Arthropathies in factor VII deficiency: a case report

Factor VII deficiency (or hypoproconvertinemia) is a rare inherited bleeding disorder that can cause hemarthrosis similar to that seen in hemophilia. We report a case.

A 28-year-old woman experienced recurrent spontaneous hemarthrosis in both elbows and one knee starting at 2 years of age. She sought advice for an episode of bleeding in the left knee. The prothrombin level was decreased to 15% and the activated partial thromboplastin time was normal. Radiographs disclosed advanced joint destruction in the right shoulder, both elbows, the left knee, and both ankles. She described similar joint symptoms in two cousins. Plasma factor VII was less than 10%. A diagnosis of arthropathy due to an inherited bleeding disorder was given. Triamcinolone hexacetonide was injected into the joint; fresh frozen plasma was given concomitantly as an intravenous infusion.

Factor VII deficiency is an extremely uncommon bleeding disorder with an estimated prevalence of 1/300,000 to 1/500,000. Bleeding occurs only in homozygotes whose factor VII level is less than 20%. Hemarthrosis is less common than hemophilia, although the characteristics of joint destruction are similar in the two conditions.