Clinical burden of hemophilia in older adults: Beyond bleeding risk

Hodroj, Mohammad Hassan; Hasbani, Georges El; Al-Shamsi, Humaid O.; Samaha, Hanady; Musallam, Khaled M.; Taher, Alì

doi:10.1016/j.blre.2021.100912

Cited by 5 publications

(9 citation statements)

References 99 publications

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“…Haemophilia A (HA) and Haemophilia B (HB) are the most common severe bleeding disorders in humans, and are caused by inherited X‐linked mutations in the genes encoding coagulation factor VIII (FVIII) in the case of HA and factor IX (FIX) in the case of HB (Hodroj et al., 2021 ). Recent reports on the worldwide distribution indicate the prevalence of HA and HB to be 17.1 and 3.8 cases per 100,000 males, respectively (Mannucci, 2020b ).…”

Section: Resultsmentioning

confidence: 99%

Proteomic analysis of MSC‐derived apoptotic vesicles identifies Fas inheritance to ameliorate haemophilia a via activating platelet functions

Zhang

Tang

Kou

et al. 2022

J of Extracellular Vesicle

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Apoptotic vesicles (apoVs) are apoptotic cell‐derived nanosized vesicles that play a crucial role in multiple pathophysiological settings. However, their detailed characteristics, specific surface markers, and biological properties are not fully elucidated. In this study, we compared mesenchymal stem cell (MSC)‐derived apoVs and exosomes from three different types of MSCs including human bone marrow MSCs (hBMSCs), human adipose MSCs (hASCs), and mouse bone marrow MSCs (mBMSCs). We established a unique protein map of MSC‐derived apoVs and identified the differences between apoVs and exosomes in terms of functional protein cargo and surface markers. Furthermore, we identified 13 proteins specifically enriched in apoVs compared to exosomes, which can be used as apoV‐specific biomarkers. In addition, we showed that apoVs inherited apoptotic imprints such as Fas to ameliorate haemophilia A in factor VIII knockout mice via binding to the platelets’ FasL to activate platelet functions, and therefore rescuing the blood clotting disorder. In summary, we systemically characterized MSC‐derived apoVs and identified their therapeutic role in haemophilia A treatment through a previously unknown Fas/FasL linkage mechanism.

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Section: Resultsmentioning

confidence: 99%

Proteomic analysis of MSC‐derived apoptotic vesicles identifies Fas inheritance to ameliorate haemophilia a via activating platelet functions

Zhang

Tang

Kou

et al. 2022

J of Extracellular Vesicle

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“…There are long-term clinical concerns associated with hemophilia outcomes, including chronic viral infections with liver disease, joint impairment with bone disease, cardiovascular disease, cancers, chronic kidney disease, covert cerebral microbleeds, impaired sexuality and development of immune factor inhibitors. 18 …”

Section: Present Health Care Status Of Females With Inherited Bleedin...mentioning

confidence: 99%

Preoperative diagnosis and management of inherited bleeding disorders in female adolescents and adults

Wilson

2023

cjs

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Although inherited bleeding disorders (IBDs) affect both females and males, this review of the preoperative diagnosis and management of IBDs focuses on genetic and gynecologic screening, diagnosis and management of affected and carrier females. A PubMed literature search was conducted, and the peer-reviewed literature on IBDs was evaluated and summarized. Best-practice considerations for screening, diagnosis and management of IBDs in female adolescents and adults, with GRADE (Grading of Recommendations, Assessment, Development, and Evaluations) evidence level and ranking of recommendation strength, are presented. Health care providers need to increase their recognition of and support for female adolescents and adults with IBDs. Improved access to counselling, screening, testing and hemostatic management is also required. Patients should be educated and encouraged to report abnormal bleeding symptoms to their health care provider when they have a concern. It is hoped that this review of preoperative IBD diagnosis and management will enhance access to women-centred care to increase patients’ understanding of IBDs and decrease their risk of IBD-related morbidity and mortality.

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“…The current global prevalence of haemophilia A and B is close to 17.1 and 3.8 cases per 100,000 males, respectively 4 . From the early 1970s, with the manufacture of specific plasma‐derived factor concentrates and the use of recombinant DNA technology, the mortality rate in haemophilia was significantly reduced 5 . Over the past decade, life expectancy for PWH has increased from 20 years in the 11th century to 55−63 years after the introduction of factor concentrates and more than 65 years in recent reports from the Netherlands and Italy 5 …”

Section: Introductionmentioning

confidence: 99%

Bone mineral density in haemophilia patients: A systematic review and meta‐analysis

Zhang,

Song,

2024

Haemophilia

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IntroductionWith the increase in life expectancy of haemophilia patients (PWH), the risk of osteoporosis increases, but there is little research on whether haemophilia is the cause of osteoporosis.AimTo conduct systematically review whether bone mineral density (BMD) in PWH decreased and the factors affecting BMD.MethodsTwo authors independently searched databases and reviewed citations from relevant articles, selecting studies published in any language and performed in humans before March 2023. Eligibility criteria were observational studies in PWH, with BMD as at least one outcome other than osteoporosis or bone loss, and analyses in a group of PWH and healthy controls.ResultsTwelve studies were ultimately identified, consisting of 1210 individuals (534 PWH and 676 healthy controls), compared with the control group, BMD in PWH decreased by 0.13 g/cm2 [95% confidence interval (CI) ‐0.18 to ‐0.08, I2 = 89%]. No evidence of publication bias was detected. There was no evidence that age, BMI, level of physical activity, the types of haemophilia, haemophilia severity, a blood‐borne virus (HCV) and treatment modality predicted the BMD in PWH.ConclusionThe results indicate that BMD in PWH is lower than in healthy controls. Therefore, we strongly recommend PWH early measurement of BMD to prevent osteoporosis.

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Clinical burden of hemophilia in older adults: Beyond bleeding risk

Cited by 5 publications

References 99 publications

Proteomic analysis of MSC‐derived apoptotic vesicles identifies Fas inheritance to ameliorate haemophilia a via activating platelet functions

Proteomic analysis of MSC‐derived apoptotic vesicles identifies Fas inheritance to ameliorate haemophilia a via activating platelet functions

Preoperative diagnosis and management of inherited bleeding disorders in female adolescents and adults

Bone mineral density in haemophilia patients: A systematic review and meta‐analysis

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