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Case Report: A hidden cause of severe hemorrhage: a diagnostic and therapeutic challenge
Ziying Han1, Jing Ruan2, Li Wang3,4
1General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Abstract:
Acquired factor XIII (FXIII) deficiency is a rare but life-threatening cause of hemorrhagic diseases. We report a case of a patient presenting with life-threatening bleeding with normal routine coagulation tests. The diagnosis was confirmed by a specific FXIII activity assay. The subsequent absence of ANA delayed the definitive diagnosis, which was ultimately established as ANA-negative systemic lupus erythematosus (SLE) based on comprehensive clinical criteria and multisystem involvement. This case presented a profound diagnostic dilemma, requiring not only the identification of a rare acquired FXIII deficiency as the cause of unexplained bleeding, but also the confirmation of SLE despite seronegativity, relying on its multisystem manifestations.
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