Successful management of anemia with sirolimus in blue rubber bleb nevus syndrome: case report and update.
Rev Esp Enferm Dig
; 111(8): 643-647, 2019 Aug.
Article
en En
| MEDLINE
| ID: mdl-31232079
Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic skin hemangiomas and vascular malformations, mostly in the gastrointestinal (GI) tract. The GI lesions are mainly located in the stomach and small intestine, usually more than a hundred, leading to gastrointestinal bleeding and severe chronic anemia. Parenteral iron infusions and scheduled transfusions are frequently necessary. We describe the case of a 21-year-old male with anemia secondary to BRBNS, who becomes unresponsive to octreotide and shows an excellent response to sirolimus (SRL), dismissing the intravenous iron supplementations and being free of transfusions. During the treatment, the patient presents avascular hip necrosis, which is adequately treated with an injection of stem cells with complete recovery, and without the suspension of SRL. Two years later, adequate response persists with no other relevant side effects.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias Cutáneas
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Nevo Azul
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Sirolimus
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Neoplasias Gastrointestinales
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Anemia
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Antibióticos Antineoplásicos
Límite:
Adult
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Humans
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Male
Idioma:
En
Revista:
Rev Esp Enferm Dig
Asunto de la revista:
GASTROENTEROLOGIA
Año:
2019
Tipo del documento:
Article
Pais de publicación:
España