66th ASH Annual Meeting: 330. Vascular Biology, Thrombosis, and Thrombotic Microangiopathies: Basic and Translational: Poster II

Symposia: Vascular Biology, Thrombosis, and Thrombotic Microangiopathies: Basic and Translational Oral and Poster Abstracts
Poster

Research, Bleeding and Clotting, Antibody Therapy, Sickle Cell Disease, Fundamental Science, Adult, Clinical Practice (Health Services and Quality), Drug development, Translational Research, Epidemiology, Genetic Disorders, Platelet disorders, Clinical Research, Hemoglobinopathies, Diseases, Immune mechanism, Thrombocytopenias, Treatment Considerations, Biological therapies, Immunology, Thrombotic disorders, Real-world evidence, Adverse Events, Biological Processes, Study Population, Human

Halls G-H (San Diego Convention Center)

2602

Interpreting High Levels of Unfolded Von Willebrand Factor in Patients with the Antiphospholipid Syndrome

Romy De Laat-Kremers, PhD¹^*, Shengshi Huang^2,3^*, Hugo Ten Cate, MD^4,5, Marisa Ninivaggi²^*, Bas De Laat^2,6 and Katrien M.J. Devreese, PhD, MD^7,8^*

¹Department of Data Analysis and Artificial Intelligence, Synapse Research Institute, Washington, MD
²Department of Functional Coagulation, Synapse Research Institute, Maastricht, Netherlands
³Department of Biochemistry, Maastricht University, Maastricht, Netherlands
⁴Departments of Internal medicine and Biochemistry, Maastricht University Medical Center, Maastricht, Netherlands
⁵Cardiovascular Research Institute Maastricht (CARIM) and department of Internal medicine, Maastricht University Medical Center, Maastricht, Netherlands
⁶Department of Data Analysis and Artificial Intelligence, Synapse Research Institute, Maastricht, Netherlands
⁷Department of Diagnostic Sciences, Ghent University, Ghent, Belgium
⁸Coagulation Laboratory, Ghent University Hospital, Ghent, Belgium

2603

Citrullination in the C-Terminus of Tissue Factor Pathway Inhibitor-Alpha Impairs the Inhibition of Factor V Activation, and the Synergy with Protein S in Factor Xa Inactivation

Bryan Bouwens, MSc¹^*, Elke Magdeleyns¹^*, Stijn Agten¹^*, Stella Thomassen¹^*, Dennis Suylen¹^*, Tilman M. Hackeng, PhD¹ and Rory R. Koenen²^*

¹Cardiovascular Research Institute Maastricht (CARIM), Dpt. of Biochemistry, Maastricht university(UM), Maastricht University Medical center (MUMC+), Maastricht, Netherlands
²CARIM - Biochemistry, Maastricht University, Maastricht, Netherlands

2604

Tissue Kallikrein 1 and Alternative Complement Pathway Activation in Hematopoietic Stem Cell Transplant-Associated Thrombotic Microangiopathy

Sarah E Sartain, MD¹, Paula Jacobi²^*, Virginie Qian³^*, Mrinal Ranjan, MPH⁴^*, Saleh Bhar, M.D.⁵^*, Ang Li, MD, MS⁴ and Andrew Yee, PhD.⁶^*

¹Department of Pediatrics, Section of Hematology-Oncology, Baylor College of Medicine/Texas Children's Hospital, Houston, TX
²Department of Pediatrics, Section of Hematology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX
³Southern Illinois University, Carbondale, IL
⁴Baylor College of Medicine, Houston, TX
⁵Department of Pediatrics, Divisions of Hematology, Oncology, and Critical Care Medicine, Cell Therapy and Bone Marrow Transplantation, Baylor College of Medicine/Texas Children's Hospital, Houston, TX
⁶Department of Pediatrics, Baylor College of Medicine, Houston, TX

2605

Complement Activation As a Biomarker for Platelet Activating Antibodies in Heparin-Induced Thrombocytopenia (HIT)

Sooho S Myoung¹^*, Samuel Francis, MD², Jonah Chen³^*, Grace M Lee⁴, Lubica Rauova, MD, PhD⁵^*, Mortimer Poncz, MD⁵^*, Douglas B. Cines, MD⁶, Maragatha Kuchibhatla⁴^*, Sanjay Khandelwal, PhD⁷^* and Gow Arepally³

¹Medical Scientist Training Program, The Ohio State University, Columbus, OH
²Emergency Medicine, Duke University School of Medicine, Durham, NC
³Division of Hematology, Duke University Medical Center, Durham, NC
⁴Duke University Medical Center, Durham, NC
⁵Division of Hematology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA
⁶Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA
⁷Duke University School of Medicine, Durham, NC

2606

The PF4-Centric Immunothrombotic Disorders, HIT, Vitt and APS, Lead to Complement Activation Responsive to Complement-Blocking Therapeutics

Amrita Sarkar, PhD¹, Conroy O Field, MS^2,3, Keith R. McCrae, MD⁴, Thomas L. Ortel, MD, PhD⁵, Gow Arepally, MD⁶, Roxanne Cofiell⁷^*, Sung-Kwon Kim⁷^*, Lubica Rauova, MD, PhD^1,8 and Mortimer Poncz, MD^3,8^*

¹Division of Hematology, Children's Hospital of Philadelphia, Philadelphia, PA
²Perelman School of Medicine Pharmacology Graduate Group, University of Pennsylvania, Philadelphia, PA
³Division of Hematology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA
⁴Department of Cardiovascular and Metabolic Sciences, Lerner Research Institute, The Cleveland Clinic Foundation, Cleveland, OH
⁵Division of Hematology, Duke University School of Medicine, Durham, NC
⁶Duke University School of Medicine, Durham, NC
⁷Alexion, AstraZeneca Rare Diseases, New Haven, CT
⁸Department of Pediatrics, University of Pennsylvania, Philadelphia, PA

2607

αIIbβ3-Dependent Platelet Procoagulant Activity Promotes Pulmonary Arterial Thrombosis Triggered By Blood-Borne Tissue Factor

Sarah E. Tashbook¹^*, Tomasz W. Kaminski, PharmD²^*, Prithu Sundd, PhD^2,3 and Tomasz Brzoska, PhD^1,4

¹Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, University of Pittsburgh School of Medicine, Pittsburgh, PA
²Thrombosis and Hemostasis Program, VERSITI Blood Research Institute and Blood Center of Wisconsin, Milwaukee, WI
³Division of Hematology and Oncology, Medical College of Wisconsin, Milwaukee, WI
⁴Division of Hematology/Oncology, University of Pittsburgh School of Medicine, Pittsburgh, PA

2608

Red Blood Cell-Derived Particles Induce Endothelial Damage Via the Alternative Complement Pathway

Kim-Anh Nguyen-Peyre¹^*, Marie-Sophie Meuleman, MD²^*, Gwellaouen Bodivit³^*, Christian Kassasseya, MD^4,5^*, Xavier Decrouy, PhD⁶^*, Benoit Vingert, PhD^7,8^*, Laura Bencheikh, PhD⁵^*, John Lambris, PhD^9,10^*, France Pirenne, MD, PhD^11,12, Lubka T. Roumenina, PhD^13,14^* and Pablo Bartolucci, MD, PhD^15,16^*

¹INSERM U955 IMRB Team Pirenne, French Blood Establishment, Créteil Mondor, Créteil, FL, France
²INSERM, Centre de Recherche des Cordeliers, Paris France, Paris, FRA
³INSERM U955, IMRB, team Pirenne, French Blood Establishment, Créteil France, CRETEIL, FRA
⁴Henri Mondor Hospital - Emergency Department, Creteil, FRA
⁵INSERM U955, IMRB, team Pirenne, Créteil France, Créteil, France
⁶INSERM U955, IMRB, Imaging platform, Créteil France, Créteil, -- None selected --, France
⁷INSERM-U955, EFS, Institut Mondor, Université Paris-Est Créteil, Team 2 Transfusion et maladies du globule rouge, Laboratoire d´Excellence GR-Ex, Créteil, France
⁸Etablissement Français du Sang, Hôpital Henri Mondor, Creteil, FRA
⁹Department of Pathology & Laboratory Medicine, Philadelphia, PA
¹⁰University of Pennsylvania School, Philadelphia, PA
¹¹Transfusion et maladies du globule rouge - Equipe Pirenne, Institut Mondor de Recherche Biomedicale (IMRB), Creteil, France
¹²Etablissement Francais Du Sang - Université Paris-Est Créteil Hospital, Creteil, France
¹³Sorbonne Université, Paris, France
¹⁴Centre de Recherche des Cordeliers, INSERM, Paris, France
¹⁵Hematology Department, Sickle Cell Referral Center, Department of Internal Medicine, Henri-Mondor University Hospital- UPEC, AP-HP, Créteil, France
¹⁶Université Paris-Est Créteil, INSERM U955, IMRB, Laboratory of excellence LABEX GRex, Créteil, France

2609

Inflammasome Cytokine Secretion in Heparin-Induced Thrombocytopenia Is Supported By Platelet-Derived Mediators and Complement

James V Michael, PhD¹, Sanjay Khandelwal, PhD²^*, Antonios Tawk, MD¹^*, Sophia Lazar, PhD¹^*, Gow Arepally³ and Steven E McKenzie, MD, PhD¹

¹The Cardeza Foundation for Hematologic Research, Thomas Jefferson University, Philadelphia, PA
²Duke University School of Medicine, Durham, NC
³Division of Hematology, Duke University Medical Center, Durham, NC

2610

Exploration of Purinergic Signaling Pathways As a Mechanism of Platelet Activation in Antiphospholipid Syndrome

Bruna Cardoso Jacintho¹^*, Guilherme Leonardi, PhD²^*, Bruna Mazetto^3,4^*, José Oliveira, PhD⁵^*, Fabiola Zakia Monica²^*, Erich Vinicius De Paula, MD, PhD⁶ and Fernanda A. Orsi, MD, PhD⁷^*

¹School of Medical Sciences, University of Campinas (UNICAMP), Campinas, AC, Brazil
²Department of Translational Medicine (Pharmacology), University of Campinas (UNICAMP),, Campinas, Brazil
³University of Campinas (UNICAMP), Campinas, BRA
⁴Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI
⁵University of Campinas (UNICAMP), Campinas, LA, Brazil
⁶Hematology and Hemotherapy Center, University of Campinas, Campinas, Brazil
⁷Department of Pathology, School of Medical Sciences, Unicamp, Campinas, Brazil

2611

Anti-Phospholipid Syndrome (APS) Antibody (Ab)-Induced Thrombosis Can be Blocked By Platelet Factor 4 (PF4)-Directed Abs: A Novel Therapeutic Approach for APS?

Amrita Sarkar, PhD¹, Santosh K Yadav, PhD¹^*, Conroy O Field, MS^2,3, Kandace Gollomp, MD^3,4^*, Keith R. McCrae, MD⁵, Thomas L. Ortel, MD, PhD⁶, Yves Gruel⁷^*, Jérôme Rollin⁷^*, Gowthami M Arepally, MD⁸^*, Lubica Rauova, MD, PhD^3,4^*, Douglas B. Cines, MD⁹ and Mortimer Poncz, MD^3,4^*

¹Division of Hematology, Children's Hospital of Philadelphia, Philadelphia, PA
²Perelman School of Medicine Pharmacology Graduate Group, University of Pennsylvania, Philadelphia, PA
³Division of Hematology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA
⁴Department of Pediatrics, University of Pennsylvania, Philadelphia, PA
⁵Department of Cardiovascular and Metabolic Sciences, Lerner Research Institute, The Cleveland Clinic Foundation, Cleveland, OH
⁶Division of Hematology, Duke University School of Medicine, Durham, NC
⁷Department of Hemostasis, Université de Tours, Tours, France
⁸Division of Hematology, Duke University Medical Center, Durham
⁹Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA

2612

Dilute Russel Viper Venom Time (DRVVT) Ratio Predicts the Identification of Triple Positive Antiphospholipid Antibody Profile

Jing Yuan Tan, MBBS¹, Wan Hui Wong¹^*, May Anne Cheong, MBBS, FRCPath (UK)¹^*, Cecilia Cheng Lai Ngan, MBBS, FRCPA²^*, Wenshan Liu¹^*, Choo Kheng Tan¹^*, Hong Kheng Neo¹^*, Lai Heng Lee, MBBS, FRCP(Edin)¹^*, Heng Joo Ng, MBBS, FRCPath(UK)¹^* and Chuen Wen Tan, MBBCh BAO, FRCPath (UK)¹^*

¹Department of Haematology, Singapore General Hospital, Singapore, Singapore
²Department of Microbiology, Singapore General Hospital, Singapore, Singapore

2613

Real-World Effectiveness of Ravulizumab Among Adults with Atypical Hemolytic Uremic Syndrome (aHUS) Who Switched to Ravulizumab within 3 Months of Eculizumab Treatment: A Physician Panel-Based Chart Review Study (aHUS IMPACT)

Shruti Chaturvedi, MBBS¹, Yan Wang²^*, Moh-Lim Ong²^*, Arpita Nag³^*, Rui Song⁴^*, Lynn Huynh, MPH⁴^*, Jordan A. Burdeau⁴^*, Mei Sheng Duh⁴^* and Ramy Magdy Hanna⁵^*

¹Division of Hematology, Department of Medicine, Johns Hopkins University, BALTIMORE, MD
²Alexion, AstraZeneca Rare Disease, Boston, MA
³Alexion, AstraZeneca Rare Disease, Boston, MA, USA, Boston, MA
⁴Analysis Group, Inc., Boston, MA
⁵University of California, Irvine, CA

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^*signifies non-member of ASH