TTS Lee, GKS Lam, CH Li, CK Li

Abstract

Haemostasis involves a complex cascade which has various pathways to allow an optimal equilibrium between thrombosis and bleeding. Factor XIII (FXIII) contributes at the end of the coagulation cascade and binds with the unstable fibrin clot to form a stable clot. We hereby report a case of Chinese girl presented with prolonged umbilical cord bleeding with normal coagulation study in the newborn period. She developed intracranial haemorrhage at age of 4 months and was subsequently diagnosed to have FXIII deficiency. This case demonstrates that in any patient presenting with bleeding tendency and seemingly normal blood counts and clotting profile, the diagnosis of FXIII deficiency should be considered.