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Hematology—the Education Program of the American Society of Hematology

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https://read.qxmd.com/read/30570487/taco-and-trali-biology-risk-factors-and-prevention-strategies
#1
REVIEW
Nareg Roubinian
Transfusion-related acute lung injury (TRALI) and transfusion-associated circulatory overload (TACO) are the leading causes of transfusion-related morbidity and mortality. These adverse events are characterized by acute pulmonary edema within 6 hours of a blood transfusion and have historically been difficult to study due to underrecognition and nonspecific diagnostic criteria. However, in the past decade, in vivo models and clinical studies utilizing active surveillance have advanced our understanding of their epidemiology and pathogenesis...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504361/neunert-ce-management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes-hematology-am-soc-hematol-educ-program-2017-2017-400-405
#2
(no author information available yet)
No abstract text is available yet for this article.
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504360/drug-associated-thrombocytopenia
#3
REVIEW
Tamam Bakchoul, Irene Marini
Many drugs have been implicated in drug-induced immune thrombocytopenia (DITP). Patients with DITP develop a drop in platelet count 5 to 10 days after drug administration with an increased risk of hemorrhage. The diagnosis of DITP is often challenging, because most hospitalized patients are taking multiple medications and have comorbidities that can also cause thrombocytopenia. Specialized laboratory diagnostic tests have been developed and are helpful to confirm the diagnosis. Treatment of DITP involves discontinuation of the offending drug...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504359/evidence-based-management-of-immune-thrombocytopenia-ash-guideline-update
#4
REVIEW
Cindy E Neunert, Nichola Cooper
In 1996 and 2011, the American Society of Hematology (ASH) supported efforts to create guidelines for the diagnosis and management of patients with immune thrombocytopenia (ITP). These guidelines used different approaches to arrive at recommendations for testing and treatment. Despite differences in methodology, in both cases there was a paucity of randomized trials to inform recommendations. As data on the diagnosis and management of ITP expands, the ASH Committee on Quality is dedicated to maintaining updated guidelines representing recent evidence and guideline methodology...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504358/how-do-we-diagnose-immune-thrombocytopenia-in-2018
#5
REVIEW
John G Kelton, John R Vrbensky, Donald M Arnold
In this report, we will review the various clinical and laboratory approaches to diagnosing immune thrombocytopenia (ITP), with a focus on its laboratory diagnosis. We will also summarize the results from a number of laboratories that have applied techniques to detect anti-platelet autoantibodies as diagnostic tests for ITP. Although there is considerable variability in methods among laboratories, there is general agreement that platelet autoantibody testing has a high specificity but low sensitivity. This suggests several possibilities: (1) the ideal test for ITP has yet to be developed, (2) current test methods need to be improved, or (3) ITP is the clinical expression of a variety of thrombocytopenic disorders with different underlying mechanisms...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504357/shifting-ground-and-gaps-in-transfusion-support-of-patients-with-hematological-malignancies
#6
REVIEW
Christine Cserti-Gazdewich
The transfusion support of hematological malignancies considers 2 dimensions: the quantity of what we order (in terms of triggers, doses, targets, and intervals), and the special qualities thereof (with respect to depths of matching and appropriate product modifications). Meanwhile, transfusion-related enhancements in the quantity and quality of life may not be dose dependent but rather tempered by unintended patient harms and system strains from overexposure. Evidence and guidelines concur in endorsing clinically noninferior conservative red blood cell (RBC) transfusion care strategies (eg, triggering at hemoglobin <7-8 g/dL and in single-unit doses for stable, nonbleeding inpatients)...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504356/ttp-long-term-outcomes-following-recovery
#7
REVIEW
James N George
Although risk for relapse may be the greatest concern following recovery from acquired, autoimmune thrombotic thrombocytopenic purpura (TTP), there are multiple other major health issues that must be recognized and appropriately addressed. Depression may be the most common disorder following recovery from TTP and may be the most important issue for the patient's quality of life. Severe or moderate depression has occurred in 44% of Oklahoma Registry patients. Recognition of depression by routine screening evaluations is essential; treatment of depression is effective...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504355/beyond-plasma-exchange-novel-therapies-for-thrombotic-thrombocytopenic-purpura
#8
REVIEW
Kathryn Dane, Shruti Chaturvedi
The advent of plasma exchange has dramatically changed the prognosis of acute thrombotic thrombocytopenic purpura (TTP). Recent insights into TTP pathogenesis have led to the development of novel therapies targeting pathogenic anti-ADAMTS13 antibody production, von Willebrand factor (VWF)-platelet interactions, and ADAMTS13 replacement. Retrospective and prospective studies have established the efficacy of rituximab as an adjunct to plasma exchange for patients with acute TTP, either upfront or for refractory disease...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504354/clinical-and-laboratory-diagnosis-of-ttp-an-integrated-approach
#9
REVIEW
Thita Chiasakul, Adam Cuker
Thrombotic thrombocytopenia purpura (TTP) is a rare, life-threatening disease with an incidence of approximately 2 persons per million per year. It is characterized by severe deficiency of the von Willebrand cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), leading to formation of platelet-rich thrombi in the microvasculature. Prompt initiation of appropriate therapy, particularly plasma exchange, may be life-saving. Diagnosis of TTP is challenging because of its diverse clinical manifestations, overlap in clinical presentation with other thrombotic microangiopathies, and limited availability of ADAMTS13 testing...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504353/preventing-and-managing-catastrophic-bleeding-during-extracorporeal-circulation
#10
REVIEW
Keyvan Karkouti, Loretta T S Ho
The use of extracorporeal circulation for cardiac surgery and extracorporeal life support poses tremendous challenges to the hemostatic equilibrium given its diametric tendency to trigger hyper- and hypocoagulopathy. The necessity of anticoagulant therapy to counteract the hemostatic activation by the extracorporeal circuitry compounded by unfavorable patient and surgical factors significantly increase the risk of catastrophic bleeding in patients who require extracorporeal circulation. Preoperative measures, such as stratification of high-risk bleeding patients, and optimization of the modifiable variables, including anemia and thrombocytopenia, provide a crude estimation of the likelihood and may modify the risk of catastrophic bleeding...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504352/an-introduction-to-point-of-care-testing-in-extracorporeal-circulation-and-lvads
#11
REVIEW
Rachel Sara Bercovitz
There is a delicate balance between bleeding and clotting in patients on circuits such as ventricular assist devices or extracorporeal membrane oxygenation. Traditional coagulation tests, prothrombin time, activated partial thromboplastin time, and anti-factor Xa levels, are used to monitor patients on these devices. However, turnaround times and inability to assess global hemostasis, including platelets and fibrinogen have contributed to a recognition that faster, accurate, and more informative coagulation tests are needed...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504351/adult-and-pediatric-mechanical-circulation-a-guide-for-the-hematologist
#12
REVIEW
Lisa Baumann Kreuziger, M Patricia Massicotte
Mechanical circulatory support (MCS) is the overarching term that encompasses the temporary and durable devices used in patients with severe heart failure. MCS disturbs the hematologic and coagulation system, leading to platelet activation, activation of the contact pathway of coagulation, and acquired von Willebrand syndrome. Ischemic stroke and major hemorrhage occur in up to 30% of patients. Hematologists are an essential part of the MCS team because they understand the delicate balance between bleeding and clotting and alteration of hemostasis with antithrombotic therapy...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504350/new-insights-into-the-pathophysiology-and-development-of-novel-therapies-for-sickle-cell-disease
#13
REVIEW
Scott Moerdler, Deepa Manwani
Although the seminal event in sickle cell disease is the polymerization of abnormal hemoglobin, the downstream pathophysiology of vasoocclusion results from heterotypic interactions between the altered, adhesive sickle cell red blood cells, neutrophils, endothelium, and platelets. Ischemia reperfusion injury, hemolysis, and oxidant damage all contribute to heightened inflammation and activation of the hemostatic system. These various pathways are the focus of emerging treatments with potential to ameliorate disease manifestations...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504349/measuring-success-utility-of-biomarkers-in-sickle-cell-disease-clinical-trials-and-care
#14
REVIEW
Ram Kalpatthi, Enrico M Novelli
Progress in the care of sickle cell disease (SCD) has been hampered by the extreme complexity of the SCD phenotype despite its monogenic inheritance. While epidemiological studies have identified clinical biomarkers of disease severity, with a few exceptions, these have not been routinely incorporated in clinical care algorithms. Furthermore, existing biomarkers have been poorly apt at providing objective parameters to diagnose sickle cell crisis, the hallmark, acute complication of SCD. The repercussions of these diagnostic limitations are reflected in suboptimal care and scarcity of adequate outcome measures for clinical research...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504348/the-current-state-of-sickle-cell-trait-implications-for-reproductive-and-genetic-counseling
#15
REVIEW
Lydia H Pecker, Rakhi P Naik
Sickle cell trait (SCT) is unique among the carrier states that are identified during newborn screening. Unlike other heterozygous states for rare recessive diseases, SCT is exceedingly prevalent throughout regions of the world, making sickle cell disease one of the most common monogenetic diseases worldwide. Because of this high frequency, reproductive counseling is of paramount importance. In addition, unlike other carrier states, SCT seems to be a risk factor for several clinical complications, such as extreme exertional injury, chronic kidney disease, and venous thromboembolism...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504347/alternative-donor-transplants-for-severe-aplastic-anemia
#16
REVIEW
Andrea Bacigalupo
Allogeneic hematopoietic stem-cell transplantation remains the only curative treatment for patients with acquired severe aplastic anemia (SAA). When a matched sibling is not available, one can search for a matched unrelated donor or a cord blood unit (CB) in the international registries or, more recently, for an HLA haploidentical (HAPLO) family member. International guidelines call for a course of antithymocyte globulin (ATG) and cyclosporine before a patient with SAA receives a transplant from a donor other than an HLA identical sibling, but whether this is necessary for patients age <20 years is less clear...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504346/a-brief-but-comprehensive-guide-to-clonal-evolution-in-aplastic-anemia
#17
REVIEW
Daria V Babushok
Acquired aplastic anemia (AA) is an immune-mediated bone marrow aplasia that is strongly associated with clonal hematopoiesis upon marrow recovery. More than 70% of AA patients develop somatic mutations in their hematopoietic cells. In contrast to other conditions linked to clonal hematopoiesis, such as myelodysplastic syndrome (MDS) or clonal hematopoiesis of indeterminate potential in the elderly, the top alterations in AA are closely related to its immune pathogenesis. Nearly 40% of AA patients carry somatic mutations in the PIGA gene manifested as clonal populations of cells with the paroxysmal nocturnal hemoglobinuria phenotype, and 17% of AA patients have loss of HLA class I alleles...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504345/activity-of-eltrombopag-in-severe-aplastic-anemia
#18
REVIEW
Phillip Scheinberg
Since the approval of horse antithymocyte globulin (ATG) decades ago, there was a long hiatus in therapies with activity in severe aplastic anemia (SAA). This scenario changed in 2014 when eltrombopag, a thrombopoietin receptor agonist, was approved for SAA after an insufficient response to initial immunosuppressive therapy (IST). The basis for this approval was the observation of single-agent activity of eltrombopag in this patient population, where 40% to 50% recovered blood counts at times involving >1 lineage...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504344/anticoagulating-patients-with-high-risk-acquired-thrombophilias
#19
REVIEW
Leslie Skeith
Antiphospholipid syndrome (APS), heparin-induced thrombocytopenia, and paroxysmal nocturnal hemoglobinuria are 3 acquired thrombophilias that carry a high risk of venous and arterial thromboembolism. Management of these conditions has largely included anticoagulation with a vitamin K antagonist after an initial period of a parenteral anticoagulant, for as long as the thrombotic risk is still present. The available evidence for the use of direct oral anticoagulants (DOACs) is limited and primarily consists of case series and cohort studies, which are summarized in this chapter...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/30504343/breadth-of-complications-of-long-term-oral-anticoagulant-care
#20
REVIEW
Walter Ageno, Marco Donadini
The majority of patients with venous thromboembolism (VTE) have a considerable long-term risk of recurrence and may require extended duration of anticoagulant treatment after the initial 3 to 6 months. The decision to extend treatment is based not only on the individual risk of recurrence, but should also consider the potential complications associated with anticoagulation, taking into account that anticoagulant drugs are among the drugs most frequently associated with hospital admission due to adverse drug reactions...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
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