bluuebunny/crossref_metadata_2025_split huggingface dataset:
PB1828 PRIMARY PROPHYLAXIS OF CHEMOTHERAPY‐INDUCED FEBRILE NEUTROPENIA IN ADULT PATIENTS WITH LYMPHOPROLIFERATIVE DISORDERS ‐ CLINICAL EXPERIENCE IN UNIVERSITY HOSPITAL SPLIT
Authors: Petrić M. , Vicelić Čutura L. , Blaslov V. , Miljak A. , Galušić D. 
Published: June 2019

Background:Febrile neutropenia (FN) is the development of fever in a patient with an abnormally low number of neutrophil granulocytes. It can be associated with other signs of infection and is often consequence of chemotherapy treatment. Granulocyte colony‐stimulating factors (G‐CSF) are used for prophylaxis of FN. We analyzed data of adult patients with lymphoproliferative disorders who were treated with chemotherapy regimens of high or intermediate risk for developing FN, according to guidelines of European organization for research and treatment of cancer (EORTC).Aims:To investigate the benefit of primary prophylaxis of chemotherapy‐induced FN in patients with lymphoproliferative disoders. To evaluate the incidence of FN following chemotherapy regimens of high or intermediate risk for developing FN.Methods:We analyzed medical history of all 97 adult patients (M = 55; F = 42, aged 21 – 81 (mean, 54) years) with lymphoproliferative disorders who were treated with chemotherapy regimens of high or intermediate risk for developing FN in the period from 1st November 2017 till 1st November 2018. Incidence of FN was analyzed according to type of chemotherapy regimen and administration of primary prophylaxis.Results:Primary prophylaxis with G‐CSF was administered to all (N = 12) patients who were treated with high risk chemotherapy regimens, in compare to only 24 (28%) patients who received intermediate risk regimens. FN was registered in total of 24 (25%) patients, of whom 20 were treated with intermediate risk regimens. Among patients who received primary prophylaxis after intermediate risk regimens, only 4 (17%) of them developed FN, in compare to 16 (26%) of patients who previously did not receive primary prophylaxis after same regimens. Incidence of FN was higher in females (N = 14) and after the first cycle of chemotherapy regimen (N = 15).Summary/Conclusion:Primary prophylaxis with G‐CSF reduces the risk of developing FN, so it is recommended to administer primary prophylaxis to all patients who are treated with high risk chemotherapy regimens. Primary prophylaxis in patients receiving intermediate risk regimens should be carefully considered because of the high incidence of FN in those who did not receive prophylaxis. In those circumstances, other risk factors (such as comorbidities, age of patient, history of previous infections and FN, stage of the disease and others) should be evaluated before the decision whether to administer primary prophylaxis or not. In our patients, low incidence of FN was observed in the group who received primary prophylaxis after intermediate risk regimens.
PB1842 ROLE OF THROMBOELASTOGRAPHY IN THE MONITORING OF PROPHYLACTIC TREATMENT IN PATIENTS WITH SEVERE HAEMOPHILIA A
Authors: Krasivska V. , Stasyshyn O. , Semerak M. , Tushnytskyi O. 
Published: June 2019

Background:According to many experts, during prophylactic treatment, the activity of factor VIII (FVIII) is not a sufficient predictor of the effectiveness of prevention and does not reflect the level of risk of bleeding. During monitoring of the therapy with activated partial thromboplastin time (APTTT) and FVIII activity, the cause of the discrepancy may be unaccounted content of the tissue factor, the amount and the aggregative function of the thrombocytes, which are also involved in the coagulation process. Therefore, global laboratory methods, in particular thromboelastography (TEG), are used to control therapyAims:To study the role of TEG in the monitoring the prophylactic treatment of clotting factors concentrates in patients with haemophiliaMethods:The study was performed in 9 patients with severe haemophilia A, who received prophylactic treatment by prolonged recombinant FVIII concentrate at a dose of 45 ± 5 IU/ kg two times a week. According to international recommendations all the patients were tested for incremental recovery (IR) of the administered concentrate FVIII in a dose of 60 ± 5 IU/kg during their routine visits. Pre‐ and post‐infusion coagulation tests and TEG (TEG 5000® Haemoscope Corp., Niles IL) were performedResults:Pre‐infusion the indicators of coagulation tests that characterized coagulation hemostasis (APTT‐72,9 sec, FVIII‐1,1%) were prolonged. 30 minutes post‐infusion the abnormal value came to the norm. The prothrombin time (PT), the fibrinogen level and quantity of platelets in the IR test did not differ from the corresponding values of healthy individuals. In TEG, the prolonged reaction time to clot formation R (21,9 sec), the time of maximum amplitude TMA (46,5 sec), the time of initial fibrin formation SP (19,1 min), the reduced rate of initial clot strengthening α‐Angel (33,0°), negative value of coagulation index CI (‐3,0) indicate a violation of the enzymatic phase of blood clotting. Post‐infusion of the calculated dose of the medication, all of the abnormal parameters returned to normal. In IR test, TEG parameters that characterize strength, quality, hemostatic capabilities, lysis of the clot and depend on the level of fibrinogen and platelets do not differ from those of healthy individualsSummary/Conclusion:TEG can be a reliable method for monitoring prophylactic treatment and IR in patients with hemophilia A without inhibitors in combination with coagulation tests (APTT, FVIII activity). The following parameters should be analyzed: the reaction time to clot formation R, the time of maximum amplitude TMA, initial fibrin formation SP, the rate of initial clot strengthening α‐Angel, the total coagulation potential CI
PB1850 DENTAL MANAGEMENT OF PATIENTS TAKING NOVEL ORAL ANTICOAGULANTS (NOAS): DABIGATRAN.
Authors: Kapsali A. , Papakitsos G. 
Published: June 2019

Background:A new group of oral anticoagulants with clear advantages over classic dicoumarin oral anticoagulants (warfarin and acenocoumarol) has been developed in recent years.Aims:Patients being treated with oral anticoagulants are at higher risk for bleeding when undergoing dental treatments.Methods:A literature search was conducted through April 2015 for publications in the ISI Web of Knowledge, PubMed and Cochrane Library using the keywords “dabigatran”, “new oral anticoagulants”, “bleeding” and “dental treatment”.Results:There is no need for regular coagulation monitoring of patients on dabigatran therapy. Whether or not to temporarily discontinue dabigatran must be assessed according to the bleeding risk involved in the dental procedure to be performed.Summary/Conclusion:The number of patients under treatment with new oral anticoagulants will increase in the coming years. It is essential to know about the pharmacokinetics and pharmacodynamics of new oral anticoagulants and about their interactions with other drugs. It is necessary to develop clinical guidelines for the perioperative and postoperative management of these new oral anticoagulants in oral surgical procedures and to carefully evaluate the bleeding risk of dental treatment, as well as the thrombotic risk of suppressing the new oral anticoagulant.
PB1851 PREDICAMENT OF SHORTENED PRE‐OPERATIVE EVALUATION OF UNDIAGNOSED BLEEDING DISORDERS IN SURGICAL PATIENTS IN UNDER DEVELOPED COUNTRY.
Authors: Imran T. , Altaf H.N. 
Published: June 2019

Background:Von Willebrand Disease (VWD) is a prevalent genetic bleeding disorder in Pakistan. it is a leading congenital bleeding disorder followed by Haemophilia. the significance of bleeding pattern in undiagnosed patients as evident after haemostatic challanges leads to the importance of the need to confirm bleeding disorders in emergency surgical patients.Aims:the aim of this retrosepctive study is to document the pattern of mortality and morbidity in undiagnosed surgical patients with various congenital bledding disorders in PakistanMethods:we analyzed the bleeding pattern of VWD and Haemophilia A and B in patients who were undiagnosed at the time of surgery. this retrospective study was done at Fauji Foundation Hospital from 2012 to 2016. patients’ charts were reviewed for primary diagnosis, surgical procedures and severity of bleeding by detailed history and laboratory workup. the study was carried out after approval of institutional ethical committee.Results:we received 14 VWD and 7 Haemophilia patients in emergency for surgical intervention. the median age was from 3 to 22 years. there were 16 male and 5 female patients. out of 21 patients 29% had laparotomy, 10% had head surgeries, 14% post trauma thoracic intervention, 10% had cholecystectomy, 19% had dental procedures and 14% had cesarean sections. collective mortality rate was 56% because of the obsecure condition added by delay in arranging appropriate treatment line for ongoing bleeding.Summary/Conclusion:congenital bleeding disorders are common in Pakistan. there is lack of diagnostic facilities and recognition by health care professionals. lack of awareness about these bleeding disorders before emergency surgeries results in fatal outcomes. awareness programes must be initiated in developing countries.
PB1854 MYELOFIBROSIS DUE TO SEVERE VITAMIN D DEFICIENCY RICKETS
Authors: El Chazli Y. , Eldomiaty B. , Balbaa N. , Hassan E. , Mikhael N. , Elsayed S. , Abdelmeguid Y. , Fawzy D. 
Published: June 2019

Background:The association between severe active infantile rickets and myelofibrosis has been reported as Von Jaksch's anemia since 1937. Vitamin D deficiency rickets is a common condition in infants and children of developing countries including Egypt. Despite this fact only 26 patients with myelofibrosis due to severe vitamin D deficiency rickets have been reported in the literature worldwide and none from Egypt.Aims:To report cases of myelofibrosis due to severe vitamin D deficiency rickets and to highlight the importance of considering this rare condition in the differential diagnosis of hypochromic microcytic anemia with splenomegaly in infants.Methods:retrospective description of patients diagnosed as myelofibrosis due to severe vitamin D deficiency rickets for a 6 month period at the Alexandria University Children's Hospital. After taking informed consent from their legal guardians, patients have been subjected to history taking, clinical examination, and laboratory investigations including bone marrow biopsy (BMB).Results:Five patients have been included. Four males and one female, with an age ranging from 6 to 22 months at presentation. They presented with clinical signs of active rickets (wide anterior fontanel, frontal bossing, rachitic rosaries, and hypotonia). They all had an enlarged spleen by examination, confirmed by ultrasonography. Their blood picture showed a hypochromic microcytic anemia (Hb 5 to 8.6 g/dL) with normoblasts, anisopoikilocytosis, teardrops and pencil cells, thrombocytopenia (platelet count 25 to 132 x103 cell/cmm, normal in one patient) and a normal white cell count showing monocytosis and immature forms (promyelocytes, myelocytes and band cells). Their chemistry was significant for low calcium, low phosphorus and very high alkaline phosphatase (ALP 922 ‐ 4678 U/L). Bone marrow was done and showed thickened trabeculae and increased bone marrow fibrosis using reticulin stain (picture). Unfortunately, the patients presenting with a symptomatic anemia had a packed red cells transfusion. All patients have been started on therapeutic doses of Vitamin D with rapid correction of their blood counts and gradual normalization of their chemistry parameters.Summary/Conclusion:It is important to raise clinicians’ awareness to such a rare but yet easy to diagnose condition to avoid unnecessary transfusions and investigations and delays in treatment. The treatment of myelofibrosis due to severe vitamin D deficiency rickets is very simple and effective.image
PB1855 UPFRONT HAPLOIDENTICAL TRANSPLANT IN YOUNG SEVERE APLASTIC ANEMIA PATIENTS ‐ RESULTS OF A PILOT STUDY FROM SOUTH INDIA
Authors: Kumar K. , Subash C. 
Published: June 2019

Background:Haploidentical bone marrow transplant is one of the second line treatment option in severe aplastic anemia patients failing immunosuppressive therapy. In younger patients, allogeneic bone marrow transplant from a matched sibling donor gives more than 80% long‐term remission rates. But unfortunately, only 30% patients will have a matched sibling donor. In a middle‐income country like India, the availability and affordability of patients to an unrelated donor is limited. Most of our patients have got a single chance for treatment and many relapsed patients go into palliative care.Aims:Aim of the present study is to evaluate the efficacy and safety of upfront Haploidentical bone marrow transplant in less than 40 years old severe aplastic anemia patients not having a 10/10 matched donor.Methods:This is a prospective non‐randomized single center study conducted during the period from January 2017 to June 2018. Eleven patients less than 40 years old underwent upfront Haploidentical bone marrow transplantation for severe aplastic anemia during the above period. Peripheral blood stem cell was used in all patients.FLU‐ATG‐PTcy was the conditioning regimen used in all patients with Cyclosporine + MMF based GVHD prophylaxis. This study got clearance from institutional review board and the transplant was sponsored by government.Results:In six patient's donors were parents (Mother in four and father in two) and rest of the donors were siblings. Two were Fanconi positive and none had a PNH clone. Seven were 5/10 matched, three were 6/10 and one was 7/10 matched. All patients had engraftment at a median of 11 days (range 8 to 13 days). One patient had fatal grade IV acute GVHD and succumbed to GVHD on day +36. Seven patients (64%) had grade I/II GVHD and three patients (27%) had grade III GVHD. 64% patients had grade I/II chronic GVHD at date cut off of January 2019. 10 patients (89%) attained complete remission and were alive till January 2019.The overall survival rate at one year post transplant was around 89% which is better than immuno‐supressive therapy in this age group. No differnces were observed in engraftment dates or survival outcomes based on type of donor and degree of mismatch. The study patients are very small to get statistically significant rates.Summary/Conclusion:With nearly 90% overall remission and survival rate at one year, upfront haploidentical transplant seems to be a safe and effective option in young patients with severe aplastic anemia. This will help to reduce the cost of Matched unrelated donor stem cells significantly in resource poor countries and increase the number of patients opting for a curative treatment.
PB1857 THE ROLE OF WHOLE‐BODY MAGNETIC RESONANCE IMAGING IN THE DETECTION OF VASCULAR EVENTS IN PATIENTS WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA.
Authors: Navarro‐Almenzar B. , Salido Fiérrez E. , Cabañas Perianes V. , Pérez López R. , García Candel F. , Fernández Poveda E. , Blanquer Blanquer M. , Leal Rubio J.D. , Torregrosa Salas B. , Moraleda Jiménez J.M. 
Published: June 2019

Background:Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder characterized by intravascular hemolysis, cytopenias, and recurrent thrombosis. Thrombotic events are just the main cause of morbidity and mortality. Therefore, it is essential to detect them early to treat them and avoid sequels.Aims:To analyze the diagnostic value of the whole‐body magnetic resonance imaging (WB‐MRI) in the detection of symptomatic and silent vascular events.Methods:Retrospective study in which a 1.5 Tesla whole‐body magnetic resonance (Philips Achieva, Philips Medical Systems, Best, the Netherlands) with Gadolinium contrast was performed along 2017 and 2018 to patients diagnosed of classical PNH in Autonomous Community of the Region of Murcia, Spain. The image findings were analyzed.Four patients were included (3 men and 1 woman) with median age of 54 years, diagnosed of either PNH (n = 2) and AA/PNH (n = 2). At the time of investigation, mean size clon in granulocytes measured by FLAER was high (90.7 %, range 75.6‐99.3 %) and mean LDH was 341.7 U/L (range 204‐465 U/L; normal range 135‐214 U/L). Only one patient had prior history of thrombosis (Budd‐Chiari syndrome). All patients were treated according to Spanish PNH guidelines, including the use of complement inhibitor Eculizumab.Results:WB‐MRI findings showed unknown bone infarctions in two (50%) patients (patient 1 and patient 3). In patient 1, symptomatic bone infarctions was diagnosed (both femoral condyle and proximal tibias). Patient 3 showed an asymptomatic area of bone edema in distal left radial bone.Summary/Conclusion:Although the sample from our study is very small, it seems that WB‐MRI could be useful to evaluate vascular events, like bone infarctions, even subclinical ones. Our findings are consistent with the only article published to date (Alaskar et al. Sci Rep. 2018; 8: 13458). Further studies are needed to test the effectiveness of WB‐MRI in the detection of vascular events and their relationship as a thrombotic event in PNH.imageimage
PB1878 β‐CATENIN IS AN EFFECTOR OF THE CROSS‐TALK BETWEEN CLL B‐CELLS AND THE MICROENVIRONMENT
Authors: Ouriemmi S. , Varin‐Blank N. , Ledoux D. , Gardano L. 
Published: June 2019

Background:CLL is a lymphoproliferative disorder characterized by the accumulation of CD5+ B cells in the blood and lymphoid organs (bone marrow, lymph nodes and spleen). This accumulation is mainly due to an increased survival and proliferation of tumor B cells within the lymphoid organs through interaction with the microenvironment as isolated B‐CLL cells rapidly undergo apoptosis in vitro. In the quest for the molecular basis of the communication between B‐CLL cells and the microenvironment, we focused our attention on β‐catenin that participates to the stabilization of cell‐cell contacts and signal transduction. Cytosolic β‐catenin level is maintained low following its phosphorylation that triggers the degradation by the proteasome. Several molecules, such as Wnt ligands inhibit β‐catenin phosphorylation and induce its stabilization.Aims:The aim of our study is to evalute the role of β‐catenin in the cross talk between CLL B cells and the microenvironnement.Methods:Co‐cultures of primary B‐CLL cells and human bone marrow stromal cells line (HS‐5).Results:Using a model of co‐culture of primary B‐CLL cells and human marrow stromal cells we observed that the co‐culture rapidly stabilizes β‐catenin in B‐CLL cells. Since in B‐CLL cells the pathway linked to antigen stimulation is essential for survival, we explored whether β‐catenin stabilization involve BCR pathway effectors. Interestingly, we observed that the inhibition of the kinase BTK hampers β‐catenin stabilization in presence of stromal cells. Concomitant with the participation of BTK, a phosphatase activity is involved in β‐catenin stabilization. We are currently addressing whether and how the stabilization of β‐catenin regulates cell survival and the interactions between B‐CLL and stromal cells.Summary/Conclusion:Our results will convey new elements to describe the cross‐talk between tumor cells and the microenvironment to define new targets that interfere with this dialogue as alternative therapeutic strategies.
PB1916 MIRNAS AND ANGIOGENIC FACTORS IN PLASMA BEFORE AND AFTER TKI TREATMENT IN CML PATIENTS
Authors: Litwińska Z. , Łuczkowska K. , Sobuś A. , Rogińska D. , Pius‐Sadowska E. , Paczkowska E. , Gniot M. , Lewandowski K. , Helbig G. , Machaliński B. 
Published: June 2019

Background:The differences of an angiogenic potential between clinical phases of chronic myeloid leukemia (CML), point at the significance of neovascularisation in CML pathogenesis. Tyrosine kinase inhibitors (TKI), possessing antiangiogenic properties and microRNAs (miRNAs), implicated in governing angiogenesis, both seem vital in this process.Aims:In this study, we aimed to investigate how TKI treatment affects angiogenesis‐related miRNAs expression and angiogenic factors concentration in plasma of CML patients.Methods:Peripheral blood plasma samples were obtained from CML patients at the diagnosis (n = 23) and during TKI treatment (n = 12). Quantitative assessment of the expression of miRNA‐126‐3p, miRNA‐150‐5p and miRNA‐21‐3p was performed with qRT‐PCR. Concentrations of selected angiogenic factors in plasma (Angiogenin, bFGF, Endostatin,aFGF, PDGF‐AA, PIGF, Thrombospondin‐2, VEGF‐D, Angiopoietin‐1, VEGF) were assessed using multiplex fluorescent bead‐based immunoassays (Luminex Corporation).Results:MiRNA‐150‐5p and miRNA‐21‐3p expression was evidently higher in the treatment group (p = 0,001 and p = 0,03, respectively). PDGF‐AA concentration in newly diagnosed patients with CML was higher than in already treated patients (942,84 pg/ml vs 512,5 pg/ml, p = 0,02), similarly to VEGF (262,82 pg/ml vs 27 pg/ml, p = 0,03).Summary/Conclusion:TKI treatment affects miRNAs expression and angiogenic factors concentration in plasma of CML patients. MiRNAs could serve as biomarkers in monitoring CML progression and drug response, however, their exact role in CML‐related angiogenesis remains to be further elucidated.