INTERFERON ALPHA-2A THERAPY FOR DISSEMINATED INTRAVASCULAR COAGULATION IN A PATIENT WITH BLUE RUBBER BLEB NEVUS SYNDROME - A CASE-REPORT

The authors present a sixteen-year-old girl with blue rubber bleb nevu s syndrome (BRBNS) associated with disseminated hemangiomas involving the skin, oral cavity, skeletal muscle, and cerebrum. Although she den ied neurologic symptoms, magnetic resonance imaging of the brain demon strated dilatated cerebral veins and the Chiari I malformation. Examin ation of hemostasis revealed disseminated intravascular coagulation (D IG) manifesting as Kasabach-Merritt syndrome, with the potential for l ife-threatening bleeding or thrombosis in the central nervous system. Since successful management of life-threatening hemangiomas with inter feron alpha-2a (IFN alpha-2a) has been reported, the authors administe red IFN alpha-2a with an improvement in hemostasis. These findings sug gest that IFN alpha-2a therapy is beneficial for relieving the life-th reatening consumptive coagulopathy associated with BRBNS.