Hereditary Combined Deficiency of Vitamin K-Dependent Clotting Factors Presenting as Postoperative Haemorrhage in A Syrian Adolescent: A Likely VKCFD Type 2 Phenotype.

Hereditary combined deficiency of vitamin K-dependent clotting factors (VKCFD) is a rare autosomal recessive disorder characterized by reduced activity of factors II, VII, IX, and X despite normal vitamin K levels. We report a 17-year-old Syrian female who presented with impaired wound healing and haemorrhagic discharge following cervical rib surgery. Laboratory evaluation revealed markedly prolonged prothrombin time and activated partial thromboplastin time both corrected on mixing studies, and selective deficiency of vitamin K-dependent factors. Vitamin K levels were normal, excluding acquired causes. The patient experienced recurrent bleeding episodes including haematuria and epistaxis, successfully managed with plasma and recombinant activated factor VII. The absence of skeletal abnormalities, mild bleeding phenotype, and delayed onset suggest a VKCFD type 2 presentation. This case highlights the importance of early recognition, differentiation from acquired coagulopathies, and targeted replacement therapy to prevent life threatening haemorrhage. LEARNING POINTS: Consider vitamin K-dependent clotting factors deficiency in unexplained prolonged prothrombin time/activated partial thromboplastin time with normal vitamin K and combined factor deficiencies.Early recognition prevents catastrophic bleeding; vitamin K and factor replacement are lifesaving.Internists play a pivotal role in differentiating inherited from acquired coagulopathies.