asingle Brazilian center.Methods:All patients treated with AHSCT between May 2004 and October 2017were evaluated, excluding patients with prior transplant therapies or insufficient records data.Variables related to the International Staging System (ISS), Durie‐Salmon staging, clinical presentation at diagnosis, regimen used for induction, number of treatment cycles, induction and transplant treatment response assessmentwere studied.Event free survival (EFS) and overall survival (OS) were calculated and associated to analyzed factors.Results:Altogether, 137 patients were included in the study, with an average age of 52 years, 59,1% of them were male and 86,9% nonblack. Fifty two patients (38%) died, 6 (4,4%) of these during AHSCT. OS was 63,8% and EFS was 25,1% in 5 years.OS univariate analysis, as confirmed by multivariate analysis, was smaller in patients with anemia at diagnosis (p = 0,006).EFS, submitted to multivariate analysis, was smaller in patients treated with more than one line of induction chemotherapy.A positive response to induction therapy was the main factor related to a very good partial response and complete response to AHSCT (p = 0,001).Summary/Conclusion:Notwithstanding the great gap in knowledge over the successful myeloma multiple AHSCT factors, our data is similar to the ones found in literature, with anemia as an important independent prognostic factor to OS.
PB2391 MYOCARDIAL CONTRACTILE FRACTION BY MAGNETIC RESONANCE AND RISK OF CARDIAC COMPLICATIONS IN THALASSEMIA MAJOR
Authors: Meloni A. , Pistoia L. , Rosso R. , Bitti P. P. , Caini M. , Carollo A. , Armari S. , Gerardi C. , Roberti M. G. , Fiorenza M. F. , Sarli R. , Renne S. , Positano V. , Pepe A. 
Published: June 2019

Background:The analysis of cine cardiovascular magnetic resonance (CMR) images allows the quantification of the myocardial contraction fraction (MCF), a volumetric analog of myocardial deforming, that is calculated by dividing left ventricular (LV) stroke volume by LV myocardial volume.Aims:We verified the association of this new marker with myocardial iron overload and cardiac events (heart failure, arrhythmias and pulmonary hypertension) in thalassemia major (TM) patients.Methods:We considered 1492 TM patients (771 females, 31.06 ± 8.95 yrs) enrolled in the MIOT network. Myocardial iron overload (MIO) was quantified by the multislice multiecho T2∗ technique and biventricular morphological and functional parameters by cine sequences.Results:MCF values were significantly lower in males (0.94 ± 0.22 vs 1.01 ± 0.22; P < 0.0001) and decreased with increasing age (R = −0.129 P < 0.0001).Patients with MIO (global heart T2∗ < 20 ms; N = 400) showed significant lower MCF values than patients without MIO (0.91 ± 0.20 vs 1.00 ± 0.22; P < 0.0001).The patient population was randomly divided in two groups of equal size (N = 746).In the first group 99 patients had at least one cardiac complication and at receiver‐operating characteristic (ROC) curve analysis a MCF ≤ 0.87 was the best predictor of cardiac complications (area under the curve 0.659, 95% CI 0.64 to 0.69; sensitivity 60%, specificity 67%).In order to validate the result, the obtained value was tested in the remaining 746 patients (group 2). One hundred and thirty‐five patients had a cardiac complication and patients with a MCF ≤ 0.87 were more likely to have cardiac complications (odds ratio – OR = 2.73, 95%CI = 1.86‐4.01; P < 0.0001), also adjusting for cardiac iron (see Figure).Summary/Conclusion:Contractile function is reduced in patients with MIO. A MCF ≤0.87 can help to identify patients with a significant higher risk of adverse cardiovascular events, independently by the presence of MIO.image
PB2399 BETA THALASSEMIA MINOR PATIENTS: NOT TIRED, BUT DEPRESSED AND ANXIOUS
Authors: Eren R. , Karışmaz A. , Aslan C. , Doğu M. H. , Altındal S. , Yokuş O. , Suyanı E. 
Published: June 2019

Background:The quality of life has been influenced unfavourably and the prevalance of either depression or anxiety has been increased in beta thalassemia major (BTM) and beta thalassemia intermedia (BTI) patients. Yet, the studies including the quality of life in beta thalassemia minor (BTm) subjects are limited and just the depression was demonstrated to be frequent in individuals with BTm, compared to the healthy population. Likewise, the data regarding the presence of fatique and anxiety in adult BTm subjects is insufficientAims:We aimed to investigate whether the severity of fatigue and the incidences of depression and anxiety of patients with BTm are different than healthy individuals using Fatigue Severity Scale (FSS) and Hospital Anxiety and Depression Scale (HADS).Methods:Seventy‐seven BTm subjects were included in this study. The individuals (n = 38) using any medications, alcohol or tobacco; and having any chronic diseases and sleeping disturbances, were excluded. The remaining 39 BTm subjects were enrolled in the study. They had also normal biochemical parameters, thyroid function tests and C‐reactive protein (CRP). Twenty‐five healthy control subjects who were matched with age, sex, marital status, educational status and body mass index (BMI), were also included.Results:The BTm and the control groups were comparable in terms of gender, age, BMI, educational status and marital status (p = 0.368, 0.755, 0.851, 0.785, 0.709 respectively) (Table 1). Fatigue Severity Scale score was ≥4 in 23 (59.0%) BTm subjects and in 15 (60%) control subjects (p = 1.0). HADS anxiety score was ≥10 in 20 (51.3%) BTm subjects and in 5 (20.0%) control subjects (p = 0.018) and HADS depression score was ≥ 7 in 20 (51.3%) BTm subjects and 6 (24.0%) healthy control subjects (p = 0.039). There was no correlation of hemoglobin with FSS score (p = 0.526, r = ‐0.105), HADS anxiety score (p = 0.703, r = ‐0.063) or HADS depression score (p = 0.718, r = ‐0.06) in BTm group (Table 2).Summary/Conclusion:We found that both depression and anxiety were higher in BTm patients than healthy individuals, but this difference was not feasible for fatigue.imageimage
PB2411 DETECTION OF PATERNALLY INHERITED MUTATION (IVS‐II‐I G → A) OF THALASSEMIA IN MATERNAL PLASMA BY USING DHPLC METHODS
Authors: Zafari M. 
Published: June 2019

Background:β‐ Thalassemia is the most common inheritable disease in the world. About 3% of populations (150 million persons) are β‐thalassemi carriers’, and they run 25% risk of having Thalassemia major baby in each pregnancy. Prenatal diagnosis (PND) if offered in many country for prevent Thalassemia, and it is a part of national prevention program by invasive methods, such as; chorionic villus sampling (CVS) at 11‐14 weeks gestation or amniocentesis (AC) from 15 weeks gestation.Aims:the purpose of this study was detection of paternally inherited mutation (IVS‐II‐I G → A) of Thalassemia in maternal plasma by using DHPLC methods with its assessments via sensitivity and specificity determinations for prenatal diagnosis of thalassemia major.Methods:Subjects included Iranian pregnant women who at risk of having fetuses with β‐thalassemia major. We focused exclusively on samples in which the father was carrier for IVS II‐I G → A mutation and the mother had been genotyped to carry another β‐globin gene mutation. 10 ml peripheral blood sample were obtained from 50 pregnant women. The CVS sample was obtained Trans abdominal puncture with a 23‐gauge needle under ultrasound guidance. The samples were analyzed by reveres dot blot analysis. We were masked as to the identity the samples with numerical coding system, so, these were examined in a blinded manner. Circulatory DNA was extracted from 200 μl of peripheral blood plasma with QIAamp DNA mini and blood kit (QIAGEN‐USA) according to the manufacture s instructions. The genomic DNA concentration was measured by NanoDrpe system (ND1000, USA) and electrophoresis. They stored at ‐20 c until used. At firs beta‐Globin gene fragments containing IVSII‐1(G > A) mutation was amplified with HRM real time PCR, also by using specific primers. DHPLC was performed on the WAVW fragment analysis system (Transgenomic Inc, USA) to detect the variants of beta globins’ gene mutation of Thalassemia. In order to generate the hetro& homoduplexes, we mixed an equal amount of HRM Real time PCR product of wild type and patients.Results:the sensivity, specifity of this method was 40. 62 and 61.12; also the PPV and NPV was 65% and 36.67% respectively.Summary/Conclusion:the specifity and NPV of DHPLC method is higher than specifity and PPV.
PB2415 HYDROXYUREA AN EFFECTIVE THERAPY TO AVOID SPLENECTOMY IN SICKLE CELL DISEASE AND THALASSEMIA: 15 YEARS EXPERIENCE IN SAUDI ARABIA
Authors: Jaouni S. Al 
Published: June 2019

Background:Background and Purpose: Spleen plays an important part in the immune system which helps the body fight infection and have a major role in children. Splenectomy have been decline in hemoglobin disorders in recent years.Aims:To assess the role of hydroxyurea if can eliminate the risk of splenectomy in sickle cell disease (SCD) and thalassemia in our medical center.Methods:Total of 84 patients enrolled in the study between 2003 to 2018 at King Abdulaziz University Hospital, Faculty of Medicine, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia, 24 sickle cell disease (SCD), 28 thalassemia major (TM) were suboptimal transfusion dependents and hypersplenism, 22 thalassemia inter media (TI).Results:Risk of splenectomy have been avoided in 23/24 SCD, 27/28 in TM and 22/22 in TI.Summary/Conclusion:Hydroxyurea is an effective and safe therapy can eliminate risk of splenectomy in SCD and thalassemia. Recent studies and meta analysis showed that hydroxyurea is safe and non‐carcinogenic.
PB2419 PEPTIDE CORRECTION OF OSMOTIC RESISTANCE DISORDERS IN INFLAMMATION
Authors: Golubeva M. , Umarova B. , Bondarenko N. 
Published: June 2019

Background:The development of inflammation is accompanied by a change in the rheology of the blood ‐ an increase in viscosity, aggregation, a decrease in the osmotic resistance of red blood cells, which indicates a change in the structure of their membrane. This leads to a change in the functional properties of red blood cells and impaired microcirculation. The search for new anti‐inflammatory drugs, devoid of side effects is relevant. Promising may be endogenous regulatory peptides of the glyproline family, which have a prophylactic and therapeutic effect in various pathological conditions.Aims:The aim of this study was to investigate changes in the osmotic resistance of erythrocytes in the dynamics of experimental peritonitis with the introduction of the peptide Pro‐Gly‐Pro (PGP).Methods:A pro‐inflammatory agent was used to simulate experimental peritonitis ‐ 40% sodium thioglyaolate solution 4 g/kg, administered intraperitoneally. The erythrocyte osmotic resistance (ORE) was determined 30 minutes, 2 and 18 hours after the induction of inflammation. The evaluation of the wholesale electricity market was carried out to determine the degree of hemolysis in hypotonic solutions of sodium chloride. The light transmittance was recorded on a photoelectrocalorimeter at a wavelength of 580 nm. All experiments were performed in accordance with the ethical principles and regulatory documents recommended by the ESF and the declaration of a humane attitude towards animals.Results:The development of inflammation is accompanied by a decrease in the osmotic resistance of erythrocytes. Statistically significant differences in the wholesale electricity market from control were observed 2 hours after the administration of the thioglycolate. The introduction of PGP peptide at a dose of 3.7 μm/kg before the induction of inflammation reduced pathological changes in the ORE. The percentage of hemolyzed cells decreased, the differences were significant in solutions with salt content of 0.4% and 0.3%. The peptide, introduced against the background of developed inflammation (1 hour and 45 minutes after the introduction of thioglycolate) did not have a corrective effect on the ORE.Summary/Conclusion:The results indicate the ability of PGP to exert a preventive protective effect on the erythrocyte membrane, preventing a decrease in the ORE.
PB2430 CHANGES OF TPA AND PAI‐1 IN PATIENTS WITH CORONARY ARTERY DISEASE.
Authors: Tyravska Y. , Lizogub V. , Vovk T. , Savchuk O. 
Published: June 2019

Background:Impaired fibrinolysis, with imbalance between the key enzyme of plasminogen to plasmin activation, tissue plasminogen activator (tPA), and its main endogenous inhibitor, plasminogen activator inhibitor‐1 (PAI‐1), is shown to play a crucial role in thrombi formation which is an essential process in acute coronary syndrome (ACS). Most studies exist regarding myocardial infarction (MI). Limited investigations include patients with unstable angina (UA). However, no data is found concerning types of UA, namely, new‐onset angina (NUA) and progressive angina (PUA). Consequently, we investigated concentrations of tPA, PAI‐1 and tPA/PAI‐1 ratio in patients with above‐mentioned types of coronary artery disease (CAD).Aims:to compare the concentration of tPA and PAI‐1, tPA/PAI‐1 ratio in patients with different types of CAD (including NUA and PUA).Methods:In this cross‐sectional study, we collected blood samples in 94 patients with CAD ‐ 10 patients with MI, 57 patients with UA (38 patients with PUA and 19 patients with NUA), 27 patients with chronic coronary disease (stable angina (SA)). Concentrations of tPA and PAI‐1 were determined by standart method ELISA. Ratio tPA/PAI‐1 was calculated. There were no significant difference between groups of patients with respect of age, gender and body mass index.Results:In our study, we observed significant difference in tPA concentration (p = 0,0001) and tPA/PAI‐1 ratio (p = 0,004). However, PAI‐1 did not differ significantly in groups (p = 0,326), though it tended to be higher in patients with ACS in comparison with SA (0,392 (0,324‐0,472) U/ml vs 0,321 (0,284‐0,456) U/ml, p = 0,100). Unexpectedly, we identified the highest PAI‐1 concentration in UA group.Both tPA and tPA/PAI‐1 were significantly lower in ACS in contrast to SA (0,226 (0,173‐0,233) U/ml and 0,514 (0,432‐0,625) vs 0,234 (0,222‐0,295) U/ml and 0,619 (0,505‐0,997), p = 0,016 and p = 0,039, respectively).We detected significantly lower tPA concentration and tPA/PAI‐1 in patients with UA in comparison with SA (0,219 (0,173‐0,230) U/ml vs 0,234 (0,222‐0,256) U/ml, p = 0,002, and 0,485 (0,427‐0,597) vs 0,619 (0,505‐0,811), p = 0,021, respectively).Finally, we revealed significant difference in tPA concentration and tPA/PAI‐1 between NUA and PUA (p = 0,0001 and p = 0,001, respectively). Moreover, there were no difference between tPA concentration and tPA/PAI‐1 in MI and NUA (p = 0,071 and p = 0,756, respectively) as well as SA and PUA (p = 0,153 and p = 0,188, respectively). Additionally, the lowest tPA/PAI‐1 ratio was in NUA group.Summary/Conclusion:We have established imbalance between concentrations of tPA and PAI‐1 depending on CAD type. Thus, the highest thrombotic activity was detected in UA group while the lowest fibrinolytic activity was in NUA group. These findings call for further studies within types of UA as well as MI.image