The Hematologist

September October 2009 Vol 6 Issue 5

September-October 2009, Volume 6, Issue 5


  • A Girl Who Cries Blood – Or Does She?September 01, 2009 | George R. Buchanan, MD

    One of the most common clinical problems that hematologists deal with is diagnosis and management of patients with prolonged, excessive, or unusual bleeding. As a pediatric hematologist interested in hemorrhagic disorders for three decades, I thought until recently that I had "seen it all." But I was wrong. This past January, I was asked by the ASH Communications Department staff to consider working with the National Geographic Channel in filming a story about a 13-yearold girl in India who was said to "cry tears of blood."

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  • Activation of Prothrombin by a Potential Staphylococcus Aureus Virulence Factor: A Lesson in Classical EnzymologySeptember 01, 2009 | Pete Lollar, MD

    Most of the trypsin-like serine proteases in the coagulation and fibrinolytic mechanisms are formed by proteolytic activation from an inactive precursor, called the zymogen. During this process, a peptide at the NH 2 -terminus of the zymogen is cleaved. In a process referred to as molecular sexuality, the newly formed NH 2 -terminus of the nascent enzyme forms an internal salt bridge that results in conformational activation of the catalytic site.

  • Bad FatSeptember 01, 2009 | Nelson Chao, MD, MBA

    In our current epidemic of obesity, fat is a dreaded word. By some estimates, Americans spend $40 billion per year on weight-loss programs and diets. Now comes yet more evidence that fat is bad … for hematopoiesis.

  • Bugs and the Vasculature: 60 Trillion Endothelial Cells Can Save Your LifeSeptember 01, 2009 | Gregory M. Vercellotti, MD

    Twenty years ago Charles Janeway proposed the concepts of innate immunity that transformed immunology. 1,2 These ideas suggested that innate immune recognition of microbes depended upon receptors that detected conserved microbial products using pathogen-associated molecular patterns. This evolutionarily conserved mechanism led to the discovery in drosophila of Toll-like receptors (TLR) that could bind ligands of bacteria, viruses, and fungi that were non-self.

  • Bypass Gene Therapy for HemophiliaSeptember 01, 2009 | Robert Flaumenhaft, MD, PhD

    The formation of inhibitory antibodies to factor VIII or IX following therapy with either plasma-derived or recombinant factors remains a challenging problem in hemophilia management. Recombinant human activated factor VIIa (rhFVIIa) could potentially provide secondary prophylaxis as a bypass agent in hemophilia complicated by inhibitor formation.

  • F18-Fluorodeoxyglucose PET Scanning in Multiple MyelomaSeptember 01, 2009 | Kenneth C. Anderson, MD

    In this paper, Bartel et al., from Barlogie's group at the University of Arkansas for Medical Sciences, compared the utility of F18-fluorodeoxyglucose positron emission tomographic (FDG-PET) imaging to skeletal x-ray survey and magnetic resonance imaging in multiple myeloma (MM). In the context of their Total Therapy (TT)-3 program, FDG-PET was the leading independent factor predictive of decreased event-free and overall survival.

  • Lenalidomide and Pomalidomide Meet RhoASeptember 01, 2009 | John C. Byrd, MD

    The immune-modulating therapeutic agent lenalidomide is a broadly active therapeutic agent for a variety of hematologic malignancies including multiple myeloma, del(5q-) myelodysplastic syndrome, acute myeloid leukemia, non-Hodgkin lymphoma, Hodgkin disease, and chronic lymphocytic leukemia. This has prompted considerable interest in developing third-generation immune modulation agents, such as pomalidomide. Several of these are currently in clinical trials for multiple myeloma where promising responses have been observed.

  • Loss-of-Function TET2 Mutations: Adding Fire to Hematologic Malignancies?September 01, 2009 | Josef Prchal, MD

    Somatic mutations in JAK2 in Philadelphia-chromosome-negative (Ph-) myeloproliferative disorders (MPD), FLT 3 and other genes in acute myelocytic leukemias (AML), and numerous others reported in myelodysplastic syndromes (MDS) are clearly not sufficient to explain the full genesis of these disorders. Thus, the recent finding of a multitude of mutations of the tumor suppressive gene TET2 in numerous malignant hematologic entities has created a lot of excitement.

  • Molecular Profiling in Lymphoma Comes of AgeSeptember 01, 2009 | Steven Grant, MD

    The emergence of gene-expression analysis has added a powerful new weapon to the oncologist's armamentarium in the classification of neoplastic diseases and in the identification of prognostic indicators. The basic premise underlying this approach is that tumors that appear to be identical from a purely morphologic standpoint may in fact harbor highly disparate genetic profiles.

  • Proof of Principle for Combined Cell and Gene Therapy for Fanconi AnemiaSeptember 01, 2009 | Diane Krause, MD, PhD

    Optimal therapy for patients with genetic hematologic diseases would be to take the patient's own cells, correct the genetic abnormality, and return the cells to the patient for long-term functional engraftment. Such therapy would require a combination of cell and gene therapy. In this paper, investigators from the laboratories of Juan Carlos Izpisúa-Belmonte in Barcelona and Juan Antonio Bueren in Madrid published proof-of-principle studies showing that cells from patients with Fanconi anemia (FA) can be genetically repaired and reprogrammed into inducible pluripotent stem (iPS) cells, which can then be guided to differentiate down the hematopoietic lineage in vitro .

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Ask the Hematologist

  • Ask the Hematologist, September-October 2009September 01, 2009 | Mikkael A. Sekeres, MD, MS

    The details of this patient's history illustrate nicely how our ability to elegantly diagnose a rare bone marrow neoplasm (and exclude the diagnoses of other molecularly characterized abnormalities) exceeds the sophistication of the drugs we have available in our arsenal to treat it effectively.

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President's Column

  • ASH’s Voice in the Health Reform DebateSeptember 01, 2009 | Nancy Berliner, MD

    As this issue of The Hematologist goes to press, the U.S. Senate and House of Representatives are preparing for votes on health reform legislation. The legislation to overhaul the nation's health-care system is constantly evolving: key congressional committees are advancing proposals, but battle lines continue to sharpen between Democrats and Republicans.

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Letters to the Editor

  • NHLBI Clinical Trial for Treatment of TTPSeptember 01, 2009

    The National Heart, Lung, and Blood Institute (NHLBI) Transfusion Medicine/Hemostasis Clinical Trials Network has opened a clinical trial to study the role of rituximab in the treatment of thrombotic thrombocytopenic purpura (TTP). The Network was formed in 2002 as a consortium of 18 academic centers to provide opportunities for clinical research on uncommon hematologic disorders.

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News and Reports

  • The Ultimate Mentorship: Q&A With Dr. John Byrd, Co-Director of the 2009 Clinical Research Training InstituteSeptember 01, 2009 | John C. Byrd, MD

    The Clinical Research Training Institute prepares hematologists for careers in patient-oriented clinical research. The yearlong education and mentoring program focuses on the foundation, methodologies, and applications of clinical research. The program begins with an intensive week-long summer workshop in California, where participants work from their own proposed clinical research projects and refine and revise their plans through formal and informal interaction with faculty.

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