bluuebunny/crossref_metadata_2025_split huggingface dataset:
PB2358 THE IMPACT OF THE NUMBER OF CD34 + CELLS TRANSPLANTED IN ENGRAFTMENT AFTER AUTOLOGOUS TRANSPLANTATION OF HEMATOPOIETIC PROGENITORS
Authors: Luís D. Andrade , Guedes C. , Roque A. , Esesumaga A. , Borges C. , Geraldes C. , Tomaz J. 
Published: June 2019

Background:Despite the emergence of new therapeutic options, autologous hematopoietic stem‐cell transplantation (AHCT) is still one of the pillars in the treatment of patients with multiple myeloma (MM), especially in younger and fit patients. However, the ideal number of transplanted CD34 + cells to maximize post‐transplant results is still under discussion.Aims:Verify the corelation between the number of cell CD34+ infused and the outcomes in neutrophils and platelets recovery and transfusional requirements.Methods:Prospective analysis of 243 autologous transplants in patients with multiple myeloma for 10 years from January 2007 to December 2017,in a single institution.For this study, where analysed: the myeloma subtype, the age of diagnosis, the time from diagnosis to transplantation, the conditioning used, the number of days of hospitalization, number of cells infused, the number of days of engraftment, number of days with neutrophils< 0,5 × 109/L, number of days with platelets< 20 × 109, number of transfused platelets pools, number of transfused red blood cells (RBC).The statistical treatment of the data was done in SPSS version 24.Results:In this sample 217 patients were analysed, of whom 79 were females (36,4%) and 138 were males (64%). The medium age of diagnosis was 54.28 years (27 – 77 years) and of transplantation was 58.42 years (28 – 72 years). The most frequent subtype of MM was IgG kappa(45,7%), IgA kappaand IgG lambda(both with 13,2%). The median time from diagnosis to transplant was 533 days. The conditioning regimen used in the majority of the patients was melphalan 200 (97%), and the mean time of hospitalization was 19 days (12 – 77). The number of cells CD34+infused ranged from 1.40 to 13 × 106cells/kg, with a average of 3.49 cells/kg.In this study, no correlation was verified between the number of cells CD34+infused and the number of days with neutrophils< 0,5 × 109/Lor with the transfusion need for RBC. The average number of days of engraftment was 13 days (9 – 28), and there is also no correlation between the number of cells CD34+infused. However, there is an inversely proportional and statistically significant correlation (p < 0.01 and p < 0.05, respectively) related to the number of days with platelets< 20 × 109/L as well as to the number of transfused platelets pools.Summary/Conclusion:An increased number of transplanted CD34+cells was shown to have a significant impact in time until recovery of the number of platelets, and was associated with lower transfusion dependencepost‐AHCT. The remaining variables evaluated did not show to be dependent on the number of transplanted cells.
PB2363 AUTOLOGOUS STEM CELL TRANSPLANTATION IN MULTIPLE MYELOMA: A SINGLE INSTITUTE OUTCOME ANALYSIS
Authors: Keat T. Sui , Soon T. Ching , Sim G. Ai , Keat C. Teng , Kien C. Su 
Published: June 2019

Background:High dose chemotherapy followed by autologous stem cell transplantation (ASCT) is now considerd as standard therapy for young patients (< 65 years) with multiple myeloma (MM).Aims:Although myeloma is the 3rd commonest hematological malignancy, only a few centres in Malaysia offer ASCT service for this group of patients. We hereby, share our 9 year's experience in Penang General Hospital.Methods:Clinical data of patients with MM who were transplanted in Penang General Hospital between 2008 to 2017 were collected retrospectively and analysed using SPSS IBM version 22.0. The patients were followed up from the time of transplant until 28 February 2018. The variables assesed included demographics, MM subtypes, remission status, induction chemotherapy pre‐transplant and maintenance therapy post‐transplant.Results:A total of 41 patients (18 males, 23 females) were evaluated. The median age at transplant was 54 years (ranged 33‐65 years). There were 19 Malays (46.3%), 15 Chinese (36.6%) and 7 Indian patients (17.1%). 73% had IgG Kappa subtype. 41.5% presented with ISS II, 34.1% ISS III and 24.4% ISS I. 61% of patients were transplanted with at least a very good partial response (VGPR) compared with 39% with partial response (PR). Majority of patients (75%) received Bortezomib based induction therapy prior to transplant. 58% patients received maintenance treatment post‐ASCT. The median follow‐up was 35 months (range 3‐103 months). The 5 years overall survival (OS) and progression‐free survival (PFS) was 70% and 44.3% respectively. There was no transplant related mortality. Median PFS of patients with ISS III was only 11 months compared with 53 months for ISS I‐II (p < 0.001). Patients who received maintenance had longer PFS (65 months versus 12 months, p = 0.032). Multivariate analysis revelaed ISS III as an independent risk factor for survival in our cohort (PFS hazard ratio (HR), 4.76;95% CI 1.81 to 12.49;p = 0.002). Other variables evaluated did not affect survival.Summary/Conclusion:ASCT improves survival in patients with multiple myeloma and is safe in patients below 65 years. ISS III is an independent risk factor of PFS.
PB2371 CAN NELARABINE RESCUE THE GRAFT AFTER ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION IN T‐ALL ?
Authors: Yuksel M. Kurt , Seval G. Cengiz , Bozdag S. Civriz , Toprak S. K. , Topcuoglu P. , Arslan O. , Muhit O. , Demirer T. , Akan H. , Beksac M. , İlhan O. , Gurman G. 
Published: June 2019

Background:Adult ALL is a rare disease, T‐ALL accounts for nearly %20 of all ALL and T‐ALL is more common in adults than children. The use of pediatric intensive chemotherapy regimens in AYAs and nelarabine for relapsed and refractory diseases has improved outcomes. Still, allogeneic SCT remains an important component of treatment. Nevertheless relapse is inevitable.Aims:Aim of this study is to evaluate the effect of nelarabine as salvage therapy after HSCT, in terms of disease control, GVHD, PFS and OS.Methods:Between 2014‐2018 Nine (F/M = 1/8; median age = 28 years (21‐45)) patients,from one center are evaluated retrospectively. None of them received nelarabine, before HSCT. Transplantation characteristics are given in table 1. Patients were treated at the time of relapse with nelarabine alone 1.5 g/M2/day on days 1, 3, 5 every 28 days.Results:Among the 9 patients 67% achieved hematologic remission during 8.2 months (range; 2.9‐19.2 mos) follow‐up. The features of nelarabine based salvage therapy are shown in table 2. The main toxicity was neurological and involved 22% of patients. At 1 year, disease‐ free and overall survival rates were 66.7% and 50% respectively (Figure 1). Only one patient (11%) received DLI and MRD was lost. On the other hand patients who had MRD (4 (44%)) and received nelarabine had a stable MRD disease those did not transform to overt leukemia.Summary/Conclusion:The OS at 1 year for salvage treatment with nelarabine at first and secondary relapse is 28%, which is lower than nelarabin usage after HSCT (50%). There is no data about interaction between immune reconstitution, relapse prevention and nelarabine after HSCT. Nelarabine is a well‐tolerated drug with minimal toxicity and can be considered for maintenance therapy post HSCT in both MRD positive, bulky and CNS disease. Even though it does not show graft versus tumor effect, it can just prevent T‐ALL transform to an aggressive leukemia.image
PB2382 FOLLOW UP POST ALLOGENIC STEM CELL TRANSPLANTATION IN MOROCCO: ABOUT 28 PATIENTS
Authors: Haidouri S. , Filali K. zine , ababou M. , mahtat E. , mikdame M. , doghmi K. 
Published: June 2019

Background:Allogenic hematpoetic stem cell transplantation is a cure for hematological malignancies and certain of immune system diseases,but is associated with high morbidity due to immunological reaction between donor and recipient:Graft versus host diseaseAims:repport our experience in management of post allograft complicationsMethods:this is a retrospective descriptive study,between 2007 and 2018,including 28patientsThe follow up was done in the clinical departement of hematology of Rabat,but allograft was performed in Percy military hospital in ParisResults:In this study, spread between 2007 and 2018, all patients included were sent to the Percy military hospital for an allograft of hematopoietic stem cells between 2007 and 2018, the median age was 32 years (15 years and 60 years), 55% were male, 17 patients were followed for acute leukemias including 09 myeloid type, 04 myeloproliferative neoplasms (01 CML, 03PMF), 03 patients for aplastic anemia, 01 MDS, 03 patients for lympho‐proliferative syndrome (01 Hodgkin's lymphoma, T lymphoma and multiple myeloma). The duration between diagnosis and bone marrow transplant was around 12 months (2 months and 6 years), 75% of patients were transplanted from a geno‐identical donor, 17% from a 10/10 pheno‐identical voluntary donor, while one patient received a haplo‐identical transplant and another a transplant of placental blood units. A peripheral stem cell graft was used in 75% of patients, several conditioning regimens were used, including: Busulfan / Endoxan in 30% (acute leukemias) Fludarabine‐Busulfan‐thymoglobulin in 28%. Anti‐GVHD prophylaxis was done by Methotrexate / ciclosporin in 58% of cases, ciclosporin alone in 22% and ciclosporin‐mycophenolate mofetil in 18% of cases. The immediate consequences of the graft were marked by the occurrence of bacterial infections in 71%, the reactivation of CMV in 9 patients, acute GVH was found in 38% of patients, cutaneous type with a good response to corticosteroids. During our follow‐up, 35% of patients showed signs of chronic GVH ‐with 35% of patients who had acute GVH. All types was present:cutaneous and oral (71%), pulmonary (58%), ocular (58%), hepatic (28%), muscular (28%), digestive and gynecologic GVHD. (14%), immunosuppressive treatments (ciclosporin) and corticosteroids were used in the first line in all patients with a response less than 30%. In the second line, Rituximab was used in 71% of patients, ITK (Imatinib) in 28% (pulmonary GVH), and extracorporeal photophoresis in a patient with musculoskeletal GVHD.The median follow‐up was 45 months with an overall survival of 67% and an event‐free survival of 60%.Summary/Conclusion:Allogenic stem cell transplantation is a real challenge in under‐developing countries, The post‐transplant represent a heavy multidisciplinary approach and a very expensiveIn our series 10% of patients have died from chronic GVH, then we need a better understanding and mastery of this complication.
PB2401 GENETICS OF IRANIAN ALPHA THALASSEMIA PATIENTS; COMPREHENSIVE ORIGINAL STUDY
Authors: Paridar M. , Khosravi A. , Keikhaei B. , Shariati G. 
Published: June 2019

Please indicate where the abstract has been published before: The full paper was published in Biochemical genetics journalBackground:Alpha thalassemia is the most prevalent monogenic gene disorder in the world and especially in Mediterranean countries. In the current hematological phenotype of patients with different genotypes, and also the effect of missense mutations on the protein function and stability were evaluated in a large cohort study.Aims:To evaluate the genetics of large populations of Iranian alpha thalassemia patientsMethods:One thousand five hundred and sixty subjects were participated in the study and divided into two groups; 259 normal subjects and 1301 alpha thalassemia carriers. Genomic DNA was extracted and analyzed using ARMS PCR, Multiplex Gap, and direct sequencing. The effects of single nucleotide change on the protein function and stability were predicted by free available databases of human polymorphisms.Results:Sixty‐three different genotypes were seen in the patients. The more prevalent was heterozygote form of ‐α3.7 (41.4%) followed by ‐α3.7 homozygote(11.6%) and ‐ ‐MED(3.8%). The significant differences were seen in mean hemoglobin level [F = 20.5, p < 0.001] between the Alpha globin genotypes when adjusted for gender. Moreover, 28 different mutations were found in our study. A significant relationship was seen between ethnicity and the alpha globin mutation frequency X2 (df;8) = 38.36, p <0.0001).Summary/Conclusion:Different genotypes could be displayed as different phenotypes. The mutation frequency distributions in our region are different from other parts of Iran. Significant differences are seen in the spectrum of mutation frequency between various ethnicity. Finally, some missense mutations maybe did not considerably effect on the proteins and could be a neutral mutation.image
PB2404 ARE DAA SAFE AND EFFECTIVE IN TREATMENT OF THALASSEMIA PATIENTS WITH CHRONIC HCV?
Authors: Ashwah S. El , Eisa N. , Ghoneem E. A. , El‐Etreby S. 
Published: June 2019

Background:Hepatitis C virus (HCV) infection is a major cause of liver‐related morbidity and mortality among thalassemic patients. Direct‐acting antivirals agents (DAAs) are highly effective and well‐tolerated by chronic HCV patients.Aims:This study was conducted to evaluate the safety and efficacy of DAAs in treatment of thalassemic patients with chronic HCV.Methods:This prospective study enrolled 22 thalassemic patients with chronic HCV infection proved by positive RT PCR HCV RNA from a total 40 patients who were positive for HCV ELISA test. Severity of liver disease was assessed in all patients. They received treatment of HCV according to guidelines of the National Committee for Control of Viral Hepatitis (NCCVH) in Egypt. About, 16 (72.72%) patients were treated with a sofosbuvir plus daclatasvir, and the other 6 (27.27 %) patients received sofosbuvir plus ledipasvir for 3 months. Primary end point was assessed by achievement of sustained virological response (SVR) at 12 weeks. Secondary end point was assessed by recording any side effects, transfusion requirements if need or stoppage of treatment.Results:The mean age of our patients was 29 years and 50% of them were male. Only 2 (9.09%) of them had compensated cirrhosis. SVR 12 was achieved in all patients (100%). Only one case required treatment discontinuation for one week due to acute kidney injury after prolonged use of NSAIDs then resumed DAAs after decline of creatinine to normal value. The most common side effects were fatigue (18%), progressive anemia (13.63%), requiring blood transfusion that occurred in three of the patients who received sofosbuvir plus daclatasvir, and lastly, headache (4.5%). There was no statistically significant difference in the level of hemoglobin before and after DAAs (8.5 ± 1.34 gm/dl vs 8.6 ± 1.38 gm/dl with p value = 0.484). There was significant improvement of mean ALT values after treatment compared to baseline one (42.45 ± 18.56 vs 57.29 ± 35.07 with p value < 0.0005).Summary/Conclusion:Direct acting antiviral drugs namely sofosbuvir plus daclatasvir or sofosbuvir plus ledipasvir are safe, effective, and well tolerated regimens in thalassemic patients with chronic HCV.
PB2414 AWARENESS AND KNOWLEDGE OF CARDIOVASCULAR DISEASE RISK AMONG THALASSEMIA PATIENTS
Authors: Meloni A. , Maffei S. , Pistoia L. , Traghella I. , Spiga A. , Massei F. , Serra M. , Guerrini G. , Giovangrossi P. , Fotzi I. , Marcheschi P. , Pepe A. 
Published: June 2019

Background:Cardiovascular diseases (CVDs) are the main cause of morbidity and mortality for thalassemia major (TM) patients but also for the general population in industrialized countries. Different studies revealed broad misconceptions with regard to the leading cause of death and impact of heart disease among the general population.Aims:We evaluated for the first time knowledge and awareness of CVD among TM patients in Italy.Methods:An anonymous questionnaire was completed by 44 TM patients (25 females, 39.95 ± 6.89 years) consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network.Results:Table 1 shows the answers to the first part of the questionnaire.The first two questions were multiple‐choice questions. The 74.4% of TM patients defined cancer (in general, breast/prostate or hepatic) as the greatest health problem for people of the same age. Out of the 13 patients with a CVD, only two defined CVD as the greatest health problem.The 44,1% of patients indicated cancer as the greatest danger to their future health.The other questions were yes/no questions. The 80.5% of TM patients thought that CVD was the leading cause of death in Italy. The percentage of patients who answered positively to this question was no significantly different among those with CVD than those without CVD. The 41.7% of patients who considered CVD to be the main cause of death named CVD as the greatest health problem.Summary/Conclusion:Although TM patients are aware that CVD is the leading cause of death, they seem to have a misperception of the impact of CVD on their personal current and future health status. Our data highlight the need to empower knowledge and perception of CVD in TM as a real health problem and not just as a potentially lethal threat.image
PB2417 SPECTRUM OF HAEMOGLOBINOPATHIES;A TERTIARY CARE HOSPITAL EXPERIENCE
Authors: Fareed N. , Fatima G. , Quraishy M. 
Published: June 2019

Background:Inherited abnormalities of hemoglobin synthesis include a myriad of disorders ranging from thalassemia syndromes to structurally abnormal hemoglobin variants.Identification of these disorders is immensely important epidemiologically and aid in prevention of more serious hemoglobin disorders.Aims:This study presents the pattern of haemoglobinopathies amongst the referred patients of anaemia in a 1 year period, it helps to identify the cases of different hemoglobin disorders.Methods:Inherited abnormalities of hemoglobin synthesis include a myriad of disorders ranging from thalassemia syndromes to structurally abnormal hemoglobin variants. Identification of these disorders is immensely important epidemiologically and aid in prevention of more serious hemoglobin disorders.Results:Out of 747 cases, 558 (74.6%) were normal and 189 (25.3%) cases had abnormal haemoglobin pattern. 356 (47.6%) were males and 391 (52.3%) were females. Of all cases of Anaemia 278 (49.8%) were microcytic hypochromic, 22 (3.9%) macrocytic and the rest 258 (46.2%) had normocytic normochromic picture. Of the 189 abnormal cases, Spectrum of haemoglobinopathies prevalent were Beta Thalassemia trait 117 (61.9%), followed by Beta thalassaemia major 27 (14.2%). Other haemoglobinopathies in descending order of frequency were sickle cell disease 15 (7.9%), Hb D Disease 16 (8.4%), sickle cell trait 01 (0.5%), Sickle/beta thalassaemia in 02 (1.0%) and Hb E in 04 (2.1%).Summary/Conclusion:Our study showed higher frequency of Beta Thalassaemia trait. It is suggested that detection of HbA2 should be carried out in all the high‐risk groups with anaemia. Further larger studies are needed to screen our population to detect thalassaemia carrier state and Iron deficiency Anaemia.
PB2424 AUGMENTED AND SYNERGISTIC EFFECT OF FACTOR V LEIDEN (FVL) AND MTHFR GENES POLYMORPHISMS AND PLATELET ACTIVITY AND REACTIVITY AS RISK FACTORS FOR ISCHEMIC CEREBROVASCULAR STROKE (IS)
Authors: Abdelhamid A.E.S. , Nashaat H.‐A. Hassan , Ahmed A. Sayed , Hosny A. O. , Hassan A.M. , ELSamahy M.A.A. , Abdelhamid M.A.S. 
Published: June 2019

Background:Ischemic cerebrovascular stroke (IS) is a polygenic multifactorial disease; effect of thrombotic gene polymorphism, platelet activation and reactivation synergistic risk effect are plausible postulation.Aims:To assess synergistic effect of FVL and MTHFR genes polymorphisms and platelet activity and reactivity as risk factors for ischemic cerebrovascular strokeMethods:A hospital‐based case‐control study; approvered by Suez Canal University (SCU) ethical committee, after obtaining an informed consent. 120 individuals were included: 60 acute IS patients, subdivided into 2 subgroups regarding the vascular risk factors recruited from neurology department, SCU Hospital; and 60 matched healthy individuals. FVL G1691A, FVLA4070G, FVL A5279G, MTHFRC677T, and MTHFR A1298C were assessed using multiplex real‐time PCR assay (AnyplexTM II Thrombosis SNP Panel Assay, seegene); platelet ex vivo activity & reactivity (CD62P; after ADP provocation) were assessed by four‐colors flow cytometry, FACS Caliber, BD‐Biosciences).Results:Both FVLG1691A & FVL A4070G (OR 19.6; 95 % CI = 2.5 to 154.4; p = 0.0001), (OR 7.6; 95 % CI = 1.13 to 178; p = 0.012), and MTHFRC677T (OR 10; 95 % CI = 1.16to 85.8; p = 0.03) and MTHFRA1298C (OR 11.6; 95 % CI = 1.35to 100.7; p = 0.03) & the presence of >1 mutation (p < 0.0001) showed a statistically significant association with IS. Multivariate logistic regression models showed that studied SNPs add synergistic effect on the vascular risk factors. A statistically significant difference was detected in platelet CD62p % after adding ADP (IS patients: 72.95 ± 14.8 ‐ controls: 62.2 ± 11.39), MFI IS patients: 248 ± 101‐controls 215 ± 75.8). both FVLG1691A and MTHFRC677T were significantly associated with higher baseline platelet activation in the co‐dominant and recessive models for FVLG1691A and in the dominant model for MTHFRC677TSummary/Conclusion:FVLG1691A, FVL14070G, MTHFRC677T & MTHFRA1298C were independent risk factors for IS especially in younger patients & existence of >1 mutation significantly increased IS risk, platelet activity and reactivity and genetic polymorphism play a potential synergistic role increasing the risk of IS.image
PB2431 CAROTID INTIMA‐MEDIA THICKNESS PROGRESSION AND BLOOD COAGULATION FACTOR XIII LEVELS IN TYPE 2 DIABETES: 8 YEARS OF FOLLOW‐UP
Authors: Sadeghi F. , Cogoi B. , Káplár M. , Csiki Z. , Kovács S. , Katona É. , Muszbek L. , Shemirani A. H. 
Published: June 2019

Background:Blood coagulation factor XIII (FXIII) main function is to strengthen and consequently protect fibrin polymers from fibrinolysis. Associations of transglutaminases in general, FXIII in particular and atherogenesis are described in the literature. Carotid intima‐media thickness (CIMT) has been shown to be a proxy end point for future vascular events. CIMT is related to premature atherosclerosis in healthy young adults.Aims:In this prospective study, the objective was to assess the relation between FXIII antigen, activity, CIMT at follow‐up as well as for CIMT progression (ΔCIMT).Methods:This prospective study comprised of 118 patients (56 females, 62 males, mean age of 62 ± SD10 years). All participants in the study cohort offered clinical, biochemical and ultrasonography investigations at baseline and at follow‐up.Results:During the period of investigation, mean CIMT, FXIII cross‐linking activity and antigen levels, both non‐adjusted and adjusted for the respective independently associated parameters, were significantly higher (p < 0.001). We divided the patients in tertiles of FXIII activity and antigen. At baseline, number of patients decreased as FXIII cross‐linking activity increased. It was reversed at follow‐up and significantly higher number of patients were present at upper tertile compare with lower tertile (p = 0.023). We observed similar results in case of FXIII antigen (p < 0.001).Summary/Conclusion:In conclusion, FXIII antigen and activity are elevated in individuals with type 2 diabetes. Increased CIMT and its progression was observed in this group of patients.