A Case of Congenital Vitamin K Dependent Coaguation Factor Defeciency / 대한소아혈액종양학회지

ABSTRACT

Congenital combined deficiency of vitamin K dependent coagulation factors is a rare coagulation disorder. We experienced a 20-month old boy who was found to have a congenital vitamin K dependent coagulation factor defeciency. He presented with continuous bleeding on lacerated hard palate and had a history of numerous hemorrhagic episodes with multiple bruises after birth. Laboratory finding showed prolonged prothrombin time and partial thromboplastin time. Blood coagulation work-up showed marked decreased activities of the coagulation factors II, VII, IX, X and the natural anticoagulants proteins C and S. Assay of coagulation factors in the parents and sibling were with the normal range. There's no evidence of malabsorption, liver disease or ingestion of a coumarin compound. Response to intravenous administration of vitamin K1 was not significant but transfusion of fresh frozen plasma corrected prothrombin time and partial thromboplastin time. We reported a case of congenital combined deficiency of vitamin K dependent coagulation factors.