Blue Rubber Bleb Nevus Syndrome: Promising Response To Sirol
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Case Report

A 6-year-old female was admitted to our hospital with multiple skin lesions and anemia. The lesions appeared at the age of six months and increased in size and number. Oral steroids were given at a dose of 1 mg/kg/day for few months and sclerotherapy was performed for her largest and painful lesion in the left cervical region without any success. At the age of five she had GI bleeding and colonoscopy showed multiple vascular ectasias and venous malformations. She had been transfused many times in previous year. On admission, she was pale with widespread small variable-sized bluish papules and large vascular masses on the face, mouth, trunk, arms, legs and fingers were noted (Fig. 1a). She had pain in her left knee where the largest vascular lesion was located. Blood tests revealed hemoglobin 6.1g/dL, white blood cell count 8100/mm³, platelets count 77,000/mm³, unconjugated bilirubin 1.6 mg/dL, fibrinogen 104 mg/dL, and D-dimer >40mg/dL. Acanthocytes and schistocytes were noted on perepheral blood smear. The patient was diagnosed as Blue rubber bleb nevus syndrome with typical clinical findings with microangiopathic hemolytic anemia and active consumptive coagulopathy. She also had mild GI bleeding shown with microscopic blood from rectum and fresh frozen plasma and packed red cell transfusions were given.....

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