57th ASH Annual Meeting and Exposition (December 5-8, 2015): 321. Blood Coagulation and Fibrinolytic Factors: Poster III

Blood Coagulation and Fibrinolytic Factors Oral and Poster Abstracts
Poster

Hall A, Level 2 (Orange County Convention Center)

Engineering a Valve-Regulated Endothelialized Microfluidic Device As an "in Vitro" Bleeding Time for Assessing Global Hemostasis

Yumiko Sakurai, MSc^1,2^*, Elaissa T. Hardy, PhD^1,2^*, Byungwook Ahn, PhD^1,2^*, Shannon L. Meeks, MD³, W. Hunter Baldwin, BS⁴^*, Shawn M Jobe, MD, PhD⁵ and Wilbur A Lam, MD, PhD^1,2

¹Department of Pediatrics, Aflac Cancer Center and Blood Disorders Service of Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA
²Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, GA
³Aflac Cancer Center and Blood Disorders Service, Emory University, Atlanta, GA
⁴Aflac Cancer and Blood Disorders Center, Emory University/Children's Healthcare of Atlanta, Atlanta, GA
⁵Blood Center of Wisconsin, Milwaukee, WI

3486

Plasma Constitutive Serum Amyloid A4 Is Procoagulant and Is Elevated in Venous Thrombosis Patients

John H. Griffin, PhD, Jose A. Fernandez, MD, PhD, Ranjeet Kumar Sinha, PhD, MSc, Darlene J. Elias, MD^* and Hiroshi Deguchi, MD, PhD^*

The Scripps Research Institute, La Jolla, CA

3487

Complete Correction of Severe Canine Hemophilia B By Skeletal Muscle Directed AAV-Based FIX-Padua Gene Therapy in Inhibitor-Prone Dogs

Robert French¹^*, Nicholas Martin²^*, Timothy C. Nichols, M.D.³, Glenn P Niemeyer⁴^*, Clinton D. Lothrop, PhD, DVM⁴^* and Valder R Arruda, MD, PhD⁵

¹University of Pennsylvania, Philadelphia, PA
²Children's Hospital of Philadelphia, Philadelphia, PA
³Francis Owen Blood Research Lab, University of North Carolina, Chapel Hill, NC
⁴University of Alabama, Birmingham, AL
⁵Perelman School of Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA

3488

Thrombin Induces Protease Activated Receptor (PAR)-3/-4 Interaction in Human Podocytes

Sharma Ruchika, MD¹^*, Amanda P Waller, PhD¹^*, Shipra Agrawal, PhD¹^*, Berend H Isermann, MD², William E Smoyer, MD¹^*, Marvin T Nieman, PhD³ and Bryce A. Kerlin, MD¹

¹Center for Clinical and Translational Research, Nationwide Children's Research Institute, Columbus, OH
²Institute of Clinical Chemistry and Pathobiochemistry, Otto-von-Guericke University Medical Center, Magdeburg, Germany
³Pharmacology, Case Western Reserve University, Cleveland, OH

3489

A Single Amino Acid in the Gla Domain Mouse FVIIa Allows Its Binding to the Endothelial Protein C Receptor and Enhances Its Coagulant Activity In Vivo

Giulia Pavani, PhD¹^*, Lacramioara Ivanciu, PhD^1,2, Katherine A. Stafford¹^* and Paris Margaritis, DPhil^1,2

¹The Children's Hospital of Philadelphia, Philadelphia, PA
²University of Pennsylvania, Philadelphia, PA

3490

At Equal Doses FIXFc (Alprolix) Is Not Significanly Better in a Saphenous Vein Bleeding Model Than Recombinant FIX (BeneFix) Seven Days Post-Infusion

Brian Cooley, Ph.D¹^*, David M Mann, PhD²^*, Dougald Monroe, Ph.D.¹^*, Feng-Chang Lin³^*, Paul E. Monahan, MD⁴ and Darrel W. Stafford, PhD⁵

¹Pathology, UNC Chapel Hill, Chapel Hill, NC
²President, Mann Bioconsulting, Gaitherburg, MD
³Biostatistics, UNC Chapel Hill, Chapel Hill
⁴Department of Pediatrics and Gene Therapy Center, University of North Carolina at Chapel Hill, Chapel Hill, NC
⁵Department of Biology, University of North Carolina, Chapel Hill, NC

3491

Frequency and Epitope Specificity of Anti-Factor VIII C1 Domain Antibodies in Acquired and Hereditary Hemophilia Inhibitor Patient Plasma

Joerg Kahle, PhD¹^*, Aleksander Orlowski¹^*, Anja Schmidt, PhD¹^*, Kerstin Brettschneider¹^*, Thomas Klingebiel, MD¹, John F Healey, BS²^*, Ernest T Parker, BA³^*, John (Pete) S Lollar, MD², Marc Jacquemin, MD⁴^*, Manuela Krause, MD⁵^*, Andreas Tiede, MD, PhD⁶, Dirk Schwabe, MD¹^* and Christoph Königs, MD, PhD¹^*

¹Department of Pediatrics, University Hospital Frankfurt, Frankfurt am Main, Germany
²Aflac Cancer and Blood Disorders Center, Department of Pediatrics, Children's Healthcare of Atlanta and Emory University, Atlanta, GA
³Aflac Cancer and Blood Disorders Center, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA
⁴Center for Molecular and Vascular Biology, Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium
⁵Hemostasis, Deutsche Klinik für Diagnostik, Wiesbaden, Germany
⁶Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany

3492

Recombinant FVIIIFc-VWF-XTEN Demonstrates Significant Bioavailability Following Subcutaneous Administration in Hemophilia A Mice

Douglas Drager¹^*, Sue Patarroyo-White¹^*, Hoson Chao¹^*, Ayman Ismail, PhD²^*, Jiayun Liu¹^*, Amy M Holthaus, BS, MS²^*, Ekta Chhabra¹^*, John Kulman, PhD²^*, Volker Schellenberger, PhD³^*, Tongyao Liu⁴^* and Robert Peters, PhD²

¹Biogen Inc, Cambridge, MA
²Hematology Research, Biogen, Cambridge, MA
³Amunix Operating Inc., Mountain View, CA
⁴Hematology Research, Biogen Idec, Cambridge, MA

3493

Thrombomodulin Fusion Proteins Coupled to Human Erythrocytes Demonstrate Anti-Thrombotic and Anti-Inflammatory Activity

Carlos Hipolito Villa, MD, PhD¹^*, Colin F Greineder, MD PhD²^*, Ian Johnston, BS³, Daniel Pan, PhD²^*, Ronald Carnemolla, PhD²^*, Douglas B. Cines, MD⁴, Mortimer Poncz, MD⁵, Don L. Siegel, M.D., Ph.D.,⁴ and Vladimir R Muzykantov, MD PhD²^*

¹Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA
²Institute for Translational Medicine and Therapeutics, University of Pennsylvania, Philadelphia, PA
³Children's Hospital of Philadelphia, Philadelphia, PA
⁴Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA
⁵Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA

3494

Histone H4-Induced Thrombin Generation in Fresh and "Reconstituted" Blood

Saulius Butenas, PhD^*, Shannon M Prior, BS^* and Kenneth G. Mann, PhD

Department of Biochemistry, University of Vermont, Colchester, VT

3495

Lethality of Mouse Tissue Factor Pathway Inhibitor Gene Disruption Is Rescued By Transgenic Human Tissue Factor Pathway Inhibitor Knock in

Robert Pachlinger¹^*, Rudolf Hartmann¹^*, Andrea Kolm¹^*, Erwin Panholzer¹^*, Nadja Ullrich¹^*, Fabian Knofl¹^*, Maria Schuster¹^*, Werner Hoellriegl, DVM¹^*, Friedrich Scheiflinger, PhD² and Michael Dockal¹^*

¹Baxalta Innovations GmbH, Vienna, Austria
²Research & Innovation, Baxalta Innovations GmbH, Vienna, Austria

3496

Antibody Engineering of Anti-TFPI Bypass Therapeutic BAY 1093884: Isotype Selection and Sequence Optimization

Pedro Paz^*, Jinger Xie^* and Fred Aswad^*

Global Biologics Research, Lead Discovery, Bayer HealthCare, San Francisco, CA

3497

Thrombomodulin Gene Mutations Associated with Thromboembolic Diseases

Qingyun Wang¹^*, Liang Tang, Ph.D.²^*, Bei Hu³^*, Yu Hu⁴^* and Han Liu^5,6

¹Institute of Hematology,Union Hospital,, Huazhong University of Science & Technology (HUST), Wuhan, China
²Institute of Hematology, Union hospital,Tongji medical college,Huazhong University of science & technology, Wuhan, China
³Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
⁴Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science & Technology, Wuhan, China
⁵Division of Molecular Oncology, Washington University in St. Louis School of Medicine, Saint Louis, MO
⁶State Key Laboratory of Medical Genomics, Shanghai, China

3498

Pharmacokinetics of Intravenous Infusion of Glu-Plasminogen Concentrate in Patients with Hypoplasminogenemia

Brandon M. Hardesty, MD¹, Stacy Plum, PhD²^*, Karen Thibaudeau, PhD²^*, Martin Lee, PhD³^* and Amy D. Shapiro, MD¹

¹Indiana Hemophilia & Thrombosis Center, Indianapolis, IN
²ProMetic BioTherapeutics, Inc., Rockville, MD
³School of Public Health, University of California, Los Angeles, Los Angeles, CA

3499

Recombinant FVIII and FVIIa Added to Flowing Hemophiliac Blood Co-Localize with Platelet Aggregates Adherent to a Collagen Surface, Promoting Local Fibrin Deposition in Wake-like Patterns Around the Aggregates

Eric F. Grabowski, MD, ScD¹, Bohan Liu, BA²^*, Matthew Gerace, BA²^*, Paul E. Grabowski, Ph.D.³^*, Peter Gunnarsson, Ph.D.⁴^* and Mirella Ezban, Ph.D.⁴^*

¹Pediatric Hematology/Oncology, Massachusetts General Hospital and Harvard Medical School, Boston, MA
²Massachusetts General Hospital/Harvard Medical School, Boston, MA
³University of California, Irvine, Irvine, CA
⁴Novo Nordisk, Maaloev, Denmark

3500

Acute Efficacy of a Novel Anti-Tissue Factor Pathway Inhibitor Antibody BAY 1093884 in Hemophilia A Mouse Severe Tail Bleeding

Yifan Xu¹^*, Maria Koellenberger²^*, Volker Laux²^*, Katalin Kauser¹^* and Derek Sim, PhD¹^*

¹Bayer HealthCare LLC, US Innovation Center, San Francisco, CA
²Bayer Pharma AG, Wuppertal, Germany

3501

A Method for Deriving Pharmacokinetic Constants for Factor VIII and IX from Limited Blood Sampling

Miguel Antonio Escobar, MD¹, Daniel Preston Bond, BS²^*, Madeline Cantini, RN³^* and Krishna Cannon, RN³^*

¹Internal Medicine & Pediatrics. Hematology, University of Texas Health Science Center, Houston, TX
²Dan Bond and Associates, Galveston, TX
³Gulf States Hemophilia and Thrombophilia Center, University of Texas Health Science Center, Houston, TX

3502

A Quantitative Systems Pharmacology Model for the Coagulation Network Describes Biomarker Changes Observed in a Clinical Study with FXa Variant and Predicts Age-Associated Biomarker Variations

Satyaprakash Nayak, PhD¹^*, Dooyoung Lee, PhD²^*, Steven Arkin, MD³, Steven W. Martin, PhD¹^*, Anne C. Heatherington, PhD²^*, William S. Denney, PhD⁴^*, Paolo Vicini, PhD⁵^* and Fei Hua, PhD²^*

¹Pharmacometrics, Global Innovative Pharma - Clinical Pharmacology, Pfizer Inc., Cambridge, MA
²PharmaTherapeutics Clinical Research, Pfizer Inc., Cambridge, MA
³Rare Diseases Research Unit, Pfizer Inc., Cambridge, MA
⁴BioTherapeutics Clinical Research, Pfizer Inc., Cambridge, MA
⁵Pharmacokinetics, Dynamics and Metabolism – New Biological Entities, Pfizer Inc, San Diego, CA

3503

Enhanced Global Hemostatic Potentials with a Bispecific Antibody to Factors IXa and X (ACE910) in the Whole Blood By Rotation Thromboelastometry (ROTEM)

Koji Yada, MD, PhD¹^*, Keiji Nogami, MD. PhD¹^*, Yasuaki Shida, MD, PhD¹^*, Masahiro Takeyama, MD, PhD¹^*, Ryu Kasai, MSc²^* and Midori Shima, MD, PhD¹

¹Department of Pediatrics, Nara Medical University, Kashihara, Japan
²Chugai Pharmaceutical Co., Ltd., Tokyo, Japan

3504

Screening Method for Hepatic Fibrinogen Storage Disease Associated with Congenital Hypofibrinogenemia

Shinpei Arai, MS¹^*, Saki Mukai, BA²^*, Yuka Takezawa, PhD³^*, Mitsutoshi Sugano, PhD³^*, Takayuki Honda, MD, PhD¹^*, Fumihiro Ishida, MD, PhD⁴ and Nobuo Okumura, PhD⁴^*

¹Department of Laboratory Medicine, Graduate School of Medicine, Shinshu University, Matsumoto, Japan
²Department of Health and Medical Sciences, Graduate School of Medicine, Shinshu University, Matsumoto, Japan
³Department of Laboratory Medicine, Shinshu University Hospital, Matsumoto, Japan
⁴Department of Biomedical Laboratory Sciences, Shinshu University, Matsumoto, Japan

3505

The Association Between SERPINC1 C.883G>a and VT in the Chinese Population

Jingdi Liu¹^*, Liang Tang, Ph.D.²^*, Wei Zeng, Ph.D.²^*, Yu Hu¹^* and Han Liu, PhD³

¹Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science & Technology, Wuhan, China
²Institute of Hematology, Union hospital,Tongji medical college,Huazhong University of science & technology, Wuhan, China
³State Key Laboratory of Medical Genomics, Shanghai Institute of Hematology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine,, Shanghai, China

3506

Clinical Use of Ultrasound Superb Micro-Vascular Imaging Technique in Patients with Hemophilia Treated with Standard Radiosynovectomy. Initial Report

Jaroslaw Cwikla¹^*, Pawel Laguna, MD^2,3^*, Piotr Zbikowski⁴^*, Pawel Olejnik⁵^*, Klukowska Anna⁶^*, Katarzyna Pawelec, MD, PhD⁷ and Michal Matysiak, Prof., MD PhD⁸^*

¹Department of Radiology;, University of Varmia and Masuria Olsztyn Poland,, Olsztyn, Poland
²Pediatric Hematology and Oncology, University, Warsaw, Poland
³Children's Hospital In Warsaw, Warsaw, Poland
⁴Department of Orthopedics, Ministry of Internal Affairs and Administration, Warsaw, Poland
⁵Department of Radiology, Institute of Cardiology, Warsaw, Poland
⁶Ped. Hematology and Oncology, Medical, Warsaw, Poland
⁷Department of Pediatrics, Hematology and Oncology, Medical University of Warsaw, Warsaw, Poland
⁸Department of Pediatrics, Hematology and Oncology, Medical University of Warsaw, Warsaw, Poland

3507

Neither Factor VIII Nor the Putative F8B Protein Is Detectable in Human Peripheral Blood Mononuclear Cells

Devi Gunasekera, MBBS¹^*, Kenneth B. Lewis, Ph.D.²^*, Alexis A. Thompson, MD³, Ronald R, Louie, MD⁴, David W. Scott, PhD¹ and Kathleen P. Pratt, Ph.D.¹

¹Department of Medicine, Uniformed Services University of the Health Sciences, Bethesda, MD
²Bloodworks, NW, Seattle, WA
³Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL
⁴University of Washington, Seattle, WA, Mary Bridge Children's Hospital, Tacoma, WA

3508

Liver Tropism and Factor VIII Transgene Expression after Intrauterine Chorionic Villus Stem Cell Transplantation

Maricel G. Miguelino, MD^1,2^*, Priyadarsini Kumar, Ph.D.²^*, Christopher Pivetti, M.Sc.²^*, Lee Lanford, M.A.²^*, Jerry S. Powell, MD¹ and Aijun Wang, PhD²^*

¹Department of Internal Medicine, University of California, Davis, Sacramento, CA
²Department of Surgery, University of California, Davis, Sacramento, CA

3509

Binding of rhFVIIa (LR769) to the Endothelial Protein C Receptor

Jerry A Grandoni, PhD

LFB USA, Framingham, MA

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