Isolated Factor VII Deficiency Diagnosed after a Life-Threatening Brain Haemorrhage

Abstract

A 65-year-old man was admitted to another hospital with a life-threatening brain haemorrhage, and laboratory examinations on admission revealed prolonged prothrombin time with normal activated partial thromboplastin time. To establish the cause of his abnormal coagulation, he was referred to our clinic. Neither the patient nor his family had any previous history of bleeding symptoms. His liver function was within normal limits but coagulation tests showed increased plasma activities of factors II, VIII, IX, X, with reduced activities of factors V and VII. The activity of factor VII was less than 2% but no inhibitor of factor VII was detected in the plasma. We concluded that the patient had a rare congenital isolated factor VII deficiency although he had not shown earlier bleeding problems, presumably because of compensation for the factor VII deficiency by enhanced activities of components of the extrinsic coagulation pathway, factors II, VIII, IX and X.

rFVII for Pediatric Acute Intracranial Hemorrhage

“…In fact, a reported 65-year-old patient with congenital isolated factor VII deficiency and bleeding problems had not shown earlier bleeding problems, presumably because of compensation for the factor VII deficiency by enhanced activities of components of the extrinsic coagulation pathway, factors II, VIII, IX, and X. 7 Third, the use of rFVIIa in hemorrhagic shock in neonates and preterm infants are increasing. For instance, neonates with massive postsurgical hemorrhage after ileostomy, with severe pulmonary hemorrhage in the course of mechanical ventilation for meconium aspiration syndrome, with congenital heart disease.…”