Disseminated intravascular coagulation (
DIC) is an extremely rare coagulopathy in the rare
factor XIII (FXIII) deficiency. Compensated
DIC occurs because of
injuries that lead to systemic coagulation activation that is amplified by impaired fibrinolysis. This challenge translates into the widespread deposition of
fibrin degradation products in the circulation. The aim of this study is to report three cases with severe FXIII deficiency who presented with
DIC and positive
D-dimer. Here we describe three patients affected by both FXIII deficiency and
DIC; two girls aged 17 and 45 days and a 3.5-year-old boy. All patients had a positive family history for FXIII deficiency. Umbilical cord
bleeding was the first presentation of FXIII deficiency in all of them, who presented also with
ecchymosis; two of them had delayed
wound bleeding.
DIC occurred simultaneously with intracranial haemorrhage in two patients, whereas the third experienced
DIC following extensive haematoma.
D-dimer measured in all patients ranged between 5 and 20 μg/ml, whereas
fibrinogen degradation product was between 4 and 8 μg/ml.