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Specialty: Hematology
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Total 331 results found since Jan 2013.

Clinical and laboratory characteristics of children with venous thromboembolism and protein C‐deficiency: an observational Israeli‐German cohort study
Summary Venous thromboembolism [TE] is a multifactorial disease and protein C deficiency [PCD] constitutes a major risk factor. In the present study the prevalence of PCD and the clinical presentation at TE onset, including neonatal purpura fulminans, in a cohort of children are reported. In 367 unselected children (0·1–19 years) recruited between July 1996 and December 2013, a comprehensive thrombophilia screening was performed along with recording of anamnestic data. Twenty‐five of 338 children (7·4%) had PCD. Mean age at first TE onset was 10 years (range 0·1–18). Leading thromboembolic manifestations were ne...
Source: British Journal of Haematology - July 18, 2014 Category: Hematology Authors: Verena Limperger, Ulrich C. Klostermeier, Gili Kenet, Susanne Holzhauer, Martine Alhenc Gelas, Ulrich Finckh, Ralf Junker, Christine Heller, Barbara Zieger, Karin Kurnik, Ralf Knöfler, Rolf Mesters, Susan Halimeh, Ulrike Nowak‐Göttl Tags: Research Paper Source Type: research

Pros and cons of new oral anticoagulants.
Authors: Bauer KA Abstract The availability of new oral anticoagulants (NOACs) targeting either thrombin (dabigatran etexilate) or factor Xa (rivaroxaban and apixaban) for the prevention and treatment of thrombosis has been highly anticipated. NOACs have major pharmacologic advantages over vitamin K antagonists (eg, warfarin), including rapid onset/offset of action, few drug interactions, and predictable pharmacokinetics, eliminating the requirement for regular coagulation monitoring. Regulatory agencies have approved several NOACs for specific indications based on the results of clinical trials demonstrating effic...
Source: Hematology ASH Education Program - November 11, 2014 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Transfusion strategies in hematologic and nonhematologic disease.
Authors: Carson JL, Strair R Abstract Substantial progress has been made in our understanding of the risks and benefits of RBC transfusion through the performance of large clinical trials. More than 7000 patients have been enrolled in trials randomly allocating patients to higher transfusion thresholds (∼9-10 g/dL), referred to as liberal transfusion, or lower transfusion thresholds (∼7-8 g/dL), referred to as restrictive transfusion. The results of most of the trials suggest that a restrictive transfusion strategy is safe and, in some cases, superior to a liberal transfusion strategy. However, in patients with...
Source: Hematology ASH Education Program - February 20, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Reversing targeted oral anticoagulants.
Authors: Hoffman M, Monroe DM Abstract Dabigatran, rivaroxaban, and apixaban are orally active anticoagulants that are approved in many countries. Dabigatran inhibits thrombin, whereas rivaroxaban and apixaban are factor Xa inhibitors. In clinical trials, these novel oral anticoagulants were at least as effective as warfarin for preventing stroke in patients with atrial fibrillation, but with a lower rate of serious bleeding. However, the lack of true antidotes for these agents has caused concern when patients suffer life-threatening bleeding or trauma or require emergent invasive procedures. True antidotes are und...
Source: Hematology ASH Education Program - February 20, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Smoothing the crescent curve: sickle cell disease.
Authors: Talano JA, Cairo MS Abstract Sickle cell disease (SCD) is an inherited disorder secondary to a point mutation at the sixth position of the beta chain of human hemoglobin that results in the replacement of valine for glutamic acid. This recessive genetic abnormality precipitates the polymerization of the deoxygenated form of hemoglobin S that induces a major distortion of red blood cells (sickle red blood cells), which decreases sickle red blood cell deformability, leading to chronic hemolysis and vasoocclusion. These processes can result in severe complications, including chronic pain, end organ dysfunctio...
Source: Hematology ASH Education Program - February 20, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Does hydroxyurea prevent pulmonary complications of sickle cell disease?
Authors: Buckner TW, Ataga KI Abstract A 27-year-old man with sickle cell disease (HbSS) presents to the sickle cell clinic for follow-up after a screening echocardiogram revealed an increased tricuspid regurgitant velocity of 2.7 m/s. He has a history of 2 painful crises per year and has been hospitalized 3 times over the past 10 years for management of painful crises. He had one episode of acute chest syndrome at age 15 that was treated with an RBC exchange transfusion, supplemental oxygen, and intravenous antibiotics; he did not require mechanical ventilation. He has not had additional episodes of acute chest sy...
Source: Hematology ASH Education Program - February 20, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Pharmacogenomics of antiplatelet drugs.
Authors: Sabatine MS, Mega JL Abstract Clopidogrel, a platelet P2Y12 inhibitor, is one of the most widely prescribed drugs in cardiovascular medicine because it reduces ischemic and thrombotic complications. It is a prodrug requiring biotransformation into the active metabolite by the hepatic cytochrome 450 system, especially the CYP2C19 enzyme. Candidate gene studies and genome-wide association studies have identified loss-of-function CYP2C19 variants to be associated with a diminished pharmacologic response. Specifically, compared with noncarriers, carriers of at least one copy of a loss-of-function CYP2C19 allel...
Source: Hematology ASH Education Program - February 20, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Monitoring target-specific oral anticoagulants.
Authors: Konkle BA Abstract Target-specific oral anticoagulants are approved for use for the prevention of stroke in atrial fibrillation and for the prevention and treatment of venous thrombosis without the need for laboratory monitoring. However, there are clinical settings in which laboratory measurement of anticoagulant effect is needed. These may include patients with life-threatening bleeding or those requiring emergency surgery, in the setting of renal or hepatic failure, or patients with thrombosis on therapy. This chapter reviews the use of laboratory testing to assess the anticoagulant effect of these drug...
Source: Hematology ASH Education Program - February 20, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Future prospects for contact factors as therapeutic targets.
Authors: Gailani D Abstract Anticoagulants currently used in clinical practice to treat or prevent thromboembolic disease are effective, but place patients at increased risk for serious bleeding because they interfere with plasma enzymes (thrombin and factor Xa) that are essential for hemostasis. In the past 10 years, work with genetically altered mice and studies in baboons and rabbits have demonstrated that the plasma contact proteases factor XI, factor XII, and prekallikrein contribute to the formation of occlusive thrombi despite having limited roles in hemostasis. In the case of factor XI, epidemiologic data f...
Source: Hematology ASH Education Program - February 20, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Implementation of non-vitamin K antagonist oral anticoagulants in daily practice: the need for comprehensive education for professionals and patients
Non-vitamin K antagonist oral anticoagulants (NOACs) are increasingly used for the prevention and treatment of venous thromboembolism and for stroke prevention in patients with atrial fibrillation. NOACs do not require routine coagulation monitoring, creating a challenge to established systems for patient follow-up based on regular blood tests. Healthcare professionals (HCPs) are required to cope with a mixture of patients receiving either a vitamin K antagonist or a NOAC for the same indications, and both professionals and patients require education about the newer drugs. A European working group convened to consider the ...
Source: Thrombosis Journal - May 26, 2015 Category: Hematology Authors: Hein HeidbuchelDana BertiManuel CamposLien DestegheAna FreixoAntónio NunesVanessa RoldánVincenzo ToschiRiitta Lassila Source Type: research

Mechanical circulatory support: balancing bleeding and clotting in high-risk patients.
Authors: Baumann Kreuziger L, Massicotte MP Abstract Mechanical circulatory support (MCS) provides a bridge to heart transplant in children and adults with life-threatening heart failure and sustains patients ineligible for transplant. Extracorporeal membrane oxygenation (ECMO) provides temporary support for patients in cardiac or pulmonary failure through external gas exchange and continuous flow of blood. Because the median time to heart transplant exceeds event-free time on ECMO, pulsatile left ventricular assist devices (LVADs) are used to support infants and children. Continuous flow LVADs are preferred in ado...
Source: Hematology ASH Education Program - December 10, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Sickle cell disease: when and how to transfuse.
Authors: Howard J Abstract Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome,...
Source: Hematology ASH Education Program - December 4, 2016 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Real-life experience with the specific reversal agent idarucizumab for the management of emergency situations in dabigatran-treated patients: a series of 11 cases
We report 11 real-life clinical cases in which idarucizumab was used after multidisciplinary consultation in a variety of emergency situations including severe postoperative bleeding, emergency high-bleeding-risk surgery (hip/spine surgery and neurosurgery), invasive diagnostic testing (lumbar puncture), intracranial bleeding (pre-pontine subarachnoid hemorrhage and lobar intracerebral hemorrhage) and thrombolysis with recombinant tissue plasminogen activator for acute ischemic stroke. This case series illustrates the role of idarucizumab in improving patient safety in rare emergency situations requiring rapid reversal of ...
Source: Journal of Thrombosis and Thrombolysis - February 15, 2017 Category: Hematology Source Type: research

Patients' experiences of atrial fibrillation and non-vitamin K antagonist oral anticoagulants (NOACs), and their educational needs: A qualitative study
This study explores patients' experiences of dabigatran and their recommendations for development of educational materials.
Source: Thrombosis Research - March 6, 2017 Category: Hematology Authors: Danielle E Clarkesmith, Gregory Y H Lip, Deirdre A Lane Tags: Full Length Article Source Type: research

Comprehensive neuropsychological evaluation of children and adolescents with sickle cell anemia: a hospital-based sample
Conclusion: As sickle cell anemia is considered a progressive cerebral vasculopathy, it is a potential risk factor for neurocognitive and psychosocial development. Therefore, periodic neuropsychological and behavioral evaluations of children and adolescents with sickle cell anemia may represent a useful measure to reduce long-term biopsychosocial repercussions.
Source: Revista Brasileira de Hematologia e Hemoterapia - April 3, 2017 Category: Hematology Source Type: research