66th ASH Annual Meeting: 113. Sickle Cell Disease, Sickle Cell Trait, and Other Hemoglobinopathies, Excluding Thalassemias: Basic and Translational: Poster II

Symposia: Sickle Cell Disease, Sickle Cell Trait, and Other Hemoglobinopathies, Excluding Thalassemias: Basic and Translational Oral and Poster Abstracts
Poster

Research, Sickle Cell Disease, Combination therapy, Fundamental Science, Adult, Drug development, Translational Research, Genetic Disorders, Genomics, Bioinformatics, Clinical Research, Hemoglobinopathies, Health disparities research, Pediatric, Diseases, Immune mechanism, Treatment Considerations, Immunology, Computational biology, Young adult , Biological Processes, Technology and Procedures, Study Population, Multi-systemic interactions, Human, Pathogenesis, Imaging, Animal model, Maternal Health

Halls G-H (San Diego Convention Center)

2480

Blockade of Angiotensin II Signaling Ameliorates Cardiac Fibrosis and Improves Diastolic Dysfunction in Mice with Sickle Cell Anemia

Keara Little, BSc¹^*, Zhenqi Zhu, PhD²^*, Oluwabukola Gbotosho, PhD³ and Punam Malik, MD⁴

¹Cancer and Blood Diseases Institute, Cincinnati Children's Hospital and Medical Center, Mount Orab, OH
²Cincinnati Children's Hospital and Medical Center, Cincinnati
³University of Cincinnati, Cincinnati, OH
⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH

2481

In Vivo Sickle Cell Blood Rheology in Humans with Ventral Tongue Microscopy

Marisa Morakis, BS¹^*, Luojie Huang, MS¹^*, Gregory N McKay, MD, PhD¹^*, Sophie Lanzkron, MD^2,3, Lydia H. Pecker, MD, MS² and Nicholas J Durr, PhD¹^*

¹Biomedical Engineering, Johns Hopkins University, Baltimore, MD
²Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD
³Thomas Jefferson University, Philadelphia, PA

2482

Bruton Tyrosine Kinase Inhibitor Rilzabrutinib Reduces Vaso-Occlusion and Markers of Inflammation and Adhesion in Transgenic Mice with Sickle Cell Disease

Ahmed Daak, MD, PhD, MSc, DPM/MFPM¹, David R. Light, PhD², Fuad Abdulla³^*, Chunsheng Chen, PhD³^*, Julia Nguyen, B.S.⁴^*, Conglin Ruan, B.S.⁴^*, Michael Storek⁵^*, Dimitry Ofengeim⁶^*, Alexandra Hicks⁶^*, Michelle Lee⁵^*, Vincent Mikol⁶^*, Gregory M. Vercellotti, MD³ and John D. Belcher, PhD³

¹Sanofi, Cambridge, MA
²Sanofi Consultant, San Mateo, CA
³Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN
⁴Department of Medicine, Division of Hematology, Oncology and Transplantation, University of Minnesota, Minneapolis, MN
⁵Immunology and Inflammation Research, Sanofi, Cambridge, MA
⁶Research & Development, Sanofi, Vitry-sur-Seine, France

2483

Alterations in the Immune Phenotype of Adaptive Immune Cells in Sickle Cell Disease: A Conventional and Computational Flow Cytometry Analysis

Elisabeth Dovern, MD¹^*, Tim R. Mocking^2,3^*, Mette D. Hazenberg, MD, PhD^4,5^*, Cornelis R van der Torren, MD, PhD⁶, Boukje M. Beuger⁷^*, Robin Van Bruggen, PhD⁷^*, Simon Tol⁷^*, Erik Mul⁸^*, Marjolein van der Pot⁶^*, Arjan A. van de Loosdrecht, MD, PhD^3,9^*, Myrddin Verheij, PhD^2,10^*, Karin Fijnvandraat, MD, PhD⁶^*, Carlijn Voermans, PhD^2,10^*, Bart J. Biemond¹, Costa Bachas, PhD^2,3^* and Erfan Nur, MD, PhD^1,11

¹Department of Hematology, Amsterdam UMC location University of Amsterdam, Amsterdam, Netherlands
²Department of Hematology, Amsterdam UMC location Vrije Universiteit, Amsterdam, Netherlands
³Imaging and Biomarkers, Cancer Center Amsterdam, Amsterdam, Netherlands
⁴Department of Experimental Immunology, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, Netherlands
⁵Sanquin Research, Amsterdam, Netherlands
⁶Department of Pediatric Hematology, Amsterdam UMC, Emma Children's Hospital, Amsterdam, Netherlands
⁷Department of Molecular Hematology, Sanquin Research and Landsteiner Laboratory, Amsterdam, Netherlands
⁸Department of Research Facilities, Sanquin Research and Landsteiner Laboratory, Amsterdam, Netherlands
⁹Department of Hematology, Amsterdam University Medical Center, Free University, Amsterdam, Netherlands
¹⁰Department of Hematopoiesis, Sanquin Research and Landsteiner Laboratory, Amsterdam, Netherlands
¹¹Department of Blood Cell Research, Sanquin Research and Landsteiner Laboratory, Amsterdam, Netherlands

2484

Concurrent GBT1118 & Losartan Treatment Improves Sickle Cell Nephropathy

Guohui Ren, MD, PhD¹^*, Maria Armila Ruiz, MS²^*, Suman Setty³^*, Victor R. Gordeuk, MD⁴ and Santosh L. Saraf, MD⁵

¹University of Illinois At Chicago, CHICAGO, IL
²University of Illinois At Chicago, Chicago, IL
³University of Illinois Chicago, Chicago, IL
⁴Department of Medicine, University of Illinois Hospital, Chicago, IL
⁵Department of Medicine, University of Illinois College of Medicine, Willowbrook, IL

2485

Acquired Mutations in the Mitochondrial Genome As Prognostic Markers in Sickle Cell Disease

Rudra Ray, PhD¹^*, Haiou Li²^*, Nancy Asomaning, MS¹^*, Kang Le, PhD¹^*, Xunde Wang, PhD¹^*, Justin Lack³^*, Yuesheng Li⁴^*, Sayuri Kamimura, MS⁵^*, Zenaide Quezado, MD^5,6 and Swee Lay Thein, MBBS, DSc, FRCP, FRCPath, MRCP, MRCPath¹

¹Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Bethesda, MD
²Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Arlington, VA
³Bioinformatics (NCBR)/Integrated Data Sciences Section(IDSS)/ Research Technology Branch/DIR, NIAID, NIH, National Institutes of Health, Bethesda, MD
⁴DNA SEQUENCING & GENOMICS CORE, NHLBI, NIH, National Institutes of Health, Bethesda, MD
⁵Department of Perioperative Medicine, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD
⁶Sickle Cell Branch, NHLBI, NIH, Bethesda, MD

2486

Inhibition of the Complement Alternative Pathway Attenuates Hemolysis and Preserves Renal Function in a Mouse Model of Sickle Cell Disease

Thiago Trovati Maciel, PhD¹^*, Nabiha Sbeih, MD¹^*, Rachel Rignault-Bricard¹^*, Isabelle Chevalme¹^*, Slimane Allali^1,2^*, Camille Brochier, PhD³^*, Anna Kiialainen⁴^*, Tovo David⁴ and Olivier Hermine, MD, PhD^1,5

¹Institut Imagine - INSERM U1163, Paris, France
²Sickle Cell Center, Pediatrics Department, Necker Hospital for Sick Children, APHP, Paris Cité University, Paris, FRA
³Institut Roche, Boulogne Billancourt, France
⁴F. Hoffmann-La Roche Ltd, Basel, Switzerland
⁵Hematology, Necker Hospital, Greater Paris University Hospitals (AP-HP), Paris, France

2487

Placental Abnormalities in the Humanized Mouse Model of Sickle Cell Disease

Christopher Chambliss, PhD¹, Elizabeth Manci, MD²^*, Elizabeth Eldeiry³^*, Earl Fields Jr., MPH¹^*, Jesse Bueno¹^*, Satheesh Chonat, MD¹ and David R Archer, PhD¹

¹Aflac Cancer and Blood Disorders Center, Emory University, Atlanta, GA
²Univ. of S. AL Children's & Women's Hosp., Mobile, AL
³Georgia Institute of Technology, Atlanta, GA

2488

Pharmacological Evaluation of a First-in-Class Hemoglobin Elevating Agent (HbEA) AND017 in Townes SCD Mouse Model

Gang Huang, PhD^1,2, Jie Bai¹^*, Mingzhe Pan¹^*, Dong Liu, PhD³^*, Min Xiong³^* and Shaojiang Deng, PhD³^*

¹Department of Cell Systems and Anatomy, UT Health San Antonio, San Antonio, TX
²Department of Pathology and Laboratory Medicine, UT Health San Antonio, San Antonio, TX
³Kind Pharmaceuticals, LLC, Redwood City, CA

2489

Defining Mechanistic Responses to Different Vaso-Occlusive Triggers in Sickle Cell Disease: A Step Towards Personalized VOC Reversal Strategies

Lucas F.S. Gushiken, PhD¹^*, Sarah Liane Linguet, MD²^*, Erica M.F. Gotardo, Ph.D.¹^*, Pamela L Brito, PhD¹^*, Flavia C. Leonardo, PhD¹^*, Laetitia Claer, MSc³^*, Slim Azouzi, PhD²^*, Caroline Le Van Kim, PhD²^*, Fernando Ferreira Costa, MD, PhD¹, Sandrine Laurance, PhD²^*, Berengere Khoel, MD, PhD²^* and Nicola Conran, PhD¹

¹Hematology and Transfusion Center, University of Campinas - UNICAMP, Campinas, Sao Paulo, Brazil
²Université Paris Cité and Université des Antilles, INSERM, Biologie intégrée du globule rouge, F-75015, Paris, France
³Université Paris Cité and Université des Antilles, INSERM, BIGR, F-75015 Paris, F97150 Pointe à Pitre, France, Paris, France

2490

8-Oxoguanine DNA Glycosylase 1 Recruits Transcription Factor STAT1 to Promote the Inflammatory Responses in Sickle Cell Disease

Kang Le, PhD¹^*, Zenaide Quezado, MD², Haiou Li³^*, Sayuri Kamimura, MS²^*, Meghann L. Smith⁴^*, Yuki Tahara⁵^*, Yujeong Lee⁵^*, Anna Conrey, NP¹^*, Josie Schultz⁶^*, Thorkell Andresson, PhD⁷^*, Eric T Kool⁵^* and Swee Lay Thein, MBBS, DSc, FRCP, FRCPath, MRCP, MRCPath⁸

¹Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Bethesda, MD
²Department of Perioperative Medicine, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD
³Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Arlington, VA
⁴Clinical Center, National Institutes of Health, Bethesda, MD
⁵Department of Chemistry, Stanford University, Stanford, CA
⁶CCR Protein Characterization Laboratory, National Cancer Institute, NIH, Frederick, MD
⁷CCR Protein Characterization Laboratory, National Cancer Institute, National Institutes of Health, Frederick, MD
⁸Sickle Cell Branch, National Heart, Lung & Blood Institute, Bethesda, MD

2491

Mitapivat-Induced Improvements in RBC Deformability and Membrane Integrity in Patients with Sickle Cell Disease Are Sustained during Extended Therapy

Xunde Wang, PhD¹^*, Yechan Cho, BS¹^*, Nancy Asomaning, MS¹^*, Maureen Lundt, MS¹^*, Anna Conrey, NP¹^*, Britney Kruah, MS¹^*, Amber M Yates, MD²^*, Megan Wind-Rotolo, PhD², Haiou Li³^*, Kang Le, PhD¹^* and Swee Lay Thein, MBBS, DSc, FRCP, FRCPath, MRCP, MRCPath⁴

¹Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Bethesda, MD
²Agios Pharmaceuticals, Inc., Cambridge, MA
³Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Arlington, VA
⁴Sickle Cell Branch, National Heart, Lung & Blood Institute, Bethesda, MD

2492

Long-Term Therapy with GBT021601 and Moderate Chronic Transfusion Therapy Prevent the Development of Hyperalgesia

Justin J Yoo, MD^1,2, Kennedy N Goldsborough, PhD³^*, Britney Hernandez, BS³^*, Ashwin P. Patel, MD, PhD, MPH⁴ and Vivien Sheehan, MD, PhD⁵

¹Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University School of Medicine, Avondale Estates, GA
²Georgia Comprehensive Sickle Cell Center at Grady Health System, Hematology and Medical Oncology, Emory University School of Medicine, Atlanta, GA
³Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA
⁴Department of Pediatrics, Emory University School of Medicine, Atlanta, GA
⁵Emory University School of Medicine, Atlanta, GA

2493

DNA Sensing By Platelets Promotes Acute Chest Syndrome in Sickle Cell Disease

Rikesh K Dubey, PhD¹, Omika Katoch, PhD¹^*, Nicholas Swendrowski, MS¹^* and Prithu Sundd, PhD^1,2

¹Thrombosis and Hemostasis Program, VERSITI Blood Research Institute, Milwaukee, WI
²Medical College of Wisconsin, Division of Hematology and Oncology, Milwaukee, WI

2494

Comparative Analysis of Whole-Brain and Regional Cerebral Blood Flow, Pain Expression, Pain Sensitivity, and Cognitive Function in Adults with Sickle Cell Disease Versus Healthy Controls

Daniel M Sop, MS, BS¹, Yue May Zhang, PhD²^* and Wally R Smith, MD²

¹Sickle Cell Disease program / General Internal Medicine, Virginia Commonwealth University, Glen Allen, VA
²Virginia Commonwealth University, Richmond, VA

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^*signifies non-member of ASH