Seminars in Thrombosis and Hemostasis

Publisher: Thieme Publishing

Description

  • Impact factor
    4.22
  • 5-year impact
    3.84
  • Cited half-life
    5.20
  • Immediacy index
    2.00
  • Eigenfactor
    0.01
  • Article influence
    1.06
  • ISSN
    1098-9064
  • OCLC
    163849709
  • Material type
    Internet resource
  • Document type
    Internet Resource, Computer File, Journal / Magazine / Newspaper

Publisher details

Thieme Publishing

  • Pre-print
    • Author cannot archive a pre-print version
  • Post-print
    • Author can archive a post-print version
  • Conditions
    • Authors and Publishers version on author's personal web site
    • Institutional Repository (including PubMed Central) after 12 months
    • Publisher's version/PDF cannot be used
    • Publisher copyright and source must be acknowledged
    • Link to Publisher version (www.thieme-connect.com) must be included if article has been published online
  • Classification
    ​ blue

Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: It is now well established that the treatment of choice for children with severe hemophilia is prophylaxis started early in life. Although, there is no consensus among the hemophilia management community to either stop or maintain prophylactic treatment in adulthood, experts, and centers advise individualized prophylaxis according to clinical bleeding pattern, condition of joints, pharmacokinetic profile, physical activity, type of employment, and patients' personal preferences. The aim of this article is to describe the impact of an individualized prophylaxis approach on young adults with severe hemophilia, in the setting of a Portuguese Haemophilia Comprehensive Care Centre. We proposed a tailored prophylaxis approach on a young adult cohort with 10 patients with severe hemophilia (7× type A and 3× type B) on standard prophylactic regimens in childhood, based on clinical outcome. Patients were evaluated and prophylaxis was adjusted (dose and/or frequency) to daily life activity and bleeding pattern. After 12 months of follow-up, one patient returned to the previous regimen due to breakthrough bleeds and the remaining nine patients maintained their new prophylaxis approach, without increasing bleeding episodes. With an individualized approach, in this cohort of nine patients, we observed no negative impact on clinical outcome, with a proposed improvement in quality of life and a reduction of costs.
    Seminars in Thrombosis and Hemostasis 10/2014;
  • Seminars in Thrombosis and Hemostasis 10/2014;
  • [Show abstract] [Hide abstract]
    ABSTRACT: The antiphospholipid syndrome (APS) is characterized by venous or arterial thrombosis and/or pregnancy morbidity in patients with persistent presence of antiphospholipid antibodies (aPL). Patients who are diagnosed with APS are identified to have a high risk of recurrent thrombosis, which can occur despite anticoagulant therapy. The optimal type, intensity, and duration of anticoagulant therapy for the treatment of APS remain controversial issues, particularly for arterial thrombosis and recurrent thrombosis. Patients with persistently positive testing for lupus anticoagulant and elevated levels of anticardiolipin antibodies and anti-β2 glycoprotein I antibodies-known as triple positivity-appear to be at increased risk for thrombosis compared with patients who test positive for a single aPL. Recognizing that patients with APS may potentially have different thrombotic risk profiles may assist clinicians in assessing the risks, benefits, and optimal duration of anticoagulation. Future studies that delineate thrombotic risk in APS and evaluate current and novel anticoagulants as well as nonanticoagulant therapies are required.
    Seminars in Thrombosis and Hemostasis 10/2014;
  • [Show abstract] [Hide abstract]
    ABSTRACT: Acquired hemophilia A (AHA) should be suspected in patients with a new onset of bleeding and an isolated prolongation of activated partial thromboplastin time. About 10% of patients do not bleed at the time of diagnosis, but are at risk of future bleeding, particularly during interventions or surgery. Diagnosis of AHA is confirmed by demonstrating markedly reduced factor VIII activity (FVIII:C) and neutralizing anti-FVIII antibodies, so-called inhibitors. Several limitations and pitfalls exist with the assays used to diagnose AHA. Interference can result from anticoagulants or lupus anticoagulant. The Bethesda assay used to measure inhibitor potency assumes a log-linear relationship between inhibitor concentration and effect on residual FVIII:C activity to allow exact quantification. However, this relationship is not present for the type 2 inhibitors typically seen in AHA. Therefore, this assay only provides a rough estimate of inhibitor potency. These limitations can explain, in part, why laboratory data, such as inhibitor potency, failed to predict bleeding or response to treatment in AHA. This article reviews the diagnostic approach to AHA, discusses assay-specific limitations and addresses some of the challenges for future research.
    Seminars in Thrombosis and Hemostasis 10/2014;
  • [Show abstract] [Hide abstract]
    ABSTRACT: There is a paucity of data on the incidence of venous thromboembolism (VTE) in Australia, particularly amongst Indigenous Australians. We have therefore conducted a retrospective review of all cases of deep vein thrombosis and pulmonary embolism over a 24 months' period in two major hospitals of the Northern Territory (NT). A total of 429 VTE diagnoses were recorded over a 2-year period and 71 of 429 (17%) patients were Indigenous Australians. The overall incidence rate was 0.9 per 1,000 person-year for the population of the NT with a rate of 0.5 per 1,000 person-year for Indigenous Australians versus 1 per 1,000 person-year for non-Indigenous Australians. Of the 71, 39 (55%) of the VTE cases in the Indigenous group occurred in patients younger than 50 years and almost half of these (n = 18) were younger than 29 years. Hospitalization was found to be a major risk factor for VTE in 20 (38%) of the 54 Indigenous Australians of whom 10 (26%) patients were younger than 50 years. Although the rate of VTE in Indigenous Australians was low, its onset was significantly earlier in life and it was often triggered by prolonged hospitalization. VTE therefore should be added to the list of adverse outcomes of poor health and chronic diseases, which cause disproportional high rates of hospitalization amongst Indigenous Australians. The low number of VTE observed in older Indigenous patients in our study possibly reflects on the lower life expectancy and ongoing wide gap in life expectancy between Indigenous and non-Indigenous Australians.
    Seminars in Thrombosis and Hemostasis 10/2014;
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    ABSTRACT: Factor XIII/13 (FXIII or F13) is a plasma protransglutaminase, which stabilizes fibrin clots, and thus plays an important role in hemostasis. Autoimmune hemo(rrha)philia due to anti-F13 autoantibodies (AH13) has been on the rise in Japan, which has become the leading superaging society in the 21st century. The mean age of Japanese AH13 cases has risen to 70.4 years. A total of 83 AH13 cases have been diagnosed in the world as of July 2014. To raise the awareness of AH13, the author and members of the Japanese Collaborative Research Group first proposed "Criterion and Algorithm of Laboratory Tests for anti F13" in February 2012. AH13 is not just an acquired isolated defect of F13 molecule itself but a disturbance caused by autoantibodies. Accordingly, AH13 cases are diagnosed in patients with otherwise unexplained hemorrhages by a combination of a severe deficiency of F13 activity and the presence of anti-F13 autoantibodies. As patients with this disease manifest life-threatening bleeding symptoms, prompt diagnosis and proper treatment are essential. Because AH13 tends to become chronic and intractable, affected patients must be closely followed for a prolonged period.
    Seminars in Thrombosis and Hemostasis 09/2014;
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    ABSTRACT: The risk for both arterial and venous thrombosis increases with age. Despite the increasing burden of strokes related to atrial fibrillation (AF) and venous thromboembolism (VTE) among older adults, the use of anticoagulant therapy is limited in this population due to the parallel increase in risk of serious hemorrhage. Understanding the risks and their underlying mechanisms would help to mitigate adverse events and improve persistence with these life-saving therapies. The objectives of this review are to: (1) elucidate the age-related physiologic changes that render this high risk subgroup susceptible to hemorrhage, (2) identify mutable risk factors and hazards contributing to an increased bleeding risk in older individuals, and (3) discuss interventions to optimize anticoagulation therapy in this population.
    Seminars in Thrombosis and Hemostasis 09/2014;
  • Seminars in Thrombosis and Hemostasis 09/2014; 40(6):619-20.
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    ABSTRACT: Microparticles (MPs) are membrane-bound vesicles with important physiologic effects. MPs exchange information intercellularly, with each kind of MP carrying antigens and receptors of the cells from which they originated. They are biologic effectors in inflammation, angiogenesis, vascular injury, and thrombosis. Thrombosis is generally caused by abnormalities in blood flow, blood composition, and/or properties of the vessel wall. Thrombosis is a well-described feature of cardiovascular disease and cerebrovascular disease. Accumulating evidence suggests that increased risk of thrombosis is also characteristic of autoimmune disorders and immune-mediated diseases affecting all age groups, although the older adults are most vulnerable. Current research has also implicated MPs as a source of autoantigenic nuclear material that can form immune complexes, activate the innate immune system, and may lead to autoimmunity. This review focuses on the contribution of MPs to both the pathogenesis of autoimmune diseases and, as the immune and coagulation systems are tightly linked, their role in hypercoagulability in the setting of autoimmunity in an aging population.
    Seminars in Thrombosis and Hemostasis 08/2014;
  • [Show abstract] [Hide abstract]
    ABSTRACT: The average age of the US population continues to increase. Age is the most important determinant of disease and disability in humans, but the fundamental mechanisms of aging remain largely unknown. Many age-related diseases are associated with an impaired fibrinolytic system. Elevated plasminogen activator inhibitor-1 (PAI-1) levels are reported in age-associated clinical conditions including cardiovascular diseases, type 2 diabetes, obesity and inflammation. PAI-1 levels are also elevated in animal models of aging. While the association of PAI-1 with physiological aging is well documented, it is only recently that its critical role in the regulation of aging and senescence has become evident. PAI-1 is synthesized and secreted in senescent cells and contributes directly to the development of senescence by acting downstream of p53 and upstream of insulin-like growth factor binding protein-3. Pharmacologic inhibition or genetic deficiency of PAI-1 was shown to be protective against senescence and the aging-like phenotypes in kl/kl and N(ω)-nitro-l-arginine methyl ester-treated wild-type mice. Further investigation into PAI-1's role in senescence and aging will likely contribute to the prevention and treatment of aging-related pathologies.
    Seminars in Thrombosis and Hemostasis 08/2014;
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    ABSTRACT: Inherited macrothrombocytopenias are a clinically heterogeneous group of disorders, many of which cause moderate-to-severe bleeding tendencies in affected individuals, but which remain under-recognized and are frequently misdiagnosed as immune thrombocytopenia purpura. Diagnostic strategies to date have included a predominant phenotypic approach. The emergence of genetic testing and the implementation of next generation sequencing strategies in the investigation and diagnosis of these disorders have broadened our understanding of their pathogenesis, classification, and presentation. This review describes the increasingly expanding group of recognized inherited macrothrombocytopenias and highlights their pathophysiology and the role of phenotypic and genetic testing in their description and diagnosis.
    Seminars in Thrombosis and Hemostasis 08/2014;
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    ABSTRACT: The nitric oxide (NO)/soluble guanylate cyclase (sGC) system is fundamental to endothelial control of vascular tone, but also plays a major role in the negative modulation of platelet aggregation. The phenomenon of platelet NO resistance, or decreased antiaggregatory response to NO, occurs increasingly with advanced age, as well as in the context of cardiovascular disease states such as heart failure, ischemic heart disease, and aortic valve disease. The central causes of NO resistance are "scavenging" of NO and dysfunction of sGC. In the current review, we discuss the roles of several modulators of NO synthesis and of the NO/sGC cascade on changes in platelet physiology with aging, together with potential therapeutic options to reduce associated thrombotic risk.
    Seminars in Thrombosis and Hemostasis 08/2014;
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    ABSTRACT: Nucleic acids (NAs) constitute the backbone of cellular life permitting conservation, transmission, and execution of genetic information. In the past few years, new unexpected functions for NAs, projecting them also beyond nuclear and cellular boundaries have been recognized: circulating cell-free nucleic acids (cfNAs), histones, DNA-histone complexes, microRNAs (miRs) may have a regulatory role in physiological and pathological processes. In particular, several lines of evidence suggest that they can constitute unconventional mediators of thrombus formation, intervening both in hemostasis and thrombosis. Furthermore, in the past decade, the possibility to detect and quantify these in plasma and/or in serum has led to their ancillary use as potential markers in various medical conditions. The use of these as markers within the fields of thrombosis and hemostasis looks promising: the potential implications include the possibility to assess patients' risk profiles for thrombotic events and the identification of more directed targets for pharmacologic intervention. The major impediment is that, to date, the methods by which NAs are explored, still largely differ between published studies and standardized procedures are still lacking. Future research should focus on the physiological mechanisms underlying the activities of such mediators in specific thrombotic conditions and on the definition of reliable methods for their quantification in biological fluids.
    Seminars in Thrombosis and Hemostasis 08/2014;
  • [Show abstract] [Hide abstract]
    ABSTRACT: Aging is associated with many physiological changes, which may in time lead to numerous pathophysiological outcomes, including adverse vascular events. For example, senescence of the immune system and cellular senescence both contribute to rising inflammation with age, potentially induced by the overall burden of comorbid illness, adipose tissue mass, diet, socioeconomic status, and physical activity. In turn, this chronic inflammation decreases physical and cognitive performance, and promotes sarcopenia and the syndrome of frailty. These events and others decrease the functionality of life as we age and include an increased risk of thrombosis and adverse cardiovascular outcomes. In this review, we aim to overview the aging process primarily as related to functional impairment, and provide evidence for the role of protein, and specifically differential quality protein, in particular whey protein, and timing and distribution of intake, to help reduce some of the morbid effects of aging, including reducing obesity, improving glycemic control, and improving vascular function.
    Seminars in Thrombosis and Hemostasis 08/2014;
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    ABSTRACT: von Willebrand factor (VWF) plays critical roles in initiating primary hemostasis and extending the half-life of coagulation factor VIII in circulation. VWF levels increase with age and elevated levels are associated with an increased risk of venous thromboembolism and cardiovascular disease (CVD). Patients with von Willebrand disease (VWD) due to a deficiency or dysfunction of VWF may have symptoms that ameliorate with aging or may have exacerbation of their disease. Bleeding sites of particular challenge in the aging patient include gastrointestinal bleeding and hematuria. Some medications used to treat VWD should be used with special precaution in older patients, including desmopressin and VWF-containing factor concentrates. Patients with VWD may have some protection from CVD, but in those patients who develop CVD, management is very challenging, given the role of antiplatelet therapy as the mainstay of treatment.
    Seminars in Thrombosis and Hemostasis 08/2014;
  • [Show abstract] [Hide abstract]
    ABSTRACT: The available evidence suggests that immunosenescence induces organismal proinflammatory responses. The chronic inflammation seen in advancing age stratifies persons into aging phenotypes. Even with adjustment for confounders, elevated inflammatory cytokines significantly decrease the odds of successful aging. This chronic inflammation seen in advancing age has varied causes, including comorbid illness, adipose tissue mass, diet, socioeconomic status, body mass index, gender, age, and physical activity. Aging can therefore be thought of as an acquired thrombophilia of increasing inflammation, impaired fibrinolytic potential, and a hypercoagulable state, out of proportion to physiological needs. Factors ranging from genetic to environmental contribute to the prothrombotic tendency of aging adults, especially those with concomitant frailty, to experience a decline in health status.
    Seminars in Thrombosis and Hemostasis 08/2014;
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    ABSTRACT: Thrombocytopenia in older patients is associated with several characteristics that are often underrecognized. Among them are the risk of bleeding complications, the senescence of the bone marrow, the increased use of multiple drugs, and greater comorbidity. In this article, the various forms of thrombocytopenia, including primary and drug-associated immune thrombocytopenias, as well as other drug-associated thrombocytopenias, are reviewed. In addition, how age-related changes in platelet function may affect the bleeding risk is discussed.
    Seminars in Thrombosis and Hemostasis 08/2014;
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    ABSTRACT: The introduction of new hemophilia management therapies, targeting extended half-lives through bioengineering, ushers in an era of potential promise and increasing complexity, more so for those with hemophilia B than hemophilia A. Questions arise for patients, caregivers, and hemophilia treatment center (HTC) staff about how to assess and incorporate novel therapies and how to determine whether new therapies offer a distinct advantage over established treatment routines. Nurses and other interdisciplinary HTC staff are well positioned to assess, educate, and support patients and families in navigating this rapidly changing landscape. To support these challenging efforts, this review offers a perspective on issues affecting therapeutic transitions and provides tools to foster ongoing adherence.
    Seminars in Thrombosis and Hemostasis 08/2014;
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    ABSTRACT: Plasminogen activator inhibitor-1 (PAI-1), a principal inhibitor of fibrinolysis, is induced in thrombotic, fibrotic, and cardiovascular diseases, which in turn primarily afflict the older population. This induction of PAI-1 may play an important role in the pathology of these diseases as PAI-1 can regulate the dissolution of fibrin and also inhibit the degradation of the extracellular matrix by reducing plasmin generation. PAI-1 expression is elevated in aged individuals and is significantly upregulated in a variety of pathologies associated with the process of aging, including myocardial and cerebral infarction, vascular (athero) sclerosis, cardiac and lung fibrosis, metabolic syndromes (e.g., hypertension, hyperlipidemia, and insulin resistance), cancer, and inflammatory/stress responses. Thus, PAI-1 may play a critical role in the development of aging-associated pathological changes. In addition, PAI-1 is recognized as a marker of senescence and a key member of a group of proteins collectively known as the senescence-messaging secretome. In this review, we highlight the role of PAI-1 in the pathophysiology of aging and aging-associated disorders.
    Seminars in Thrombosis and Hemostasis 08/2014;