bluuebunny/crossref_metadata_2025 huggingface dataset:
PB2340 INCIDENCE AND SEVERITY OF ORAL MUCOSITIS AMONG ALLOGENEIC STEM CELL TRANSPLANTATION PATIENTS FOLLOWING FOUR TYPES CONDITIONING REGIMENS
Authors: Torihata S. , Sukedai M. , Shimoide T. , Ehara H. , Kanazawa H. , Yasumoto M. , Yasutake N. , Shimoide A. , Enomoto A. , Hamada S. , Ashida T.
Published: June 2019

Background:

Oral mucositis (OM) is a painful and debilitating complication of allogeneic hematopoietic stem cell transplantation (HSCT). Conditioning regimen correlates development of OM, but no studies have analyzed incidence and severity of OM among various condition regimens.

Aims:

This study was designed to evaluate the incidence and severity of OM receiving various conditioning regimens in early period after HSCT.

Methods:

We included 120 adult patients with a median age of 54.5 years (range 18 ‐ 72) who received allogeneic HSCT at Kindai University Hospital. Four conditioning regimens were administered for patients before transplantation based on the underlying diseases. Twenty‐two patients were received cyclophosphamide (CY)/total body irradiation (TBI) regimen (CY/TBI), 27 were received busulfan (BU)/CY regimen (BU/CY), 54 were received fludarabine (FLU)/BU regimen (FLU/BU), and 17 were received FLU/ melphalan (MEL) regimen (FLU/MEL). National Cancer Institute Common Toxicity Criteria for Adverse Events scale was used to assess the severity of OM. Severe OM defined as grade 2 to 4.

Results:

OM of any grades occurred 77.3% of CY/TBI, 48.1% of BU/CY, 85.2% of FLU/BU, and 70.6% of FLU/MEL. There were significant differences between CY/TBI and BU/CY (p = 0.05), and BU/CY and FLU/BU (p = 0.001). There was no patient with grade 4 OM. The incidence of severe OM (grade 2 ‐ 3) was 54.5% of CY/TBI, 18.5% of BU/CY, 57.4% of FLU/BU, and 52.9% of FLU/MEL. There were significant differences between CY/TBI and BU/CY (p = 0.02), BU/CY and FLU/BU (p = 0.0009), and BU/CY and FLU/MEL (p = 0.02).

Summary/Conclusion:

Incidence and severity of OM in patients receiving BU/CY regimen was the lowest among 4 types conditioning regimens.
PB2343 INDIVIDUALIZED TREATMENT AGAINST BACTERIAL INFECTIONS IN STEM CELL RECIPIENTS ‐ SINGLE CENTER EXPERIENCE
Authors: Stojanoski Z. , Pivkova A. , Panovska‐Stavridis I. , Cadievski L. , Georgievski B. , Ivanovski M. , Karanfilski O. , Cevreska L.
Published: June 2019

Background:

Multidrug resistant (MDR) bacteria are increasingly frequent in HSCT recipients, but significant differences in etiology of bacterial infections and prevalence of resistant strains exist between different transplant centers Extended‐spectrum beta‐lactamase producing Escherichia coli, Vancomicyn resistant enterococci, Penicillin‐resistant Pneumococci Methicillin‐resistant Staphylococcus aureus and are major problem to manage.

Aims:

Aim: to evaluate the frequency of multidrug resistant bacteria in our center.

Methods:

During a 19 years period we have transplanted 500 patients with different hematological malignant and nonmalignant diseases. All patients were treated in sterile room conditioned with HEPA filters and low bacterial diet. Antibacterial prophylaxis consisted Ciprofloxacine 1,0gr/day. As empirical antibiotics regimen we administered combination of third‐generation cephalosporin + amynoglicoside, while as second line therapy Imipenem + Vancomycin

Results:

in every patient 3 times a week we monitor: blood culture, central venous catheter culture, sputum, urine culture. From all 288 bacterial isolates the distribution of MDR bacteria were: ESBL positive Escherichia coli 3 (0,10%) VR Enterococcus 2(0,06%) MDR Pseudomonas aer. 1 (0,03%) Klebsiella aerogenes 2 (0,06%) MRSA 17(0,06%) Acinetobacter baumanii 2(0,06%) Stenotrophomonas Maltophilia 2(0,06%). There were fatal outcome due to sepsis in 3 patients (VRE 1, MDR Pseudomonas 1, Stenotrophomonas maltophilia 1).

Summary/Conclusion:

The monitoring of local microflora is crucial for every transplant center. Empirical first‐line therapy must be individualized and according to ECIL 4 escalation and de‐escalation strategy is necessery for better outcome in this group of immunocompromised patients.
PB2346 RISK FACTORS ANALYSIS FOR DISEASE RECURRENCE AFTER ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION IN PATIENTS WITH HIGH‐RISK ACUTE LEUKEMIA
Authors: Zhang R. , Wu Q. , Jiang Z. , Wan D. , Li L. , Xia L.
Published: June 2019

Background:

Relapse is a major cause of treatment failure for high‐risk acute leukemia after allogeneic hematopoietic stem cell transplantation (allo‐HSCT). Prognosis is considered dismal but data about risk factors associated with disease recurrence have not been elucidated.

Aims:

We aim to explore the pre‐ and post‐transplantation risk factors on relapse and survival in order to formulate individualized prophylaxis and treatment protocol for primary reoccurrence in high‐risk patients before and after transplantation.

Methods:

We retrospectively analyzed the outcomes of 321 high‐risk acute leukemia patients who underwent allogeneic hematopoietic stem cell transplantation (allo‐HSCT) who were diagnosed and treated at Union Hospital, and further evaluated the impact of pre‐ and post‐transplantation risk factors on relapse and survival by multivariate analysis.

Results:

Overall, 104 patients relapsed after allo‐HSCT with relapse cumulative incidence of 31 ± 2%. The median time of relapse was 4 (1.5‐26) months. With a median follow‐up of 50 months, the 3‐year overall survival (OS) and disease‐free survival (DFS) were 70%, 60%, respectively. Multivariate analysis showed that conditioning regimen (HR, 0.309; 95% CI, 0.119‐0.806, p = 0.016), type of disease (HR, 0.335; 95% CI, 0.125‐0.896, p = 0.029), and disease status at transplantation (HR, 1.964; 95% CI, 1.156‐3.338, p = 0.013) were independent factors influencing relapse for high‐risk acute leukemia patients following allo‐HSCT.

Summary/Conclusion:

Since the prognosis of patients with acute leukemia who experienced relapse after allo‐HSCT was poor, we emphasize the urgent need for formulating individualized prophylaxis and treatment protocol for primary reoccurrence in high‐risk patients before and after transplantation.
PB2356 IMPACT OF ABO BLOOD GROUP INCOMPATIBILITY ON THE OUTCOMES OF ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION
Authors: Çiftçiler R. , Goker H. , Buyukasık Y. , Aksu S. , Demiroglu H.
Published: June 2019

Background:

Impact of ABO Blood Group Incompatibility on the Outcomes of Allogeneic Hematopoietic Stem Cell Transplantation

Aims:

ABO and Rh compatibility are not required between the donor and recipient for alloHSCT. Although ABO incompatibility is not considered a contraindication in HSCT, its clinical outcomes are still a doubtful. In this study, we analyzed the neutrophil and platelet recovery, GVHD, relapse rate, mortality rate, non‐relapse mortality and survival in patients who underwent alloHSCT.

Methods:

Two hundred and sixty eight patients with hematopoietic malign diseases and inborn errors of metabolism or the immune system who received an alloHSCT in our HSC transplant center between the years of 2001 and 2018 were evaluated.

Results:

There were 264 (98.5%) matched sibling, and 4 (1.5%) haploidentic transplants. Indications for HSCT included both hematological malignancies (n = 261) and benign conditions (n = 7). Of these donor recipient pairs, there were 193 (72%) matches, 36 (13.4%) major, 29 (10.8%) minor and 10 (3.7%) bidirectional ABO mismatches. The median follow‐up of 120 months (range, 6‐213) for the entire group. The OS and DFS were not statistically significant different between two groups. The seventy one (41.1%) of the ABO match patients and 27 (35.5%) of the ABO mismatch patients developed GvHD. There was no significant difference between the two groups in terms of GvHD (p = 0.86). The median neutrophil engraftment day was 11 (7‐20) day in ABO match patients and 11 (7‐19) day in ABO mismatch patients (p = 0.44). The median platelet engraftment day was 12 (8‐38) day in ABO match patients and 12 (8‐40) day in ABO mismatch patients (p = 0.48).

Summary/Conclusion:

In conclusion, this study showed major ABO incompatibility does not lead mainly to delayed RBC recovery, platelet engraftment and neutrophil engraftment after alloHSCT. Major ABO mismatch does not seem to have a significant effect on other major outcomes after alloHSCT, such as GVHD, relapse rate, mortality rate, non‐relapse mortality rate, DFS and OS.

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PB2373 LIVER DISEASE DURING AND AFTER AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION IN EGYPT (SINGLE CENTER EXPERIENCE)
Authors: Moaty I. Abdel
Published: June 2019

Please indicate where the abstract has been published before: egyptian socitey of hepatology and gastroenterology journal

Background:

Hematopoietic stem cell transplantation has a rapid increase over the last decade. It is an established therapy for many hematological disorders. HSCT had many possible serious complications. One of those complications is hepatic dysfunction.

Hepatic complication in autologous HSCT survivors could be early complications include drug toxicity, infections and long‐term liver complications from chronic viral hepatitis, iron overload or original disease relapse.

Aims:

is to determine the prevalence, etiology of liver disease in Egyptian population who had HSCT

Methods:

this study is cross‐sectional study in December 2016 including 88 patients who had autologus transplantation in Ain Shams University Hospitals

Results:

In autologus patients the first cause of hepatotoxicity is conditioning regimen involving 27% of the patients. Second cause is flare of viral hepatitis affecting 8% of the patients. In third place is sepsis that leads to elevated liver function which occurred in 6% of our patients, these figures were also dramatically changed after D + 100 and lower hepatotoxicity percentage occurs as 11% of the patients had liver injury caused by drugs and only 1% patients had viral hepatitis reactivation.

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Summary/Conclusion:

Drug hepatotoxicity is main cause of liver injury in BMT unit in Ain Shams University in Egyptian population.
PB2377 SECONDARY HYPOGAMMAGLOBULINEMIA AFTER HIGH DOSE CHEMOTHERAPY WITH AUTOLOGOUS STEM CELL TRANSPLANTATION FOR LYMPHOMA
Authors: Nakaya A. , Ishii K. , Fujita S. , Satake A. , Nakanishi T. , Azuma Y. , Tsubokura Y. , Saito R. , Konishi A. , Hotta M. , Yoshimura H. , Ito T. , Nomura S.
Published: June 2019

Background:

Secondary hypogammaglobulinemia is associated with hematological malignancy, particularly related to lymphoid malignancy. Other than malignancy itself, treatments induce iatrogenic hypogammaglobulinemia. In standard, eligible patients with advanced lymphoma receive high dose chemotherapy with autologous stem cell transplantation (HDT/ASCT). However, frequency and risk of hypogammaglobulinemia in auto‐setting is unknown.

Aims:

To clarify frequency and risk of hypogammaglobulinemia in auto‐setting.

Methods:

We retrospectively analyzed 101 patients with advanced lymphoma who received HDT/ASCT at Kansai Medical University Hospital and Kansai Medical University Medical Center from August 2006 to September 2018. All patients received MEAM regimen with/without rituximab as a conditioning.

Results:

Median age was 59(24‐71) y/o. Among 101 patients, 92% of patients(n = 93) was measured immunoglobulin levels routinely. 52% of patients(n = 53) had hypogammaglobulinemia(IgG < 700 mg/dl) and 25%(n = 25) of patients remains hypogammaglobulinemia presently. 14 patients recovered from hypogammaglobulinemia. Median period to recovery was 28(0.7‐56.8)months. Among the patients developed hypogammaglobulinemia, 87% was with B cell lymphoma, 9% with T cell lymphoma and 4% with Hodgkin lymphoma. Severe infection(≥grade3) was seen in 16 patients. The most frequent infection was bacterial infection unknown species(n = 5), followed HBV reactivation(n = 3) and Herpes zoster(n = 3), and BKV(n = 2).

Summary/Conclusion:

52% of patients had hypogammaglobulinemia after HDT/ASCT, which was not only with B cell lymphoma but also with T cell and Hodgkin lymphoma. Median period of immunoglobulin recover is over one year. Addition to common bacterial infections, we should be careful about virus infections.
PB2379 INVASIVE FUNGAL INFECTIONS IN PATIENTS UNDERGOING ALLOGENEIC STEM CELL TRANSPLANTATION CONDITIONED WITH REDUCED BUCY2
Authors: Armillas F. M. , Franco M. Rivera , Rodríguez E. León
Published: June 2019

Background:

Invasive fungal infection (IFI) is a major cause of morbidity and mortality in patients undergoing allogeneic stem cell transplantation (allo‐SCT). We previously reported (Ann Hematol 2017) that reduced BUCY2 (rBUCY2) preserves an immunosuppressive and myeloablative effect, achieving an adequate bone marrow engraftment with acceptable toxicity, low graft‐versus‐host disease (GVHD), and low transplant‐related mortality (TRM)

Aims:

To determine the frequency of IFI in patients undergoing allo‐HSCT using rBUCY2 as the conditioning regimen

Methods:

We conducted a retrospective study of consecutive patients undergoing allo‐SCT using rBUCY2 between November 1998 and December 2014. The median follow‐up was 90 months. Data was analyzed in March 2018.All the SCT procedure and follow‐up was performed at the National Institute of Medical Sciences and Nutrition “Salvador Zubiran”, a public tertiary/referral center in Mexico City.Patients were conditioned with rBUCY2 (busulfan 12 mg/kg, ORAL and cyclophosphamide 80 mg/kg, IV), using G‐CSF‐primed bone marrow as the stem cell source. Antifungal prophylaxis consisted of low‐dose amphotericin B (0.2 mg/kg/day) administered until neutrophil engraftment.The primary endpoint was 1‐year cumulative incidence of proven or probable IFI and the secondary endpoint was the IFI‐associated mortality. Risk factors of IFI were also assessed.

Results:

From all the cohort, 57% were male and the median age was 39 years (range, 17‐67). Hematological malignancies were the underlying diseases in most patients (n = 55, 94.8%). Within 100 days post‐SCT, IFI was presented in 4 patients (6.8%) and only 1 patient presented late IFI. The multivariate analysis showed that cytomegalovirus (CMV) viremia was the main risk factor associated with the development of IFI (p = 0.031). IFI‐associated mortality was 5% and showed an association with matched unrelated donors (p = 0.022), parenteral nutrition (p = 0.027), and early IFI (p = 0.004). The TRM was 17%

Summary/Conclusion:

Our results highlight the low incidence of IFI using our modified conditioning regimen compared to international literature. As other studies have reported, we demonstrated that CMV was associated with the development of IFI. We emphasize the safety of rBUCY2 demonstrated by the low incidence of IFI and the low TRM.
PB2389 UNDERDOSING DEFERIPRONE IN TM: MYTH OR REALITY?
Authors: Spasiano A. , Meloni A. , Pistoia L. , Roccamo G. , Commendatore F. V. , Pulini S. , Benni M. , Iacono N. Dello , Campisi S. , Allò M. , Vallone A. , Riva A. , Positano V. , Pepe A.
Published: June 2019

Background:

Data from clinical practice show that some patients with thalassemia major (TM) received underdoses of deferiprone (DFP).

Aims:

This retrospective real‐life study aimed to investigate the reasons of the underdosing of DFP and to assess if the underdosing could result in an inadequate response.

Methods:

Among the 1746 TM patients consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network between 2006 and 2015, we selected the 171 consecutively patients (100 M and 71 F) patients who were treated with DFP dosages<75 mg/Kg/day. Mean age at starting underdosed therapy was 30.95 ± 8.78 years. Heart and liver iron overload were measured by T2∗ Magnetic resonance imaging (MRI) technique.

Results:

Fifty‐four patients were treated with DFP in monotherapy (60.31 ± 12.82 mg/kg/die for 6.96 ± 0.19 days/week), 87 with combined DFP + deferoxamine (DFO) therapy (52.28 ± 21.29 mg/kg/die for 6.00 ± 1.43 days/week), and 30 with sequential DFO/DFP therapy (60.53 ± 9.75 mg/kg/die for 4.07 ± 0.69 days/week).

Table 1 shows the motivations of underdosage, divided by chelation regimens. The most frequent motivation was the weight‐driven adjustment of the dosage, followed by the presence of low ferritin levels and side effects (gastric disturbances = 14/33 pts, increase of transaminases 6/33 pts and neutropenia 5/33).

Sixty‐eight patients maintained the same chelation regimen (24 monotherapy, 27 combined and 17 sequential) during the execution of two consecutive MRI scans, performed in a time span of 18 months. At the first MRI 17 patients showed myocardial iron overload (MIO‐global heart T2∗ value<20 ms). In this subgroup there was a significant increase of global heart T2∗ values (12.39 ± 3.77 ms vs 20.43 ± 10.71 ms; P = 0.002) and six (35.3%) patients reached a global heart T2∗ > 20 ms at the second MRI. None of the 51 patients with a normal global heart T2∗ value at the first MRI showed MIO at the second MRI. In the 39 patients with hepatic iron overload (MRI liver iron concentration‐LIC≥3 mg/g dw) at the first MRI, there was not a significant decrease in LIC values, although 7 (17.9%) patients reached a normal value at the second MRI. Out of the 21 patients with a normal LIC value at the first MRI, 5 (17.2%) showed hepatic iron at the second MRI.

Summary/Conclusion:

In the real world the weight‐driven adjustment of the dosage, low ferritin levels and sides effects were the main causes of the DFP underdosage in TM. DFP dosages<75 mg/Kg/day can be effective in improving myocardial siderosis but do not allow an improvement in terms of hepatic iron.

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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 severity

Aims:

This study aims to explore the modifying effects of XmnI and BCL11A loci on HbF levels in Egyptian β‐thalassemia patients

Methods:

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.

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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.

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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 Fya

Summary/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.

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PB2453 DISTRIBUTION OF BLOOD DONOR IN DIFFERENT AGE GROUP IN KATHMANDU NEPAL
Authors: Nepal B.
Published: June 2019

Background:

Voluntary non‐remunerated blood donor consists of 78% blood donor's population in Nepal. Therefore demographic about the distribution of blood donors according to the age group is important to achieve 100% Voluntary non‐remunerated blood donors in Nepal.

Aims:

To explore the demographic distribution of the blood donor in different age group in the Kathmandu Nepal.

Methods:

This is retrospective study conducted at Nepal Red Cross Society Central Blood Transfusion Service. Data from January 2013 to January 2017 were collected from donor management software. The data includes socio demographic data. Data has been process with SPSS version ‐17

Results:

During 4 years study period, total of 276,290 Blood donation happened from both mobile blood collection and in‐house blood collection.

Out of 276,290 Collection, 48351 (17.5%) are from 18‐24 age group; 106924 (38.7%) are from 25‐31 age group 53324 (19.3%) are from 32‐38 age group; 34536 (12.5%) are from group 39‐45 age group; 23761 (8.6%) are from age group 46‐52 and 9394 (3.4%) from age group above 53 respectively.

Summary/Conclusion:

The distribution of ABO blood group varies regionally and from one population to another. In Kathmandu, Nepal 18‐38 years age group is the most common age group encountered donating blood. The data generated in the present study and several other studies of different geographical region of India will be useful to health planners and future health challenges in the region
Graduate-Entry Education for Nonnurses: Preparation, Pathways, and Progress
Authors: Jackson Molly , Marchi Nadine
Published: June 2019

AIM

The aim of the study was to review education and role preparation in US direct-entry graduate nursing programs.

BACKGROUND

In the last 15 years, there has been an increase in nursing programs targeting college graduates with nonnurse degrees. Educational pathways, role preparation, and doctoral study options vary.

METHOD

A literature search was conducted. A secondary search of direct-entry graduate nursing programs was performed using electronic databases, including academic nursing sources and university websites.

RESULTS

Forty-three journal articles were located. The secondary search identified 42 direct-entry graduate nursing programs. The majority of programs emphasize role preparation for clinical nurse leadership and advanced practice registered nursing (APRN) certification; 62 percent require doctoral preparation for entry to APRN practice.

CONCLUSION

The growth of direct-entry graduate nursing programs will require educators to prepare and evaluate student outcomes to ensure competent entry to practice.
Heterogeneous Trajectories of Delayed Communicative Development From 12 to 36 Months: Predictors and Consequences
Authors: Hentges Rochelle F. , Madigan Sheri , Plamondon Andre , Racine Nicole , Collisson Beverly , Tough Suzanne , Graham Susan
Published: June 2019

Objective:

The objective of the study was to identify distinct trajectories of delayed communicative development from 12 to 36 months and examine differences in risk factors and developmental outcomes for each trajectory.

Methods:

Participants were 2192 children drawn from a prospective longitudinal pregnancy cohort in a large Canadian city. Maternal pregnancy medical records were used to determine perinatal risk factors. The Ages and Stages Questionnaire Communication subscale was administered at 12, 24, and 36 months. At 36 months, mothers reported on the child's health, cognitive, and behavioral development.

Results:

Using growth mixture modeling, we identified 4 trajectories of communicative development. Most children (81.1%) were characterized by high and stable scores from 12 to 36 months. The remaining children fell into a low-increasing class (13.0%), a moderate-stable class (4.5%), and a low-decreasing class (1.4%). At 36 months, the low-increasing class had caught up to the high-stable group. However, by 36 months, the low-decreasing class fell under the recommended “referral” cutoff, and the moderate-stable class fell under the “monitoring” cutoff criteria. Children with continued communication problems at 36 months were more likely to have a congenital anomaly and lower family income than late-talking children who had caught up.

Conclusion:

Repeated assessments of a brief screening tool were able to differentiate patterns of communicative development over time, each with unique risk factors and developmental outcomes. Results highlight the potential for risk factors and repeated screenings to help identify children most at risk for persistent communication delays and in need of early support services.
Effect of amphetamine dose on wheel-running functioning as reinforcement or operant behavior on a multiple schedule of reinforcement
Authors: Belke Terry W. , Pierce W. David , Sexton Christine A.
Published: June 2019

Does the effect of amphetamine on behavior (wheel running) differ depending on the functional role (operant, reinforcement) of that behavior? This study addressed this question using a multiple schedule of reinforcement in which wheel running served as reinforcement for lever pressing in one component and as operant behavior for sucrose reinforcement in the other component. Seven female Long–Evans rats were exposed to a multiple schedule in which pressing a lever on a variable ratio 10 schedule produced the opportunity to run for 15 revolutions in one component and running 15 revolutions produced a drop of 15% sucrose solution in the other component. Doses of 0.5, 1.0, and 2.0 mg/kg d-amphetamine were administered by intraperitoneal injection 20 min prior to a session. As amphetamine dose increased, wheel running decreased in both components – showing no evidence that the effect of the drug on wheel running depended on the function of wheel activity. Notably, lever pressing for wheel-running reinforcement also decreased with amphetamine dose. Drug dose increased the initiation of operant lever pressing, but not the initiation of operant wheel running. We propose that amphetamine dose had common effects on wheel running regardless of its function (reinforcement vs. operant) because wheel-running generates automatic reinforcement and the automatic-reinforcement value of wheel activity is modulated by drug dose.
Inflammatory Reaction to Decellularized Porcine-Derived Xenograft for Lower Eyelid Retraction
Authors: Mancera Norberto , Schneider Alexander , Margo Curtis E. , Bajric Jasmina
Published: June 2019

A 54-year-old woman underwent uncomplicated bilateral lower eyelid surgery for eyelid retraction using porcine decellularized membrane graft (TarSys). Left lower eyelid pain began several months after surgery and persisted until the graft was removed 15 months after implantation. The opposite eyelid graft caused no symptoms. Histologically, the lesion consisted of acellular collagen surrounded by florid granulomatous inflammation. Although the inflammatory reaction suggested a cell-mediated immune response, this pathway of injury is difficult to reconcile given the asymptomatic opposite eyelid. The literature reflects limited experience with porcine decellularized membranes of the eyelid. Complications and long-term tolerance remain to be determined.