Hematology
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Erythrocytosis results when there is an increased red cell mass and thus an increased hemoglobin. The causes can be divided into primary intrinsic defects of the erythroid progenitor cell and secondary defects, where factors external to the erythroid compartment are responsible. Both can then be further divided into congenital and acquired categories. ⋯ Having eliminated the common entity polycythemia vera, further direction for investigation is guided by the erythropoietin level. Clinical consequences of the various erythrocytoses are not clear, but in some groups thromboembolic events have been described in young patients. Evidence is lacking to define best management, but aspirin and venesection to a target hematocrit should be considered.
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Hematopoietic stem cell (HSC)-targeted gene transfer is an attractive approach for the treatment of a number of hematopoietic disorders caused by single gene defects. Indeed, in a series of gene transfer trials for two different primary immunodeficiencies beginning early in this decade, outstanding success has been achieved. Despite generally low levels of engrafted, genetically modified HSCs, these trials were successful because of the marked selective advantage of gene-corrected lymphoid precursors that allowed reconstitution of the immune system. ⋯ Encouragingly, gene transfer levels in this range have recently been reported in a lentiviral vector gene transfer clinical trial for children with adrenoleukodystrophy. A clinical gene transfer trial for beta-thalassemia has begun in France, and one patient with transfusion-dependent HbE/beta-thalassemia has demonstrated a therapeutic effect after transplantation with autologous CD34(+) cells genetically modified with a beta-globin lentiviral vector. Here, the development and recent progress of gene therapy for the hemoglobin disorders is reviewed.
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Securing a junior faculty position is an important early step in an academic career in hematology. Shortly thereafter one should begin to plan for eventual promotion and possible tenure. ⋯ This understanding includes the definition of and criteria for achieving tenure, the academic tracks and the policies for advancement on each track, and the process by which the institutional committee responsible for promotion and tenure conducts its activities. Learning the rules and successfully navigating the academic pathway will help ensure success by achieving the desired promotion and the self-satisfaction, prestige, and financial awards that may accompany it.
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Recommendations in the form of clinical practice guidelines are increasingly common. Clinical guidelines are systematically developed statements designed to help administrators, practitioners and patients make decisions about appropriate health care for specific circumstances. In North America, guidelines developed by professional societies, government panels and cooperative groups are frequently used to measure quality, to allocate resources and to determine how health care dollars are spent. For clinicians, guidelines provide a summary of the relevant medical literature and offer assistance in deciding which diagnostic tests to order, which treatments to use for specific conditions, when to discharge patients from the hospital, and many other aspects of clinical practice.