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 nevus syndrome (BRBNS) associated with disseminated hemangiomas involving the skin, oral cavity, skeletal muscle, and cerebrum. Although she denied neurologic symptoms, magnetic resonance imaging of the brain demonstrated dilatated cerebral veins and the Chiari I malformation. Examination of hemostasis revealed disseminated intravascular coagulation (DIC) manifesting as Kasabach-Merritt syndrome, with the potential for life-threatening bleeding or thrombosis in the central nervous system. Since successful management of life-threatening hemangiomas with interferon alpha-2a (IFN alpha-2a) has been reported, the authors administered IFN alpha-2a with an improvement in hemostasis. These findings suggest that IFN alpha-2a therapy is beneficial for relieving the life-threatening consumptive coagulopathy associated with BRBNS.