Posters

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POSTERS PRESENTATION _ LUNCHTIME, FRIDAY, 3RD OCTOBER

01. HEMOPHILIA B AND RARE COAGULATION DISORDERS

PO.01.01 – 149 CELL THERAPY MODEL IN HEMOPHILIA B BY GENETICALLY MODIFIED KERATINOCYTES BY LENTIVIRAL VECTORS TO PRODUCE HUMAN CLOTTING FACTOR IX. A. R. JALOMA-CRUZ

PO.01.02 – 161 INFLUENCE OF APTT REAGENTS ON THE 1-STAGE CLOTTING ASSAY ACTIVITY IN HUMAN PLASMA DETERMINED FOR RECOMBINANT FIX PRODUCTS. P. L. TURECEK

PO.01.03 – 160 DOES THE FACTOR IXA CONTENT OF FACTOR IX PRODUCTS IMPACT FUNCTION, SAFETY AND EFFICACY? P. L. TURECEK

PO.01.04 – 72 ASSOCIATION OF MIR-132 AND MIR-185 GENES METHYLATION AND THEIR EXPRESSION PROFILE WITH RISK CONGENITAL FACTOR XIII DEFICIENCY. A. DORGALALEH

PO.01.05 – 73 DISSEMINATED INTRAVASCULAR COAGULATION A CONTROVERSIAL CLINICAL FEATURE IN CONGENITAL FACTOR XIII DEFICIENCY IN SOUTHEAST OF IRAN. A. DORGALALEH

PO.01.06 – 80 GLOBAL DEVELOPMENT PLAN FOR A DOUBLE VIRUS INACTIVATED FIBRINOGEN CONCENTRATE FOR THE TREATMENT OF CONGENITAL FIBRINOGEN DEFICIENCY. O. HEGENER

PO.01.07 – 88 CORRELATION OF GENOTYPE AND PHENOTYPE IN PATIENTS WITH RARE BLEEDING DISORDERS IN PAKISTAN. N. FATIMA

PO.01.08 – 144 GENOTYPE AND PHENOTYPE RELATIONSHIPS IN 10 PAKISTANI UNRELATED PATIENTS WITH INHERITED FACTOR VII DEFICIENCY. N. FATIMA

02. VON WILLEBRAND FACTOR

PO.02.01 – 95 ELUCIDATION OF THE MECHANISM OF VON WILLEBRAND FACTOR INCORPORATION INTO A FIBRIN NETWORK. L. PELKMANS

PO.02.02 – 99 DEFINING THE MOLECULAR BASIS UNDERLYING THE PHYSIOLOGICAL INTERACTION BETWEEN VON WILLEBRAND FACTOR AND GALECTINS IN NORMAL PLASMA. O. RAWLEY

PO.02.03 – 111 THE INFLUENCE OF RECOMBINANT VON WILLEBRAND FACTOR OF DIFFERENT MULTIMER SIZES ON THE ACTIVITY OF FACTOR VIII IN THROMBIN GENERATION AND CHROMOGENIC ASSAY. S. KNAPPE

PO.02.04 – 158 COMPARISON OF AN RVWF DRUG CANDIDATE HAVING AN INTACT MULTIMER SPECTRUM WITH PDVWF TO PROMOTE PLATELET ADHESION UNDER SHEAR STRESS. P. L. TURECEK

PO.02.05 – 112 FACTOR VIII BINDING CAPACITY AND AFFINITY OF RECOMBINANT VON WILLEBRAND FACTOR OF DIFFERENT MOLECULAR WEIGHT RANGES. S. KNAPPE

PO.02.06 – 133 SINGLE NUCLEOTIDE VARIANTS RS1063857 AND RS1063856 ARE ASSOCIATED WITH INCREASED VWF PLASMA LEVELS. A. H. MUFTI

PO.02.07 – 127 VON WILLEBRAND FACTOR IN RELATION TO CORONARY PLAQUE CHARACTERISTICS AND CARDIOVASCULAR OUTCOME. M. A. H. SONNEVELD

PO.02.08 – 52 PLASMA LEVELS OF ACTIVE VWF ARE INCREASED IN PATIENTS WITH FIRST ST-SEGMENT ELEVATION MYOCARDIAL INFARCTION: A MULTICENTER AND MULTIETHNIC STUDY. B. RUTTEN

PO.02.09 – 159 VISUALIZATION OF VWF-FVIII-COMPLEX FORMATION BY SINGLE MOLECULE IMAGING. P. L. TURECEK

PO.02.10 – 153 MAPPING VWF BINDING REGIONS IN FVIII BY HYDROGEN/DEUTERIUM EXCHANGE (HDX) MASS SPECTROMETRY: LIGHT CHAIN OF FVIIIFC SHOWS DIFFERENTIAL DEUTERIUM UPTAKE ON BINDING TO VWF-D’D3. E. CHHABRA

PO.02.11 – 148 VON WILLEBRAND FACTOR DIRECTLY INTERACTS WITH PROTEIN DISULFIDE ISOMERASE PDI. M. A. BREHM

PO.02.12 – 93 REGULATION OF STIMULATED AND BASAL RELEASE OF WEIBEL-PALADE BODIES BY STXBP1 AND SYNTAXIN-3. R. BIERINGS

03. VON WILLEBRAND DISEASE

PO.03.01 – 75 AN ALTERNATIVE ASSAY (ELISA USING GPIB GAIN-OF-FUNCTION) TO RISTOCETIN INDUCED PLATELET AGGLUTINATION (RIPA) IN THE DIAGNOSIS OF TYPE 2B VON WILLEBRAND. F. STUFANO

PO.03.02 – 77 TOWARDS PATIENT TAILORED THERAPY IN VON WILLEBRAND DISEASE PATIENTS IN THE PERIOPERATIVE SETTING. H. C. A. M. HAZENDONK

PO.03.03 – 97 VON WILLEBRAND DISEASE IN POPULATION OF WESTERN MEXICO: BLOOD GROUP IMPACT ON DIAGNOSIS. A. R. JALOMA-CRUZ

PO.03.04 – 102 IN VITRO CHARACTERISATION OF MISSENSE MUTATIONS LOCATED IN THE VON WILLEBRAND FACTOR (VWF) D1 DOMAIN ASSOCIATED WITH QUANTITATIVE VWF DEFICIENCY. D. J. HAMPSHIRE

PO.03.05 – 103 RELATIONSHIP BETWEEN AGE AT FIRST BLEEDING AND BASELINE FACTOR VIII PLASMA LEVELS IN PATIENTS WITH SEVERE FORMS OF VON WILLEBRAND DISEASE (VWD). S. M. SIBONI

PO.03.06 – 106 DEVELOPMENT AND VALIDATION OF ARRAY COMPARATIVE GENOMIC HYBRIDISATION (ACGH) IN VWD GENETIC ANALYSIS. S. J. WEBSTER

PO.03.07 – 116 VON WILLEBRAND DISEASE (VWD) TYPE 1 MUTATION P.R1379C AND P.A1377V SYNERGISTICALLY DETERMINATE A 2M PHENOTYPE IN FOUR UNRELATED ITALIAN PATIENTS. M. T. PAGLIARI

POSTERS PRESENTATION _ LUNCHTIME, SATURDAY, 4TH OCTOBER

PO.03.08 – 164 TYPE 2B/2M VON WILLEBRAND DISEASE MUTATIONS MISFOLD THE VWF A1 DOMAIN. M. AUTON

PO.03.09 – 132 DETERMINATION OF THE VWF SUBTYPES WITH THE RISTOCETIN INDEPENDENT GAIN OF FUNCTION GLYCOPROTEIN 1B INNOVANCE VON WILLEBRAND ACTIVITY ASSAY. C. VAN DUREN

PO.03.10 – 108 PREDICTORS OF VON WILLEBRAND DISEASE DIAGNOSIS IN INDIVIDUALS WITH BORDERLINE VON WILLEBRAND FACTOR PLASMA LEVELS. P. BUCCIARELLI

PO.03.11 – 98 VON WILLEBRAND FACTOR IN ESSENTIAL THROMBOCYTHEMIA IS AFFECTED BY RETICULATED PLATELETS AND BY NON-ADAMTS-13-DEPENDENT PROTEOLYTIC PROCESSING. R. DE CRISTOFARO

04. FACTOR VIII – HEMOPHILIA A

PO.04.01 – 87 LATEST RESULTS FROM THE PUP-GCP CLINICAL TRIAL: A LOW INHIBITOR RATE IN PREVIOUSLY UNTREATED PATIENTS WITH SEVERE HEMOPHILIA A TREATED WITH OCTANATE. S. LOWNDES

PO.04.02 – 83 A CLINICAL STUDY IN PUPS WITH SEVERE HAEMOPHILIA A – IMMUNOGENICITY, EFFICACY AND SAFETY OF TREATMENT WITH HUMAN-CL RHFVIII. S. KNAUB

PO.04.03 – 86 OBSERVATIONAL IMMUNE TOLERANCE INDUCTION RESEARCH PROGRAM (OBSITI) – A MULTIFACETED APPROACH TO EXPLORE IMMUNE TOLERANCE INDUCTION. C. ESCURIOLA-ETTINGSHAUSEN

PO.04.04 – 85 OCTAPLEX® STATE-OF-THE-ART: IMPLEMENTATION OF A NEW NANOFILTER. P. M. SCHULZ

PO.04.05 – 105 WHOLE-EXOME SEQUENCING APPROACH TO STUDY INHIBITOR DEVELOPMENT IN SEVERE HEMOPHILIA A PATIENTS. M. M. GORSKI

PO.04.06 – 110 PATIENTS WITH HEMOPHILIA A (HA) UNDERGOING TOTAL KNEE REPLACEMENT: DOES THROMBIN GENERATION ASSAY (TGA) ADD INFORMATION ON CLOTTING ACTIVATION DURING REPLACEMENT THERAPY. M. E. MANCUSO

PO.04.07 – 121 DENTAL HEALTH AND ORAL HEALTH-RELATED QUALITY OF LIFE IN HEMOPHILIC CHILDREN TREATED IN AN IRANIAN NATIONAL REFERRAL CENTER OF HEMOPHILIA. K. SALEM

PO.04.08 – 155 FAILURE OF CORN TRYPSIN INHIBITOR TO AFFECT THE THROMBIN GENERATION ASSAY IN HEMOPHILIA PLASMA. G. A. YOUNG

PO.04.11 – 124 ORAL HEALTH STATUS AMONG THE HEMOPHILIA PATIENTS IN NORTH OF IRAN. K. SALEM

PO.04.12 – 126 IN VIVO FVIII RECOVERY IN HEMOPHILIA A MICE MODEL WITH INHIBITORS TREATED WITH DIFFERENT FVIII CONCENTRATES: IMPACT OF VWF. M. I. BRAVO

PO.04.13 – 147 SEVERE HAEMOPHILIA A (HA) WITH INHIBITORS AND A NOVEL FVIII SILENT MUTATION: MOLECULAR AND BIOLOGICAL CHARACTERIZATION. A. BORCHIELLINI

PO.04.14 – 151 TARGETING FVIII EXPRESSION TO SINUSOIDAL CELLS TO OVERCOME IMMUNOLOGICAL RESPONSES TO GENE THERAPY FOR HEMOPHILIA A. A. FOLLENZI

PO.04.15 – 152 PHARMACOKINETIC MODELING OF PROPHYLAXIS WITH RECOMBINANT FULL-LENGTH FVIII AND B DOMAIN-DELETED FC-FUSION FVIII. A. GRINGERI

PO.04.16 – 162 DIFFERENCES IN HEMOSTATIC POTENCY OF NATURAL, FULL LENGTH RECOMBINANT FVIII MOLECULE AND B-DOMAIN DELETED RECOMBINANT FVIII. P. L. TURECEK

PO.04.17 – 91 POPULATION PHARMACOKINETICS IN HEMOPHILIA A: TOWARDS INDIVIDUALIZATION OF PERI-OPERATIVE REPLACEMENT THERAPY. H. C. A. M. HAZENDONK

PO.04.18 – 84 PERSONALIZED PROPHYLAXIS WITH HUMAN-CL RHFVIII IN ADULT PATIENTS WITH SEVERE HAEMOPHILIA A. S. KNAUB

PO.04.19 – 79 FACTOR VIII ASSESSMENT USING ONE-STAGE CLOT AND CHROMOGENIC ASSAY IN TRIALS INVESTIGATING PHARMACOKINETICS OF DIFFERENT FVIII PRODUCTS. M. MORFINI

PO.04.20 – 69 THE PURIFICATION OF FACTOR VIII BY THE METHOD NEGATIVE AFFINITY CHROMATOGRAPHY.  N. SHURKO

PO.04.21 – 53 GENERATION AND FUNCTIONAL PROPERTIES OF ENGINEERED ANTIGEN-SPECIFIC HUMAN T REGULATORY CELLS TO TREAT HEMOPHILIA INHIBITOR FORMATION. D. W. SCOTT

PO.04.22 – 58 INFLUENCING FACTORS ON THE FUNCTIONAL LEVEL OF HAEMOPHILIC PATIENTS ASSESSED BY FISH. A. R. KACHOOEI

POSTERS PRESENTATION _ LUNCHTIME, SUNDAY, 5TH OCTOBER

PO.04.23 – 59 VICIOUS CYCLE OF MULTIPLE INVASIVE TREATMENTS IN A HEMOPHILIC INHIBITOR POSITIVE CHILD WITH RESISTANT KNEE FLEXION CONTRACTURE. A CASE REPORT. A. R. KACHOOEI

PO.04.24 – 100 IDENTIFICATION OF GALECTIN-1 AND GALECTIN-3 AS NOVEL BINDING PARTNERS FOR FACTOR VIII.
J. M. O’SULLIVAN

PO.04.25 – 131 ENHANCED FCΓR-DEPENDENT UPTAKE OF BLOOD COAGULATION FACTOR VIII CONTAINING IMMUNE COMPLEXES BY ANTIGEN PRESENTING CELLS. R. B. HARTHOLT

05. THROMBOTIC MICROANGIOPATHIES – ADAMTS-13

PO.05.01 – 66 INHIBITION OF COMPLEMENT-MEDIATED THROMBOTIC MICROANGIOPATHY WITH ECULIZUMAB IMPROVES HEMATOLOGIC AND RENAL OUTCOMES IN ADULT PATIENTS WITH AHUS. S. R. CATALAND

PO.05.02 – 50 COMPLEMENT REGULATORY COMPONENTS IN PATIENTS WITH ATYPICAL HEMOLYTIC UREMIC SYNDROME IN IRAN. A. DORGALALEH

PO.05.03 – 163 EARLY VOLUME EXPANSION FOR MITIGATING SHIGATOXIN-ASSOCIATED HEMOLYTIC UREMIC SINDROME. DATA OF THE NORTH ITALIAN HUS NETWORK. G. ARDISSINO

PO.05.04 – 89 THE ROLE OF ADAMTS-13 IN DVT: IN VITRO CHARACTERIZATION OF ADAMTS-13 SINGLE NUCLEOTIDE VARIANTS IDENTIFIED BY NEXT-GENERATION SEQUENCING IN A GROUP OF ITALIAN DVT PATIENTS. M. T. PAGLIARI

PO.05.05 – 90 ANTI-TAIL MONOCLONAL ANTIBODIES CHANGE THE CONFORMATION OF ADAMTS-13 INTO A HYPERACTIVE STATE. L. DEFORCHE

PO.05.06 – 101 THE CHEMICAL CHAPERONE BETAINE LEADS TO INCREASED SECRETION OF AN ADAMTS-13 SECRETION DEFECT MUTANT. M. UNDERWOOD

PO.05.07 – 107 DENSE GENOTYPING OF IMMUNE-RELATED DISEASE REGIONS FOR THE IDENTIFICATION OF GENETIC RISK FACTORS IN ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA. I. MANCINI

PO.05.08 – 119 THE NATURAL MUTATION ASP173GLY IN THE CATALYTIC SITE OF THE ADAMTS-13 GENE CAUSES A SEVERE UPSHAW-SCHÜLMAN SYNDROME: CLINICAL COURSE, BIOCHEMISTRY. R. DE CRISTOFARO

PO.05.09 – 122 CLINICAL RELEVANCE OF ADAMTS-13-SPECIFIC CIRCULATING IMMUNE COMPLEXES IN ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA. B. FERRARI

PO.05.10 – 135 ENDOCYTOTIC MECHANISMS CONTRIBUTING TO THE INTERNALIZATION OF ADAMTS-13 BY MACROPHAGES. F. VERBIJ

PO.05.11 – 141 DEMONSTRATION OF DISULFIDE BOND FORMATION-MEDIATED INTERACTION OF ADAMTS-13 WITH VWF UNDER SHEAR STRESS. H. ROTTENSTEINER

PO.05.12 – 136 RESTRICTED HLA-DRB1*11 DEPENDENT PEPTIDE PRESENTATION OF ADAMTS-13 BY ANTIGEN PRESENTING CELLS PROVIDES NOVEL INSIGHT INTO THE ETIOLOGY OF ACQUIRED TTP. N. SORVILLO

PO.05.13 – 142 RECOMBINANT HUMAN ADAMTS-13 (BAX 930) IN HEREDITARY TTP: CHARACTERIZATION OF PRODUCT AND PHASE I STUDY DESIGN. H. ROTTENSTEINER

PO.05.14 – 157 VISUALIZATION OF VWF CLEAVAGE BY ADAMTS-13 ON A SINGLE MOLECULE LEVEL. P. L. TURECEK

PO.05.15 – 143 PLATELET-ASSOCIATED OXIDATIVE STRESS AND ADAMTS-13 LEVELS ARE INVERSELY ASSOCIATED WITH SURVIVAL IN SEPTIC SHOCK. R. DE CRISTOFARO

PO.05.16 – 61 RITUXIMAB AS PRE-EMPTIVE THERAPY IN PATIENTS WITH THROMBOTIC THROMBOCYTOPENIC PURPURA AND ANTI-ADAMTS-13 ANTIBODIES: EFFECTIVENESS OF A SINGLE INFUSION. E. BRESIN

PO.05.17 – 140 A NOVEL FLOW-BASED ASSAY REVEALS DISCREPANCIES IN INHIBITOR ASSESSMENT OF TTP PATIENTS COMPARED WITH A CONVENTIONAL CLINICAL STATIC ASSAY. H. ROTTENSTEINER

PO.05.18 – 146 DEVELOPMENT OF A RAT INHIBITOR MODEL TO DEMONSTRATE FEASIBILITY OF RECOMBINANT ADAMTS-13 THERAPY FOR ACQUIRED TTP. H. ROTTENSTEINER

PO.05.22 – 165 ACQUIRED HEMOPHILIA A AND SEVERE SYMPTOMATIC THROMBOCYTOPENIA IN TWO PATIENTS WITH NON-VALVULAR ATRIAL FIBRILLATION TREATED WITH RIVAROXABAN AND DABIGATRAN ETEXILATE RESPECTIVELY. M. SCHIAVONI