Blue rubber bleb nevus syndrome associated with consumption coagulopathy: treatment with interferon.
The blue rubber bleb nevus syndrome (BRBNS) is a rare vascular malformation syndrome with cutaneous and visceral lesions frequently associated with serious, even fatal bleeding. No systemic therapy is currently available. We report here a case with disseminated skin and gastrointestinal venous malformations and findings of disseminated intravascular coagulation that was treated with interferon beta. The disseminated intravascular coagulation manifestations were cleared with the treatment. The regression of the coagulopathy in our patient led us to think that the use of interferon beta in BRBNS might be beneficial.
Apak
H
H
Pediatric Hematology-Oncology, Cerrahpasa Medical School, Istanbul Medical School, Istanbul University, Istanbul, Turkey. apakh@istanbul.edu.tr
Celkan
T
T
Ozkan
A
A
Yildiz
I
I
Aydemir
E H
EH
Ozdil
S
S
Kuruoglu
S
S
D002675:Child, Preschool; D003937:Diagnosis, Differential; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D017809:Fatal Outcome; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D008297:Male; D018329:Nevus, Blue / Q000150:complications*; D011994:Recombinant Proteins; D012878:Skin Neoplasms / Q000150:complications*; D013577:Syndrome
D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins