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Prevalence of selected bleeding and thrombotic events in  persons with hemophilia versus the general population: A scoping review
Res Pract Thromb Haemost. 2022 Dec 9;7(1):100007. doi: 10.1016/j.rpth.2022.100007. eCollection 2023 Jan.ABSTRACTLife expectancy for persons with hemophilia has increased over recent decades due to advances in treatment practice and patient care. Those with hemophilia are now more likely to be affected by conditions associated with aging, such as myocardial infarction, hemorrhagic/ischemic stroke, deep vein thrombosis, pulmonary embolism, and intracranial hemorrhage. Here, we describe the results of a literature search designed to summarize current data on the prevalence of the above selected bleeding and thrombotic events ...
Source: Thrombosis and Haemostasis - March 9, 2023 Category: Hematology Authors: Amy D Shapiro Brandon M Hardesty Flora Peyvandi Alfonso Iorio Source Type: research

Left atrial appendage occlusion for management of atrial fibrillation in persons with hemophilia
Due to advancements in hemophilia care, the overall life expectancy of persons with hemophilia is almost approaching that of the general population [1]. As a result, a growing population of elderly hemophilia patients is now prone to the same age-related cardiovascular comorbidities, such as coronary artery disease (CAD) and atrial fibrillation (AF) [2,3]. The management of AF is a challenge as antithrombotic therapy, the standard of care, is usually contraindicated in persons with hemophilia. Left atrial appendage occlusion (LAAO) is an alternative option for stroke prevention in patients with AF with a contraindication t...
Source: Thrombosis Research - August 4, 2021 Category: Hematology Authors: Ming Y. Lim, Mouhamed Yazan Abou-Ismail Tags: Letter to the Editors-in-Chief Source Type: research

Left atrial appendage closure: a balanced management of the thromboembolic risk in patients with hemophilia and atrial fibrillation
AbstractAtrial fibrillation is the most common cardiac arrhythmia and is a major cause of embolic stroke. In patients at high bleeding risk such as those with hemophilia, the thromboembolic prevention is challenging. We herein present three patients affected by moderate to severe hemophilia and atrial fibrillation that were treated in our Institution between March 2018 and September 2019, with percutaneous left atrial appendage closure. In patients with relative or absolute contraindications to long-term anticoagulant therapy, the left atrial appendage closure could represent a reasonable strategy.
Source: Journal of Thrombosis and Thrombolysis - April 7, 2020 Category: Hematology Source Type: research

Emicizumab for the Treatment of Acquired Hemophilia_A: Lessons Learned from 4 Very Different Cases
In conclusion, emiczumab seems to be an effective hemostatic therapy also for AHA, which offers several advantages: subcutaneous weekly therapy, good hemostatic efficacy, possible outpatient therapy, the option to reduce the intensity of immunosuppressive therapy to avoid side effects (as the patients are protected from bleeding), and seems even to be more cost effective than bypassing agents. However, special attention is necessary on the use of appropriate lab assays (chromogenic FVIII assays), the artificial effects on APTT and Bethesda assays, the recognition of the remission, and to avoid the concomitant use of APCC. ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Knoebl, P., Sperr, W. R., Schellongowski, P., Staudinger, T., Jilma-Stohlawetz, P., Quehenberger, P., Koder, S., Ay, C., Gleixner, K. V. Tags: 322. Disorders of Coagulation or Fibrinolysis: Poster II Source Type: research

Phase 2/3 Trial of Subcutaneously Administered Marzeptacog Alfa (activated) an Engineered FVIIa in Hemophilia with Inhibitors - Pharmacokinetics, Pharmacodynamics, Safety and Efficacy
Conclusion: This study demonstrates that an individualized dose of daily SQ MarzAA can provide effective prophylaxis in hemophilia patients with inhibitors.Supported by Catalyst BiosciencesDisclosuresLevy: Catalyst Biosciences: Employment, Equity Ownership. Mahlangu: Sanofi: Research Funding, Speakers Bureau; Roche: Consultancy, Research Funding, Speakers Bureau; LFB: Consultancy; NovoNordisk: Consultancy, Research Funding, Speakers Bureau; CSL Behring: Consultancy, Research Funding, Speakers Bureau; Chugai: Consultancy; Catalyst Biosciences: Consultancy, Research Funding; Biomarin: Research Funding, Speakers Bureau; Bioge...
Source: Blood - November 21, 2018 Category: Hematology Authors: Levy, H., Makhaldiani, L., Iosava, G., Mahlangu, J., Kosinova, M. V., Khacchatryan, H., Del Greco, F., Booth, F. V. M. Tags: 322. Disorders of Coagulation or Fibrinolysis: Poster I Source Type: research

Chronic Kidney Disease (CKD) in the U.S. Hemophilia Population: A Cohort Study
Conclusions: In this interim analysis of an ongoing national prospective cohort study, older men with moderate to severe hemophilia commonly report risk factors for CKD, including HTN (51.5%), DM, viral infection, and potential renal damaging medication use. Only 11.6% had CVD. Urological symptoms were also common, including hematuria and obstructive symptoms with urination.In our cohort, 11.4% met the definition of CKD, defined as the presence of either kidney damage or GFR < 60 ml/min/1.73 m2 for ≥ 3 months. The distribution of GFR values appeared similar to the general population. As with risk factors associated w...
Source: Blood - November 21, 2018 Category: Hematology Authors: Sood, S. L., Cheng, D., Shapiro, A., Kessler, C. M., Key, N. S., Quon, D., Eyster, M. E., Manco-Johnson, M. J., Kempton, C. L., Cuker, A., Ragni, M. V., Kuriakose, P., von Drygalski, A., Kouides, P. A., Escobar, M. A., Wheeler, A. P., Wang, T.-F., Leissin Tags: 322. Disorders of Coagulation or Fibrinolysis: Poster II Source Type: research

How to compare cardiovascular disease and risk factors in elderly patients with haemophilia with the general population
ConclusionIncreased prevalence of hypertension in PWH should trigger regular screening. CHD does occur in PWH aged ≥60 years though apparently with lower prevalence. Given the growing population of elderly PWH, guidelines for prevention and treatment of CVD should be developed.
Source: Haemophilia - September 20, 2016 Category: Hematology Authors: K. Berger, D. Schopohl, G. Lowe, P. A. Holme, R. C. Tait, C. Combescure, S. Rauchensteiner, R. Klamroth Tags: Original Article Source Type: research

How I treat patients with inherited bleeding disorders who need anticoagulant therapy
Situations that ordinarily necessitate consideration of anticoagulation, such as arterial and venous thrombotic events and prevention of stroke in atrial fibrillation, become challenging in patients with inherited bleeding disorders such as hemophilia A, hemophilia B, and von Willebrand disease. There are no evidence-based guidelines to direct therapy in these patients, and management strategies that incorporate anticoagulation must weigh a treatment that carries a risk of hemorrhage in a patient who is already at heightened risk against the potential consequences of not treating the thrombotic event. In this paper, we rev...
Source: Blood - July 13, 2016 Category: Hematology Authors: Martin, K., Key, N. S. Tags: How I Treat, Free Research Articles, Thrombosis and Hemostasis, Clinical Trials and Observations Source Type: research

Atrial fibrillation in patients with haemophilia: a cross‐sectional evaluation in Europe
Summary Atrial fibrillation (AF) is a common health problem in the general population, but data on prevalence or management in patients with haemophilia (PWH) are lacking. The aims of this study were to analyse the prevalence of AF and risk factors for stroke using a cross‐sectional pan‐European design and to document current anticoagulation practice. The ADVANCE Working Group consists of members from 14 European haemophilia centres. Each centre retrieved data on their PWH with AF. From the total of 3952 adult PWH, 33 had AF with a mean age of 69 years (IQR 62–76). Haemophilia was severe in seven (21%), moderate in ...
Source: Haemophilia - March 1, 2014 Category: Hematology Authors: Roger E. G. Schutgens, R. Klamroth, I. Pabinger, M. Malerba, G. Dolan, Tags: Original Article Source Type: research

Cardiovascular comorbidities are increased in US patients with haemophilia A: a retrospective database analysis
This study evaluated the prevalence of cardiovascular comorbidities among USA males with haemophilia A, relative to an unaffected general male population with similar characteristics. Males with haemophilia A and continuous insurance coverage were identified by ICD‐9‐CM code 286.0 (1 January 2007–31 December 2009) using the MarketScan® Commercial and Medicare Research Databases. Individuals with haemophilia A were exact matched 1:3 with males without a diagnosis of haemophilia A. The prevalence of cardiovascular comorbidities identified by ICD‐9‐CM code was determined for matched cohorts. Of the study population...
Source: Haemophilia - November 29, 2013 Category: Hematology Authors: J. Pocoski, A. Ma, C. M. Kessler, S. Boklage, T. J. Humphries Tags: Original Article Source Type: research