Laboratory Assessment of Factor VIII Inhibitors: When Is It Required? A Perspective Informed by Local Practice DOI Open Access
Emmanuel J. Favaloro, Jennifer Curnow, Leonardo Pasalic

и другие.

Journal of Clinical Medicine, Год журнала: 2024, Номер 14(1), С. 13 - 13

Опубликована: Дек. 24, 2024

This perspective discusses the critical role of laboratory assessments in assessing factor VIII (FVIII) inhibitors. These are auto- and alloantibodies that can develop against both endogenous exogenous FVIII, respectively. Assessment for inhibitors represents a key part management congenital hemophilia A (CHA), an inherited deficiency, acquired (AHA), autoimmune condition. Both conditions pose significant bleeding risks, necessitating careful monitoring FVIII levels inhibitor presence level. Laboratory assays, particularly Bethesda assay, essential detecting these their levels. The complexities kinetics may challenges to interpretation assay results, such even normal do not always exclude presence. Clinical practice guidelines recommend ongoing AHA/CHA patients until no longer detectable. Overall, timely evaluations optimizing treatment strategies with hemophilia, aiming improve patient outcomes quality life. We summarize our approach assessment inhibitors, as reflecting informed by local practice.

Язык: Английский

Cerebromicrovascular mechanisms contributing to long COVID: implications for neurocognitive health DOI Creative Commons
Mónika Fekete, Andrea Ceglédi,

Ágnes Szappanos

и другие.

GeroScience, Год журнала: 2025, Номер unknown

Опубликована: Янв. 7, 2025

Abstract Long COVID (also known as post-acute sequelae of SARS-CoV-2 infection [PASC] or post-COVID syndrome) is characterized by persistent symptoms that extend beyond the acute phase infection, affecting approximately 10% to over 30% those infected. It presents a significant clinical challenge, notably due pronounced neurocognitive such brain fog. The mechanisms underlying these effects are multifactorial, with mounting evidence pointing central role cerebromicrovascular dysfunction. This review investigates key pathophysiological contributing cerebrovascular dysfunction in long and their impacts on health. We discuss how endothelial tropism direct vascular trigger dysfunction, impaired neurovascular coupling, blood–brain barrier disruption, resulting compromised cerebral perfusion. Furthermore, appears induce mitochondrial enhancing oxidative stress inflammation within cells. Autoantibody formation following also potentially exacerbates injury, chronic ongoing compromise. These factors collectively contribute emergence white matter hyperintensities, promote amyloid pathology, may accelerate neurodegenerative processes, including Alzheimer’s disease. emphasizes critical advanced imaging techniques assessing health need for targeted interventions address complications. A deeper understanding essential advance treatments mitigate its long-term consequences.

Язык: Английский

Процитировано

2

Acquired Hemophilia—A Case Series and Review DOI Open Access
Liat Waldman Radinsky,

Maayan Sivan,

Aharon Lubetsky

и другие.

Journal of Clinical Medicine, Год журнала: 2025, Номер 14(5), С. 1597 - 1597

Опубликована: Фев. 26, 2025

Background: Acquired hemophilia A (AHA) is a rare, life-threatening autoimmune disorder characterized by inhibitory autoantibodies against factor VIII (FVIII), resulting in spontaneous or trauma-related bleeding. This study reviews single-center cohort to evaluate patient characteristics, treatments, and outcomes. Methods: We retrospectively reviewed the records of 22 adult patients diagnosed with AHA between 2012 2024. The data included demographics, clinical presentation, laboratory findings, Statistical analysis compared genders evaluated treatment strategies remission Results: had an equal gender distribution average age 62 years (22–102 years). Suspected etiologies pregnancy (27%), malignancy (23%), diseases (5%), idiopathic causes (45%). most common presentation was cutaneous hematoma (82%). Severe bleeding necessitating hemostatic therapy occurred 9% cases. Initial immunosuppressive (IST) corticosteroids achieved 45% patients, while additional Rituximab Cyclophosphamide required others. Emicizumab, novel FVIII-mimetic, successfully used one severe refractory Remission 64% within median 3 months, recurrence rate 14%. No thrombotic events were observed, although corticosteroid side effects, including hip fracture, noted. Conclusions: IST remains cornerstone treatment, though effects necessitate individualized care. Emicizumab shows promise, particularly cases fragile populations. Future research needed long-term outcomes rates, especially special populations like post-partum women elderly.

Язык: Английский

Процитировано

0

Laboratory Assessment of Factor VIII Inhibitors: When Is It Required? A Perspective Informed by Local Practice DOI Open Access
Emmanuel J. Favaloro, Jennifer Curnow, Leonardo Pasalic

и другие.

Journal of Clinical Medicine, Год журнала: 2024, Номер 14(1), С. 13 - 13

Опубликована: Дек. 24, 2024

This perspective discusses the critical role of laboratory assessments in assessing factor VIII (FVIII) inhibitors. These are auto- and alloantibodies that can develop against both endogenous exogenous FVIII, respectively. Assessment for inhibitors represents a key part management congenital hemophilia A (CHA), an inherited deficiency, acquired (AHA), autoimmune condition. Both conditions pose significant bleeding risks, necessitating careful monitoring FVIII levels inhibitor presence level. Laboratory assays, particularly Bethesda assay, essential detecting these their levels. The complexities kinetics may challenges to interpretation assay results, such even normal do not always exclude presence. Clinical practice guidelines recommend ongoing AHA/CHA patients until no longer detectable. Overall, timely evaluations optimizing treatment strategies with hemophilia, aiming improve patient outcomes quality life. We summarize our approach assessment inhibitors, as reflecting informed by local practice.

Язык: Английский

Процитировано

1