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

C C Kariyawasan *

Department of Haematology, Sri Jayewardenepura General Hospital, Thalapathpitiya, Nugegoda, Sri Lanka.

P H D Athukorala

Department of Haematology, Sri Jayewardenepura General Hospital, Thalapathpitiya, Nugegoda, Sri Lanka.

N L Priyantha

Pathology Laboratory, Sri Jayewardenepura General Hospital, Thalapathpitiya, Nugegoda, Sri Lanka.

B L T Balasuriya

Department of Haematology, Sri Jayewardenepura General Hospital, Thalapathpitiya, Nugegoda, Sri Lanka.

*Author to whom correspondence should be addressed.


Abstract

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.

Keywords: Factor XIII deficiency, acquired factor XIII deficiency, bleeding disorders


How to Cite

Kariyawasan, C C, P H D Athukorala, N L Priyantha, and B L T Balasuriya. 2024. “Diagnosis and Management of an Acquired Factor XIII Deficiency in a Limited Resource Setting: A Case Report”. Asian Hematology Research Journal 7 (4):224-28. https://doi.org/10.9734/ahrj/2024/v7i4185.

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