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Interferon Alpha-2a Therapy for Disseminated Intravascular Coagulation in a Patient with Blue Rubber Bleb Nevus Syndrome

1997; SAGE Publishing; Volume: 48; Issue: 3 Linguagem: Inglês

10.1177/000331979704800312

1940-1574

Makoto Kunishige, Hiroyuki Azuma, Kenjiro Masuda, Toshio Shigekiyo, Yoshiharu Arii, Hisaomi Kawai, Shiro Saito, Makoto Kunishige,

Vascular Malformations Diagnosis and Treatment

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 coagula tion (DIC) manifesting as Kasabach-Merritt syndrome, with the potential for life-threat ening bleeding or thrombosis in the central nervous system. Since successful management of life-threatening hemangiomas with interferon alpha-2a (IFN α-2a) has been reported, the authors administered IFN α-2a with an improvement in hemostasis. These findings suggest that IFN α-2a therapy is beneficial for relieving the life-threatening consumptive coagulopathy associated with BRBNS.