Congenital Factor XII Deficiency and Lupus Anticoagulant

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An unusual case of congenital factor XII deficiency with systemic lupus erythematosus (SLE) showing positive lupus anticoagulant (LA) has been treated at the Akita University Hospital since April 1991. The 29 year-old woman displayed a thrombophlebitis on the left leg probably due to LA, as she had been diagnosed as having SLE at age 14 based on positive ANA and anti-DNA, and clinical symptoms including skin changes high fever, and etc. Although LA with SLE is not unusual, the complication of congenital coagulation factor deficiency is rare. Factor XII deficiency was first discovered by Ratnoff & Colopy in 1955 (1), and this disease is thought to have a tendency to cause thrombosis. LA in SLE has been reported since 1972, and is also known to have hemostatic changes. To my knowledge, no patients with both diseases have been reported.