57th ASH Annual Meeting and Exposition (December 5-8, 2015): 113. Hemoglobinopathies, Excluding Thalassemia – Basic and Translational Science: Poster III

Hemoglobinopathies, Excluding Thalassemia – Basic and Translational Science Oral and Poster Abstracts
Poster

Hall A, Level 2 (Orange County Convention Center)

Point-of-Care Screening for Sickle Cell Disease By a Mobile Micro-Electrophoresis Platform

Ryan Ung¹^*, Yunus Alapan, MS¹^*, Muhammad Noman Hasan, MS¹^*, Megan Romelfanger¹^*, Ping He, MD PhD¹^*, Aaron Tam¹^*, Tolulope Rosanwo, BA¹, Asya Akkus¹^*, Mehmet A Cakar²^*, Kutay Icoz, PhD²^*, Connie M. Piccone, MD¹, Jane A Little, MD¹ and Umut A Gurkan, PhD¹^*

¹Case Western Reserve University, Cleveland, OH
²Abdullah Gul University, Kayseri, Turkey

3380

Hydroxyurea Treatment Is Associated with Elevated Serum Erythropoietin Concentration but Suppressed Global Hypoxic Transcriptional Responses in Sickle Cell Disease

Xu Zhang, PhD¹^*, Wei Zhang, PhD²^*, Binal Shah, PhD³^*, Galina Miasnikova, MD⁴^*, Adelina Sergueeva, MD⁵^*, Tatiana Ammosova, PhD⁶^*, Xiaomei Niu⁷^*, Mehdi Nouraie, MD, PhD⁸, Sergei Nekhai, PhD⁶, Roberto F. Machado, MD⁹^*, Joe G.N. Garcia¹⁰^*, Mark T. Gladwin, MD¹¹, Oswaldo L Castro, MD¹² and Victor R. Gordeuk, MD¹

¹Comprehensive Sickle Cell Center, Section of Hematology/Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, IL
²Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago
³Comprehensive Sickle Cell Center, Section of Hematology/Oncology, Department of Medicine, University of Illinois at Chicago, Chicago
⁴Chuvash Republic Clinical Hospital, Cheboksary, Russia
⁵Cheboksary Children's Hospital, Cheboksary, Russia
⁶Center for Sickle Cell Disease, Howard University, Washington, DC
⁷Center for Sickle Cell Disease, Howard University, Washington
⁸Center For Sickle Cell Disease, Howard University, Washington, DC
⁹Division of Pulmonary, Critical Care, Sleep and Allergy Medicine, Department of Medicine, University of Illinois at Chicago, Chicago, IL
¹⁰College of Medicine, University of Arizona, Tucson
¹¹Heart, Lung and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA
¹²Center for Sickle cell Disease, Howard University, Washington, DC

3381

Characterizing Cellular Interactions Contributing to Vaso-Occlusion in Patients with Sickle Cell Disease Utilizing a Novel Endothelialized Microfluidic Device

Margo Renee Rollins, MD¹, Byungwook Ahn, PhD²^*, Yumiko Sakurai, MSc²^* and Wilbur A Lam, MD, PhD^2,3

¹Department of Pediatric Hematology Oncology, Aflac Cancer and Blood Disorder Service of Children's Healthcare of Atlanta, Atlanta, GA
²Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, GA
³Department of Pediatrics, Aflac Cancer Center and Blood Disorders Service of Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA

3382

Protection of Microvascular Liver Perfusion with 5-Hydroxymethylfurfural in Sickle Cell Disease Mice Following Hypoxia-Induced Vasoocclusion

Michael Wright¹^*, Derek Sim¹^*, Magdalena Alonso-Galicia¹^*, Katalin Kauser²^* and Keith Abe¹^*

¹Hematology Research, Bayer HealthCare LLC, US Innovation Center, San Francisco, CA
²Bayer HealthCare LLC, US Innovation Center, San Francisco, CA

3383

Co-Administration of Hydroxyurea and a Specific AKT2 Inhibitor Has Beneficial Effects on Acute Vaso-Occlusive Events and Survival in Sickle Cell Disease Mice

Jing Li, Ph.D.¹, Andrew Barazia¹^*, Kyungho Kim, PhD¹^* and Jaehyung Cho, Ph.D.^1,2

¹Department of Pharmacology, University of Illinois College of Medicine, Chicago, IL
²Department of Anesthesiology, University of Illinois College of Medicine, Chicago, IL

3384

Analysis of Hemodynamic Changes and Bold Signals of Sickle Cell Disease Patients during Desaturation

Julie Coloigner †, PhD¹^*, Chau Vu †¹^*, Matt Borzage, PhD^2,3,4^*, Adam M Bush, MS^5,6^*, Natasha Lepore, PhD⁷^*, Thomas D. Coates, MD⁶ and John C Wood, MD, PhD⁸^*

¹Children's hospital, Los Angeles, CA
²Department of Radiology, Children's hospital, Los Angeles, CA
³Department of Neonatology, Children's hospital, Los Angeles, CA
⁴Keck School of Medicine, USC, Los Angeles, CA
⁵Biomedical Engineering, University of Southern California, Los Angeles, CA
⁶Hematology Oncology, Children's Hospital Los Angeles, Los Angeles, CA
⁷CIBORG, Department of Radiology, Children's hospital, Los Angeles, CA
⁸Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, CA

3385

Hemin-Induced Mast Cell-Extracellular Traps Impart Resistance to Therapy in a Sickle Microenvironment

Huy Tran, BS^*, Ritu Jha, M.sc^*, Julia Nguyen, BS^*, Sarita Jarrett, High School^*, Jonathan Rodriguez, Undergraduate^*, Aditya Mittal, High School^*, Jianxun Lei, PhD^* and Kalpna Gupta, PhD

Vascular Biology Center, Division of Hematology, Oncology & Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN

3386

Investigation of Two Combination HbF Induction Regimens, RN-1 and Hydroxyurea Versus RN-1 and Decitabine, in a Humanized Sickle Cell Mouse Model

Ramasamy Jagadeeswaran, PhD.¹^*, Yash Agarwal¹^*, Vinzon Ibanez²^*, Maria Armila Ruiz²^*, Kestis Vaitkus²^*, Joseph DeSimone, PhD², Donald Lavelle, PhD² and Angela Rivers, MD, PhD¹

¹Department of Pediatrics, University of Illinois at Chicago, Jesse Brown VA Medical Center, Chicago, IL
²Department of Medicine, University of Illinois at Chicago, Jesse Brown VA Medical Center, Chicago, IL

3387

Delta-Aminolevulinate Preferentially Induces Gamma-Globin Expression in Erythroid Cells through Activation of NRF2 Stress Response

Li Liu, PhD, BS, MS¹^*, Christina Torres²^*, Baolin Kang, PhD²^* and Betty Pace, MD^2,3

¹Department of Biological Sciences, University of Texas at Dallas, Richardson, TX
²Department of Pediatrics, Georgia Regents University, Augusta, GA
³Biochemistry and Molecular Biology, Georgia Regents University, Augusta, GA

3388

Polymorphisms Associated with the Arab-Indian Haplotype of Sickle Cell Anemia Are Candidate Fetal Hemoglobin Gene Modulators

Vinod Vathipadiekal, PhD¹^*, Abdulrahman Alsultan, MD², John Farrell, PhD³^*, A.M Al-Rubaish⁴^*, Fahad Al-Muhanna⁴^*, Z Naserullah⁵^*, A Alsuliman⁶^*, P.K. Patra⁷^*, Jacqueline Milton⁸^*, Lindsay Farrer⁹^*, David H.K. Chui, MD¹⁰, Amein Al-Ali⁴^*, Paola Sebastiani, PhD¹¹^* and Martin H. Steinberg, MD¹

¹Boston University School of Medicine, Boston, MA
²Department of Pediatrics and Sickle Cell Disease Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia
³Boston University Medical School, Boston, MA
⁴University of Dammam, Dammam, Saudi Arabia
⁵King Faisal University & Departments of Pediatrics, Dammam, Saudi Arabia
⁶King Faisal University, Al-Ahsa, Saudi Arabia
⁷Pt. J.N.M. Medical College, Raipur, India
⁸Department of Biostatistics, Boston University School of Public Health, Boston
⁹Boston University School of Medicine, Boston
¹⁰School of Medicine, Boston University, Boston, MA
¹¹Department of Biostatistics, Boston University, Boston, MA

3389

Circulating Invariant Natural Killer T Cell Subsets Are Functionally Preserved in Splenectomized Children with Sickle Cell Disease Compared to Patients with Large Spleen

Mohamed-Rachid Boulassel, DEMS, PhD¹^*, Abeer Al-Zubaidi²^*, Ismail Beshlawi³^*, Zahra Al-Qarni²^*, Nidaa Al-Naamany²^*, Shoaib Al-Zadjali⁴^*, Sulaiman Al-Hashmi⁵^*, Hamad Khan⁶^*, Mohammed Al Huneini⁶^*, Khalil Al Farsi⁶, Murtadha K. Al-Khabori, BSc, MD, FRCPC, ABIM, M.Sc, CIP⁷, Salam Al-Kindi⁶^* and Yasser Wali³^*

¹Allied Health Sciences and Haematology, Sultan Qaboos University Hospital, Muscat, Oman
²Sultan Qaboos University, College of Medicine and Health Sciences, Muscat, Oman
³Department of Child Health, Sultan Qaboos University Hospital, College of Medicine and Health Sciences, Muscat, Oman
⁴Department of Haematology, Sultan Qaboos University Hospital, College of Medicine and Health Sciences, Muscat, Oman
⁵Department of Genetics, Sultan Qaboos University, College of Medicine and Health Sciences, Muscat, Oman
⁶Department of Haematology, Sultan Qaboos University Hospital, College of Medicine and Health Sciences, Muscat, Oman
⁷Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman

3390

miRNA-146a, miRNA-203a, and miRNA-223 Modulate Inflammatory Response in LPS- Acute Lung Injury in Sickle Cell Transgenic Mice

Roger Barbosa Ferreira^*, Regiane Ferreira, MSc, BSc^*, Dulcineia Martins Albuquerque, PhD^*, Fernando F Costa, PhD, MD and Carla Fernanda Franco-Penteado, BSc, PhD

Hematology and Hemotherapy Center, University of Campinas, Campinas, SP, Brazil

3391

Effect of PSI697, a Small Molecule Inhibitor of P-Selectin, in the Townes Model of Sickle Cell Disease

Reema Jasuja^*, Sunita Patel Hett^*, Neelu Kaila^* and Debra Pittman

Pfizer Inc., Cambridge, MA

3392

Sickle Cell Disease Patients Show Sensitization of Myelinated Sensory Nerve Fibers

Nicholas Spornick, MS^1,2^*, Luis E. Almeida, MD, PhD²^*, Barbara Speller-Brown, DNP³^*, Deepika Darbari, MD⁴, Kevin Jackson, BS²^*, Julie Finkel, MD²^* and Zena Quezado, MD²^*

¹Frank H. Netter School of Medicine, Quinnipiac University, Hamden, CT
²Children's Research Institute, Children's National Health System, Washington, DC
³Children's National Health System, Washington, DC
⁴Pediatric Hematology and Oncology/ NHLBI Pulmonary and Vascular Medicine, Children's National Medical Center / National Heart, Lung, and Blood Institute, Washington, DC

3393

Genetic Modifiers Influencing the Development of Albuminuria in Children with Sickle Cell Anemia

Beverly A Schaefer, MD¹, Jonathan M Flanagan, PhD², Banu Aygun, MD³, Ofelia A. Alvarez, MD⁴, Stephen C Nelson, MD⁵, Kerri A Nottage, MD, MPH⁶, Carla W Roberts, MD⁷^*, Thad A. Howard, MS¹^*, Barry R Davis, MD PhD⁸^* and Russell E. Ware, MD, PhD⁹

¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH
²Baylor College of Medicine, Houston, TX
³Cohen Children's Medical Center of New York, New Hyde Park, NY
⁴Univ. of Miami School of Med., Miami, FL
⁵Children’s Hospitals and Clinics of Minnesota, Minneapolis, MN
⁶St. Jude Children's Research Hospital, Memphis, TN
⁷University of South Carolina, Columbia, SC
⁸University of Texas School of Public Health, Houston, TX
⁹Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

3394

Prophylactic Nrf2 Activation Halts Progression of the Severe Adult Phenotype of Sickle Cell Disease in Transgenic Mice

Samit Ghosh, PhD¹, Chibueze A. Ihunnah, PhD²^*, Rimi Hazra, PhD²^*, Amma T. Owusu-Ansah, MD³, Jason M Hansen, PhD⁴^*, David R. Archer, PhD⁵ and Solomon Fiifi Ofori-Acquah, PhD⁶

¹Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA
²Department of Medicine, University of Pittsburgh, Pittsburgh, PA
³Division of Hematology/Oncology, Department of Medicine, Center for Translational and International Hematology, University of Pittsburgh, Pittsburgh, PA
⁴Brigham Young University, Provo, UT
⁵Emory University, Atlanta, GA
⁶Division of Hematology/Oncology, Department of Medicine, Center for Translational and International Hematology, University of Pittsburgh, Pittsburgh, PA

3395

Dissection of the Expression Patterns of Hydroxyurea Transporters during Erythropoiesis and Myelopoiesis

Aisha L Walker, PhD¹ and Solomon Fiifi Ofori-Acquah, PhD²

¹Division of Pulmonary, Allergy, and Critical Care Medicine, Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA
²Division of Hematology/Oncology, Department of Medicine, Center for Translational and International Hematology, University of Pittsburgh, Pittsburgh, PA

3396

Hemolysis Controls Pro-Inflammatory Monocyte Priming Via Induction of and Interaction with Circadian Clock Protein Rev-Erbα in Sickle Cell Disease

Julia Brittain, PhD¹, Itia Lee¹^* and Ciprian Anea, MD²^*

¹Vascular Biology Center, Georgia Regents University, Augusta, GA
²Georgia Regents University, Augusta, GA

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^*signifies non-member of ASH