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311.O2.8 Thrombotic Thrombocytopenic Purpura and Heparin Induced Thrombocytopenia

Monday, December 8, 2008: 7:00 AM-9:00 AM
3007-3009-3011 - West (Moscone Center)
Moderators:
Alan D. Michelson, MD and J. Evan Sadler, MD, PhD
7:00 AM
PRT060318, a Novel Syk Inhibitor, Prevents Heparin-Induced Thrombocytopenia in a Transgenic Mouse ModelClinically Relevant Abstract

Michael P. Reilly, PhD1, Uma Sinha, PhD2, Pierrette Andre1*, Scott M. Taylor, MS1*, Yvonne Pak, PhD2*, Francis R. DeGuzman2*, Nisha Nanda, PhD2*, Anjali Pandey, PhD2* and Steven E. McKenzie, MD, PhD1

1Cardeza Foundation for Hematological Research, Thomas Jefferson University, Philadelphia, PA
2Portola Pharmaceuticals Inc, S San Francisco, CA

7:15 AM
Development of mPF4/Heparin Antibodies in a Murine Platelet Activation Model

Shayela Suvarna, PhD1*, Rui Qi2* and Gowthami M Arepally, MD3

1Medicine/Division of Hematology, Duke University Medical Center, Durham, NC
2Medicine, Duke University Medical Center, Durham, NC
3Medicine, Division of Hematology, Duke University Med. Ctr., Durham, NC

7:30 AM
Monocytes Are a Particularly Favorable Target for Surface Platelet Factor 4 (PF4) Antigenic Complex Formation in Heparin-Induced Thrombocytopenia: New Insights into the Thrombotic Risk in HITClinically Relevant Abstract

Lubica Rauova, MD, PhD1, Gowthami M Arepally, MD2, Douglas B. Cines, MD3 and Mortimer Poncz, MD4*

1University of Pennsylvania School of Medicine, Philadelphia, PA
2Medicine, Division of Hematology, Duke University Med. Ctr., Durham, NC
3Dept. Pathol. and Lab. Medicine, Univ. of Pennsylvania Hosp., Philadelphia, PA
4Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA

7:45 AM
Genetic Modifiers of VWF Cleavage by ADAMTS13

Wenhua Zhou, Ph.D.* and Han-Mou Tsai, M.D.

Division of Hematology, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, NY

8:00 AM
Genotyp-Phenotype Correlation in Congenital ADAMTS13 Deficient PatientsClinically Relevant Abstract

Luca Andrea Lotta1*, Isabella Garagiola1*, Andrea Cairo1*, Robert Klaassen, MD, FRCP2, Ayse Metin3*, Aytemiz Gurgey4* and Flora Peyvandi5*

1Department of Medicine and Medical Specialities, A.B.B. Hemophilia and Thrombosis Center, University of Milan, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena, Milan, Italy
2Pediatrics, Div. Hem./Onc., Children's Hosp. of Eastern Ontario, Ottawa, ON, Canada
3Pediatric Immunology Division, Children's Hospital, Ankara
4Department of Pediatrics, Section of Pediatric Hematology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
5Department of Medicine and Medical Specialties, University of Milan, IRCCS Maggiore Hospital, Milan, Milan, Italy

8:15 AM
Characterization of Five Homozygous ADAMTS13 Mutations in Hereditary Thrombotic Thrombocytopenic Purpura – Towards a Phenotype-Genotype Correlation?Clinically Relevant Abstract

Sara C Meyer1*, Shengyu Jin2*, Wenjing Cao2*, X. Long Zheng2, Bernhard Lammle1 and Johanna A Kremer Hovinga1*

1Department of Hematology and Central Hematology Laboratory, Bern University Hospital and University of Bern, Bern, Switzerland
2Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia and The University of Pennsylvania, Philadelphia, PA

8:30 AM
Critical Amino Acids in a Conserved Region of the Spacer Domain Contributing to the Binding of Anti-ADAMTS13 AntibodiesClinically Relevant Abstract

Wouter Pos, M.Sc.1*, Brenda M. Luken, Ph.D.1*, Ellen A.M. Turenhout1*, Bas de Laat, Ph.D.1*, Rob Fijnheer, Ph.D./M.D.2* and Jan Voorberg, Ph.D.1*

1Department of Plasma Proteins, Sanquin-AMC Landsteiner Laboratory, Amsterdam, Netherlands
2Department of Clinical Chemistry and Hematology, University of Utrecht, The Netherlands, Utrecht

8:45 AM
Multiple Non-Linear Epitopes of ADAMTS13 Were Targeted by Anti-ADAMTS13 IgG in Patients with Acquired Idiopathic Thrombotic Thrombocytopenic PurpuraClinically Relevant Abstract

Dezhi Shang1*, Haifeng Wu2, Spero Cataland2, Charles L. Bennett, MD, PhD, MPP3, Hau C. Kwaan4 and X. Long Zheng1

1Pathology and Laboratory Medicine, Children's Hospital of Philadelphia and University of Pennsylvania, Philadelphia, PA
2Ohio State University, Columbus, OH
3Hematology/Oncology, Northwestern University, Chicago, IL
4Northwestern University, Chicago, IL

*signifies non-member of ASH