Acquired Coagulation Factor XIII Deficiency With Spontaneous Splenic Rupture: A Case Report

TL;DR

This case report highlights a rare bleeding disorder where a man with normal blood tests experienced a life-threatening spleen rupture due to a coagulation factor deficiency.

Contribution

The paper emphasizes the need to test for factor XIII deficiency in unexplained bleeding cases despite normal coagulation tests.

Findings

FXIII deficiency can cause severe bleeding despite normal PT/APTT and platelet counts.

Spontaneous splenic rupture occurred in a patient with undiagnosed FXIII deficiency.

Testing for FXIII activity is crucial in cases of unexplained hematomas or before surgery.

Abstract

Coagulation factor XIII deficiency (FXIIID) is a rare hemorrhagic disease, mainly manifested as skin ecchymosis and hematoma. Because of its atypical clinical manifestations and normal results of routine coagulation test, platelet count and function, it has brought great challenges to the diagnosis. This case report introduces the diagnosis and treatment of an elderly male patient with FXIIID in detail, and aims to emphasize the necessity of detecting FXIII activity when spontaneous bleeding occurs in patients with normal routine coagulation function and platelet count. At the same time, it emphasizes the importance of etiology finding and individualized treatment and management for patients with FXIIID.

In FXIII‐deficiency, normal PT/APTT can mask life‐threatening bleeding; add FXIII assay in unexplained hematomas and pre‐operatively to avert catastrophic events like spontaneous splenic rupture.

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