Seminars in hematology
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Atrial fibrillation (AF), the most frequent sustained arrhythmia, is associated with an increased risk of thromboembolic events. The risk of stroke depends on risk factors such as age, hypertension, heart failure, and vascular disease. Thus, antithrombotic therapy is a cornerstone in the management of AF. ⋯ Severe bleeding, such as fatal and intracranial bleeding, was less frequent with direct oral anticoagulants. Results of the studies and subgroup analyses are discussed. Further trials using direct oral anticoagulants in special populations such as very old and patients with kidney disease are needed.
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Seminars in hematology · Oct 2013
ReviewGenomic characterization of the inherited bone marrow failure syndromes.
The inherited bone marrow failure syndromes (IBMFS) are a set of clinically related yet heterogeneous disorders in which at least one hematopoietic cell lineage is significantly reduced. Many of the IBMFS have notably increased cancer risks, as well as other physical findings. Highly penetrant germline mutations in key pathways, such as DNA repair, telomere biology, or ribosomal biogenesis, are causative of Fanconi anemia (FA), dyskeratosis congenita (DC), and Diamond-Blackfan anemia (DBA), respectively. ⋯ Panels of large numbers of genes are being used to molecularly characterize patients with IBMFS, such as FA and DBA. NGS is also accelerating the discovery of the genetic etiology of previously unclassified IBMFS. In this review, we will highlight recent studies that have employed NGS to ascertain the genetic etiology of IBMFS, namely, FA, DC, DBA, and TAR, and discuss the translational utility of these findings.
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Seminars in hematology · Jul 2013
ReviewThrombocytopenia in the intensive care unit-diagnostic approach and management.
Thrombocytopenia often complicates critical illness and is associated with increased morbidity and mortality. Approaching thrombocytopenia is challenging in the intensive care unit (ICU) because of the multifactorial pathogenesis of this disorder. Interpretation of the platelet count course after ICU admission is helpful to narrow down the cause of thrombocytopenia. ⋯ A rapid decrease in platelet counts of more than 50% within 1-2 days, especially if occurring after an intermittent rise, requires immediate attention as it may be a symptom of immune-mediated mechanisms, eg, heparin-induced thrombocytopenia. Treatment should target the underlying disease. Platelet transfusions are indicated in bleeding patients, while there is no strong evidence supporting the usefulness of prophylactic transfusions in ICU patients.
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Trauma is a leading cause of death and disability. Hemorrhage is the major mechanism responsible for death during the first 24 hours following trauma. One quarter of severely injured patients present in the emergency room with acute coagulopathy of trauma and shock (ACOT). ⋯ The resuscitation-associated coagulopathy (RAC) is secondary to a combination of acidosis, hypothermia and dilution from intravenous blood and fluid therapy. RAC may further aggravate acidosis and hypoxia resulting in a vicious cycle. This review focuses on the biology of the trauma-associated coagulopathy, and reviews current therapeutic strategies.
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Seminars in hematology · Jul 2013
ReviewReview of the American College of Chest Physicians 2012 Guidelines for Anticoagulation Therapy and Prevention of Thrombosis.
The eighth edition (AT8) of the American College of Chest Physicians (ACCP) Antithrombotic Therapy and Prevention of Thrombosis Guideline, published in June 2008, was a comprehensive presentation of primary studies and detailed discussions of rationale for recommendations. This resulted in an approximately 900-page Chest Supplement publication. Updating the guidelines in a succinct fashion posed a formidable challenge for the ninth edition (AT9), published in February 2012. ⋯ Major innovative changes include a recognition of the value of estimating the risk reductions in symptomatic, as opposed to asymptomatic (venographically), detected venous thrombosis, using nonconflicted methodologists as topic editors, new insights into evidence, and increasing emphasis on what is known about patients' values and preferences that have served to improve this edition of the guidelines. This review provides a summary of the updates of the guidelines for anticoagulation therapy and prevention of thrombosis. The AT9 recommendations are presented with, if included, the AT8 recommendations in parenthesis for comparison purposes.