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508.P1.39 Bone Marrow Failure Poster I

Saturday, December 6, 2008: 5:30 PM-7:30 PM
Hall A (Moscone Center)
Critical Role of the Th1 Transcription Factor T-Bet in An Animal Model of Immune-Mediated Bone Marrow Failure

Yong Tang1*, Jichun Chen, PhD2 and Neal Young3

1Hematology, National Heart, Lung, and Blood Institute, Bethesda, MD
2National Institutes of Health, National Heart, Lung, and Blood Inst., Bethesda, MD
3Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, MD

Circulating Cytokine Profiles of Patients with Acquired Aplastic Anemia and Myelodysplastic SyndromeClinically Relevant Abstract

Xingmin Feng, PhD1, Phillip Scheinberg, MD2, Colin O. Wu, PhD3*, Leigh Samsel4*, Olga Nunez1*, Courtney Prince5*, Barbara Weinstein1*, Rebecca D Ganetzky, B.A.6*, J. Philip McCoy, PhD4, Jaroslaw P. Maciejewski, MD, PhD5 and Neal S. Young, MD1

1Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD
2National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD
3Office of Biostatistics Research, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD
4Flow Cytometry Core, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD
5Experimental Hematology and Hematopoiesis Section, Taussig Cancer Center, Cleveland Clinic, Cleveland, OH
6Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH

Fas- and Fas Ligand Rather Than Perforin-Mediated Apoptosis Has a Dominant Role in Target Cell Death in Murine Models of Immune-Mediated Bone Marrow Failure

Jichun Chen, Ph.D., Stephanie O. Omokaro, M.D.*, Annahita K. Sarcon, M.S.* and Neal Young, M.D.

Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, MD

A Potential Novel Therapeutic Approach for Fanconi Anemia

W. Clark Lambert, MD, PhD* and Monique M Brown, PhD*

Pathology and Laboratory Medicine, UMDNJ-New Jersey Medical School, Newark, NJ

The Fanconi Anemia Protein, FANCG, Binds Directly to the SH3 Domain of aII Spectrin and This Interaction May Play An Important Role in the Stability of aII Spectrin and Its Function in Repair of DNA Interstrand Cross-Links

Joel A Lefferts, Ph.D.*, Chuan Wang, Ph.D.*, Melissa Baralt, M.A.* and Muriel W Lambert, PhD

Pathology and Laboratory Medicine, UMDNJ - New Jersey Medical School, Newark, NJ

Subcutaneous Alemtuzumab Is a Safe and Effective Treatment for Global or Single-Lineage Immune-Mediated Marrow Failures: a Survey from the EBMT-WPSAAClinically Relevant Abstract

Antonio M. Risitano, MD, PhD1, Elisa Seneca1*, Ludovica Marando1*, Bianca Serio1*, Carmine Selleri1*, Giulia Scalia1*, Luigi Del Vecchio, MD2*, Annapaola Iori, M.D.3*, Alexander Kulagin4*, Sebastien Maury5*, Joerg Halter6*, Vikas Gupta, MD, MRCP, MRCPath7, Andrea Bacigalupo, MD8, Gerard Socié9*, Andre Tichelli10*, Judith Marsh11, Hubert Schrezenmeier12*, Jakob R. Passweg, MD, MS13 and Bruno Rotoli1*

1Hematology, Federico II, Naples, Italy
2Hematology Unit - CEINGE and Dip. di Biochimica e Biotecnologie Mediche, Università di Napoli Federico II, Italy, 80131, CEINGE, Naples, Italy
3Hematology La Sapienza
4Hematology, Institute of Clinical Immunology SB RAMS, Novosibirsk, Russia
5Hematology, Hôpital Henri Mondor
6Hematology, University Hospital of Basel, Basel, Switzerland
7Princess Margaret Hospital, Toronto, ON, Canada
8Hem-Onc, Ospedale S. Martino, Genova, Italy
9Hematology / Transplantation, Hopital Saint-Louis, Paris, France
10Hematology, University Hospital of Basel, Switzerland
11King's College Hospital, London, United Kingdom
12Medical School, Ulm, Germany
13Hopitaux Universitaires de Geneve, Geneve, Switzerland

The Role of Stress Activated Protein Kinases in Promoting Fanconi Anemia Hematopoietic Stem and Progenitor Cell Dysfunction

M. Reza Saadatzadeh, PhD1*, Khadijeh Bijangi-Vishehsaraei, PhD2* and Laura S. Haneline, MD3

1Vascular Surgery, Indiana University School of Medicine, Indianapolis, IN
2Department of Pharmacology, Indiana University School of Medicine, Indianapolis, IN
3Pediatrics and Microbiology and Immunology, Indiana University School of Medicine, Indianapolis, IN

Very Short Telomeres Are Characteristic of Dyskeratosis Congenita and Not Other Inherited Bone Marrow Failure SyndromesClinically Relevant Abstract

Blanche P. Alter, MD, MPH1, Gabriela Baerlocher, MD2, Neelam Giri, MD3, Peter M. Lansdorp, MD, PhD4 and Sharon A Savage, MD, FAAP5

1Clinical Genetics Branch, National Cancer Institute, Rockville, MD
2Dept. of Hematology, University Hospital Berne, Berne, Switzerland
3National Cancer Institute, Rockville, MD
4BC Cancer Research Centre, Terry Fox Laboratory, Vancouver, BC, Canada
5Division of Cancer Epidemiology and Gene, National Cancer Institute, Bethesda, MD

Frequency and Natural History of Inherited Bone Marrow Failure Syndromes: The Israeli Inherited Bone Marrow Failure Registry

Hannah Tamary, MD1, Daniella Nishri, MD1*, Joanne Yacobovich, MD1*, Rama Zilber, RN1*, Shraga Aviner, MD, PhD2, Polina Stepensky, MD3*, Shoshana S Vilk-Ravel3*, Menachem Bitan3*, Chaim Kaplinsky, MD4*, Ayelet Ben Barak, MD5*, Joseph Kapelusnik, MD6*, Ariel Koren, MD7*, Carina Levin, MD1*, Isaac Yaniv, MD1*, Philip S Rosenberg, PhD8* and Blanche P. Alter, MD9*

1Schneider Children's Med. Ctr., Petah Tikva, Israel
2Pediatrics, The Barzilai Medical Center, Ashkelon, Israel
3Hadassah Medical Center, Israel
4Pediatric Hem/Onc, Sheba Medical Center, Israel
5Pediatric Hem/Onc, Rambam Medical Center, Israel
6Pediatric Hem/Onc, Soroka Medical Center, Israel
7Pediatric Hematology, Haemk Medical Center, Israel
8Biostatistics Branch, National Cancer Institute, Rockville, MD
9Clinical Genetics, National Cancer Institute, Rockville, MD

Androgen Treatment for Acquired Aplastic Anemia in Mexican Adults

Victoria E. Ferrer Argote, MD, Hematologist1, Christian O. Ramos Peñafiel, MD, Hematology, Resident2*, Merit Hernández Sánchez, MD, Hematology, Resident3*, Juan José García García, MD, M.S.4*, Humberto Castellanos Sinco, Hematology, Resident2*, Guadalupe León González, MD, Hematologist2* and EfréN. Montaño Figueroa, MD, Hematologist2*

1Hematology, Hospital General de México, O.D., Mexico City, Mexico
2Hematology, Hospital General de Mexico, O.D., Mexico City, Mexico
3Hematology, Hospital General de Mexico, O.D., Mexico City
4Public Health, Universidad Nacional Autónoma de México, Mexico City, Mexico

Telomerase Gene Mutation Screening and Telomere Length Detection in Chinese Patients with Bone Marrow Failure Syndrome

Bing Han, MD, PhD*, Bo Liu, MD, PhD* and Yongqiang Zhao, MD, PhD*

Dept. of Hematology, Peking Union Med. College Hosp., Beijing, China

Efficacy and Safety of 1 Year’s Treatment with Deferasirox (Exjade®): Assessment of Regularly Transfused Patients with Diamond-Blackfan Anemia Enrolled in the EPIC StudyClinically Relevant Abstract

John B Porter1*, Gian Luca Forni2*, Photis Beris3, Ali Taher4, Dany Habr5*, Gabor Domokos6*, Abdel Hmissi6* and Maria Domenica Cappellini7

1University College London, London, United Kingdom
2Ospedale Galliera, Genova, Italy
3Geneva University Hospital, Geneva, Switzerland
4American University Beirut, Beirut, Lebanon
5Novartis, East Hanover, NJ
6Novartis, Basel, Switzerland
7Universitá di Milano, Policlinico Foundation IRCCS, Milan, Italy

Predicting Response to Immunosuppressive Therapy in Childhood Aplastic Anemia

Nao Yoshida, MD1*, Hiroshi Yagasaki, MD.PhD2*, Yoshiyuki Kosaka, MD.PhD3*, Ryoji Kobayashi, MD.PhD4*, Hiromasa Yabe, MD.PhD5*, Takashi Kaneko, MD.PhD6*, Masahiro Tsuchida, MD.PhD7, Akira Ohara, MD.PhD8, Tatsutoshi Nakahata, MD.PhD9 and Seiji Kojima, MD.PhD2*

1Division of Hematology and Oncology, Children's Medical Center, Japanese Red Cross Nagoya First Hospital, Nagoya, Japan
2Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
3Department of Hematology and Oncology, Kobe Children's Hospital, Kobe, Japan
4Department of Pediatrics, Sapporo Hokuyu Hospital, Sapporo, Japan
5Department of Cell Transplantation, Tokai University School of Medicine, Isehara, Japan
6Department of Pediatrics, Kiyose Children's Hospital, Japan
7Department of Pediatrics, Ibaraki Children's Hospital, Japan
8Department of Pediatrics, Toho University School of Medicine, Tokyo, Japan
9Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan

*signifies non-member of ASH