[Recurrent atypical hemolytic uremic syndrome after renal transplantation: treatment with eculizumab]. / Síndrome urémico hemolítico atípico recurrente post-trasplante renal: tratamiento con eculizumab.
Medicina (B Aires)
; 78(2): 119-122, 2018.
Article
en Es
| MEDLINE
| ID: mdl-29659362
ABSTRACT
Atypical hemolytic uremic syndrome (aHUS) is a rare entity. It is characterized by a thrombotic microangiopathy (nonimmune hemolytic anemia, thrombocytopenia, and acute renal failure), with a typical histopathology of thickening of capillary and arteriolar walls and an obstructive thrombosis of the vascular lumen. The syndrome is produced by a genetic or acquired deregulation of the alternative pathway of the complement system, with high rates of end stage renal disease, post-transplant recurrence, and high mortality. Mutations associated with factor H, factor B and complement C3 show the worst prognosis. Even though plasma therapy is occasionally useful, eculizumab is effective both for treatment and prevention of post-transplant recurrence. We describe here an adult case of congenital aHUS (C3 mutation) under preventive treatment with eculizumab after renal transplantation, with neither disease recurrence nor drug-related adverse events after a 36-months follow-up.
Palabras clave
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Trasplante de Riñón
/
Anticuerpos Monoclonales Humanizados
/
Síndrome Hemolítico Urémico Atípico
/
Inmunosupresores
Tipo de estudio:
Prognostic_studies
Límite:
Adolescent
/
Female
/
Humans
Idioma:
Es
Revista:
Medicina (B Aires)
Año:
2018
Tipo del documento:
Article