Acquired factor XIII deficiency: A scoping review

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.
• 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.