Acquired FXIII inhibitors: a systematic review

Area of Science:

• Hematology
• Immunology
• Protein Biochemistry

Background:

• Coagulation factor XIII (FXIII) stabilizes fibrin clots via covalent cross-linking.
• Acquired FXIII deficiency can result from autoantibodies (inhibitors) against FXIII.
• These rare inhibitors can lead to life-threatening bleeding, often linked to autoimmune or lymphoproliferative disorders, or medications.

Purpose of the Study:

• To systematically review published case reports of anti-FXIII autoantibodies.
• To analyze the clinical manifestations of acquired FXIII deficiency caused by these inhibitors.
• To evaluate the treatment strategies employed for this rare hemorrhagic condition.

Main Methods:

• Systematic literature search of case reports involving anti-FXIII autoantibodies.
• Data extraction on patient demographics, clinical presentation, and bleeding complications.
• Analysis of treatment modalities, including inhibitor eradication and FXIII level restoration.

Main Results:

• Acquired FXIII deficiency due to autoantibodies is a rare but serious condition.
• Bleeding complications associated with anti-FXIII inhibitors can be severe and life-threatening.
• Management requires a dual approach: targeting inhibitor removal and boosting FXIII plasma levels.

Conclusions:

• Anti-FXIII autoantibodies represent a critical, albeit rare, cause of acquired FXIII deficiency.
• Effective management strategies are crucial for patients with this condition.
• Further research may elucidate optimal treatment protocols for acquired FXIII inhibitors.