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Related Experiment Videos

Acquired factor XIII deficiency: A scoping review.

Olivier Duranteau1, Guney Tatar1, Anne Demulder1

  • 1From Anesthesiology Service Department, CUB-ULB Hôpital Erasme, Brussels, Belgium (OD, GT, TT), Intensive Care Unit, Percy Military Training Hospital, Clamart, France (OD), Faculté de Médecine, Université Libre de Bruxelles, Brussels, Belgium (OD, AD, TT), Laboratory of Hematology LHUB-ULB ULB, Université Libre de Bruxelles, Brussels, Belgium (AD).

European Journal of Anaesthesiology and Intensive Care
|February 7, 2025
PubMed
Summary

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Disorders of Hemostasis01:24

Disorders of Hemostasis

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Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
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Anticoagulant Drugs: Low-Molecular-Weight Heparins01:30

Anticoagulant Drugs: Low-Molecular-Weight Heparins

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Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...
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Acquired factor XIII (FXIII) deficiency, often autoimmune, presents with bleeding symptoms. Treatment involves FXIII concentrate, immunosuppressants like corticosteroids or rituximab for autoimmune cases, or concentrate alone for consumption-based deficiency.

Area of Science:

  • Hematology
  • Immunology
  • Internal Medicine

Background:

  • Acquired factor XIII (FXIII) deficiency is a rare hemostatic disorder.
  • It can stem from autoimmune antibody production or non-immune factors like diminished synthesis or increased consumption.
  • This condition presents a diagnostic and therapeutic challenge.

Purpose of the Study:

  • To review the clinical presentation, diagnostic approaches, and treatment strategies for acquired FXIII deficiency.
  • To identify the most effective and commonly used treatments.
  • To analyze patient outcomes based on different therapeutic interventions.

Main Methods:

  • A systematic literature review was conducted.
  • Databases searched included PubMed, Medline, and Embase/Ovid from January 2012 to April 2022.

Related Experiment Videos

  • Keywords used were 'Acquired FXIII deficiency'; 36 articles met eligibility criteria.

    • Main Results:

    • The majority of acquired FXIII deficiency cases (24/30) were autoimmune in origin.
    • Local hematoma was the most frequent symptom (31 cases).
    • Treatment involved FXIII concentrate, corticosteroids, cyclophosphamide, or rituximab for autoimmune cases, and FXIII concentrate for consumption-based deficiency.

    Conclusions:

    • Acquired FXIII deficiency requires tailored treatment based on its underlying cause (autoimmune vs. non-immune).
    • Effective management strategies include immunosuppressive therapy and FXIII concentrate supplementation.
    • Prompt and appropriate treatment is crucial for improving patient outcomes and reducing mortality.