Acquired Factor XIII Deficiency with Multiple Hematoma

Acquired factor XIII (FXIII) deficiency is a rare but potentially life-threatening hemorrhagic disorder, most commonly observed in middle-aged and elderly individuals. Due to its normal routine coagulation profile, it is frequently misdiagnosed or diagnosed late, especially in patients presenting with unexplained bleeding or hematoma.

We report a case of acquired FXIII deficiency in a patient with recurrent, unexplained spontaneous hematomas and normal coagulation results. The diagnostic process includes a urea clot lysis test as an initial screen for FXIII deficiency, followed by specific tests, such as the chloroacetic acid lysis test, and assessment of residual FXIII activity. It is treated with infusion of cold precipitates and the use of immunosuppressive therapies, including corticosteroids and azathioprine.

The patient showed poor response to conventional multidisciplinary management. Finally, the patient is automatically discharged to a higher-level hospital.

This case underscores the need to consider rare coagulation disorders like acquired FXIII deficiency in patients with unexplained hematomas and normal routine coagulation results. Early identification using targeted diagnostic tests can guide prompt and effective management. Treatment remains challenging in patients with poor response to cold precipitation, corticosteroids, and azathioprine.