SANGRADO DE DIFÍCIL CONTROL: DÉFICIT DEL FACTOR XIII

Abstract

INTRODUCTION:

Factor XIII or fibrin stabilizer deficiency (FXIIID), a rare alteration affecting the coagulation factors, is caused by the mutation of different genes, most frequently the F13A1 which codifies the catalytic subunit A. The XIII factor intervenes in the final phase of the blood clotting cascade, in the common one. The FXIIID is an autosomal recessive inherited disease that can be shown in a congenial or acquired way. It’s most frequent symptom is the difficult-to-treat bleeding. Even though it is a rare condition, it is potentially serious, since its more feared complication is the intracranial bleeding.

METHODS AND MATERIAL:

Male, 89, admitted because of oral bleeding approximately two moths after a dental extraction. The bleeding was tried to be stopped through compression and cauterization and through surgery, resulting ineffective.

RESULTS:

A consultation request was made to Hematology, who made an assessment and a blood clotting study. The coagulation times were normal. Nonetheless, a mild FXIIID was revealed, so the patient was treated with the administration of recombinant XIII factor, getting better.

CONCLUSION:

The FXIIID is a rare, little-known and potentially serious condition if remaining untreated. Therefore, before a patient with an unwavering or difficult-to-treat bleeding, we should think of it. Once it is diagnosed, the administration of recombinant XIII factor mustn’t be delayed, specially if symptomatic.