Does haemophilia slow down the development of liver fibrosis?

Dievoet, Marie-Astrid van; Leclercq, Isabelle; Hermans, Cédric; Lambert, Catherine; Horsmans, Yves; Jacquemin, Marc; Eeckhoudt, Stéphane

doi:10.1111/hae.13630

Cited by 3 publications

(2 citation statements)

References 15 publications

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“…Generally, however, the more severe disorders present earlier on in life. Another case report demonstrates that moderate factor X deficiency does not necessarily present early in life or as a spontaneous hemorrhage as in our patient [7].…”

Section: Discussionmentioning

confidence: 50%

Inherited Moderate Factor X Deficiency Presenting as Cardiac Tamponade

Othman

Abdelkarim

Huynh

et al. 2019

Case Reports in Hematology

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Factor X deficiency is a rare bleeding disorder that varies in the severity of its clinical manifestations. The symptoms of this disorder can occur at any age, although most severe cases appear in childhood. The rarity of this condition has not allowed for the establishment of evidence‐based management guidelines, and thus, individuals afflicted with factor X deficiency are treated based on limited literature and the opinions of clinicians with extensive experience. In this case report, we discuss a unique presentation of a 38-year-old male who was found to have cardiac tamponade as a result of his newly diagnosed inherited moderate factor X deficiency. This was discovered by obtaining a factor X activity assay and confirmed with genetic testing which demonstrated a missense variant on the factor X gene on chromosome 13. His management involved correction of his factor X deficiency with fresh frozen plasma, a pericardiocentesis, and placement of a pericardial window. He has been asymptomatic and without hemorrhagic episodes for the 10 months following his discharge.

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Section: Discussionmentioning

confidence: 50%

Inherited Moderate Factor X Deficiency Presenting as Cardiac Tamponade

Othman

Abdelkarim

Huynh

et al. 2019

Case Reports in Hematology

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“…One study also suggests that HA has a protective effect against liver fibrosis development in a murine model. 33 It could be attributed to reduced thrombin generation, resulting in a lowered ability to activate hepatic stellate cells and platelets via the protease-activated receptor pathway. Emerging evidence also shows that FVIII is involved in multiple biological systems, including bone, vascular, and immune systems.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of non-Alcoholic Fatty Liver Disease and Associated Factors in Patients with Moderate or Severe Hemophilia: A Multicenter-Based Study

Shen

Chiou

Chou

et al. 2022

Clin Appl Thromb Hemost

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Introduction Liver health is essential for persons with hemophilia (PWH) in order to maintain access to new therapies, such as gene therapy. Non-alcoholic fatty liver disease (NAFLD) is seldom reported in the hemophilia population. The study aimed to investigate the prevalence of NAFLD and associated factors in PWH. Methods Data of this cross-sectional study were obtained from a multicenter collaborative registry database. Results A total of 163 moderate or severe PWH with a complete data of liver examination were analyzed. There were 77 (47.2%) PWH diagnosed with NAFLD. The multivariate analysis showed that overweight/obesity was associated with NAFLD (OR, 4.31, P < .001). In comparison with hemophilia B patients, hemophilia A patients showed a weaker correlation with NAFLD, (OR, 0.30, P = .009). A total of 17 (25.8%) PWH with NAFLD had an elevated level of alanine transaminase (ALT). Both overweight/obesity and presence of inhibitor to clotting factor were independently associated with elevated ALT in PWH with NAFLD. Conclusions The study indicated that a high prevalence of NAFLD existed in the hemophilia population. Overweight/obesity was an independent factor for NAFLD and elevated ALT.

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Healing gone wrong: convergence of hemostatic pathways and liver fibrosis?

Davis

Caldwell

2020

Clinical Science

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Fibrosis results from a disordered wound healing response within the liver with activated hepatic stellate cells laying down dense, collagen-rich extracellular matrix that eventually restricts liver hepatic synthetic function and causes increased sinusoidal resistance. The end result of progressive fibrosis, cirrhosis, is associated with significant morbidity and mortality as well as tremendous economic burden. Fibrosis can be conceptualized as an aberrant wound healing response analogous to a chronic ankle sprain that is driven by chronic liver injury commonly over decades. Two unique aspects of hepatic fibrosis – the chronic nature of insult required and the liver’s unique ability to regenerate – give an opportunity for pharmacologic intervention to stop or slow the pace of fibrosis in patients early in the course of their liver disease. Two potential biologic mechanisms link together hemostasis and fibrosis: focal parenchymal extinction and direct stellate cell activation by thrombin and Factor Xa. Available translational research further supports the role of thrombosis in fibrosis. In this review, we will summarize what is known about the convergence of hemostatic changes and hepatic fibrosis in chronic liver disease and present current preclinical and clinical data exploring the relationship between the two. We will also present clinical trial data that underscores the potential use of anticoagulant therapy as an antifibrotic factor in liver disease.

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Does haemophilia slow down the development of liver fibrosis?

Cited by 3 publications

References 15 publications

Inherited Moderate Factor X Deficiency Presenting as Cardiac Tamponade

Inherited Moderate Factor X Deficiency Presenting as Cardiac Tamponade

Prevalence of non-Alcoholic Fatty Liver Disease and Associated Factors in Patients with Moderate or Severe Hemophilia: A Multicenter-Based Study

Healing gone wrong: convergence of hemostatic pathways and liver fibrosis?

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