Diagnosis and Management of an Acquired Factor XIII Deficiency in a Limited Resource Setting: A Case Report

Factor XIII deficiency, a rare bleeding disorder, can be either immune – mediated or non -immune and present significant challenges due to potential life-threatening bleeding. This report underscores the importance of targeted management in FXIII deficiency, particularly in low- resource setting where advanced testing may be inaccessible.

The case centers on 16-year-old boy with a history of mild bleeding who -presented with a subdural hemorrhage after a fall. Initial tests, including standard clotting profiles and screening for von Willebrand factor deficiency, were normal, prompting further investigation. A positive clot solubility test suggested Factor XIII deficiency. Due to limited facilities, FXIII assays were unavailable, so the patient was managed with fresh frozen plasma, cryoprecipitate, and tranexamic acid. Post-surgery bleeding revealed potential FXIII inhibitors, which were managed with additional plasma, cryoprecipitate, and oral prednisolone. The patient responded well and remained asymptomatic after discharge.