A 21-year-old male diagnosed with BRBN at 5 years of age with multiple vascular lesions with cutaneous, mucosal, hepatic and gastrointestinal involvement.
The patient was operated on several occasions for removal of lesions, hemangiomas and intestinal invagination.
Chronic iron deficiency anemia treated with oral iron and folic acid.
Since 2011, the inclusion of intravenous iron due to a worse control without hemoglobin (Hb) levels and an increase in transfusions [12-14 cold storage concentrates (HC)/year].
In November 2011, the patient came to the emergency department with asthenia, epigastric pain and dark stools. She was admitted for transfusion due to anemization [Hb:6.4 g/dl (13-17g/dl)] lower digestive bleeding.
After admission she came for analytical control and administration of intravenous iron and vitamin-K, requiring new transfusions for Hb of 5.4 g/dl in December 2011.
Given the increase in transfusions and worsening of anemia associated with gastrointestinal losses, treatment with subcutaneous octreotide is proposed without indication.
In January 2012, subcutaneous octreotide was started at a dose of 200 mcg/week, improving Hb levels after the first two doses (8.2 and 9 g/dl, respectively) without requiring new transfusions.
Given the good initial tolerance it was decided to change to monthly subcutaneous presentation of 20 mg in March 2012 with good Hb values (10.4 g/dl).
After two years, the patient maintained treatment with good tolerance; he only reported mild discomfort after administration and diarrhea, and stabilized Hb levels between 10-11 g/dl without the need for further transfusions until February 2014, when 1 CH/dl decreased.
1.
In August 2014, treatment was suspended due to a diagnosis of osteonecrosis of the hip with suspected "possible" adverse reaction (ADR) related to octreotide according to the Karch-Lasagna6 algorithm.
ADR is reported to the Autonomic Centre for Personality.
After discontinuation of treatment, she required 1 CH due to a decrease to 7.6 g/dl of Hb in October 2014 and 2 CH upon admission for autotransplantation of stem cells in the hip by Hb of 7 g/dl at discharge (8.8).
In December 2014, she came to the review with a further decrease in Hb (6.8 g/dl) and dark stools, restarting octreotide after assessing the benefit/risk, needing 6 CH from its suspension and treatment.
In January 2015 she came to the clinic after restarting octreotide with improvement of digestive loss, Hb of 12.4 g/dl and without need for new transfusions.
A therapeutic gastroscopy was performed to check for lesions in February and April 2015 with no incidents. Currently, treatment is maintained.
