RESUMEN
We report a case of a 19-year-old woman with acute liver failure, Coombs negative hemolytic anemia, and renal failure as initial manifestations of Wilson disease with recovery following medical treatment. The clinical picture and low serum transaminase and alkaline phosphatase levels gave us a clue to suspect Wilson disease and to initiate plasmapheresis and D-penicillamine soon after admission. The serum and urinary copper levels were elevated with low serum ceruloplasmin. We proceeded to ambulatory follow-up with medical treatment with D-penicillamine. A few months later, during the course of a laparoscopic cholecystectomy because of symptomatic gallstone disease, a liver biopsy sample was obtained that showed histological liver fibrosis and strongly elevated levels of liver tissue copper.
Asunto(s)
Quelantes/uso terapéutico , Degeneración Hepatolenticular/complicaciones , Fallo Hepático Agudo/etiología , Fallo Hepático Agudo/terapia , Penicilamina/uso terapéutico , Plasmaféresis , Adulto , Anemia Hemolítica/complicaciones , Biopsia , Ceruloplasmina/análisis , Cobre/sangre , Cobre/orina , Femenino , Estudios de Seguimiento , Degeneración Hepatolenticular/sangre , Degeneración Hepatolenticular/diagnóstico , Degeneración Hepatolenticular/patología , Degeneración Hepatolenticular/orina , Humanos , Hígado/patología , Fallo Hepático Agudo/tratamiento farmacológico , Factores de TiempoRESUMEN
Exponemos el caso de una paciente de 19 años que ingresó en nuestro centro con insuficiencia hepática aguda grave, anemia hemolítica Coomb´s negativa e insuficiencia renal. La clínica y los niveles séricos bajos de transaminasas y fosfatasa alcalina nos hicieron sospechar enfermedad de Wilson e iniciar plasmaféresis y D-penicilamina de forma precoz, a los 2 y 5 días respectivamente, con buena evolución posterior. Los niveles de cobre en sangre y orina resultaron ser elevados con niveles bajos de ceruloplasmina sérica. Se procedió a un seguimiento ambulatorio con tratamiento de mantenimiento con D-penicilamina, realizádose una biopsia hepática que demostró fibrosis en el examen anátomo-patológico y una concentración elevada de cobre en tejido hepático seco.En conclusión, en este caso de insuficiencia hepática aguda con hemolisis severa como forma de presentación de la enfermedad de Wilson, la instauración precoz de sesiones de plamaféresis y tratamiento con D-penicilamina fueron medidas eficaces con supervivencia sin necesidad de trasplante hepático ortotópico (AU)
No disponible
Asunto(s)
Adulto , Femenino , Humanos , Plasmaféresis , Factores de Tiempo , Penicilamina , Biopsia , Quelantes , Ceruloplasmina , Cobre , Anemia Hemolítica , Degeneración Hepatolenticular , Hígado , Estudios de Seguimiento , Fallo Hepático AgudoRESUMEN
Autoimmune hemolytic anemia is a rare complication of ulcerative colitis. A retrospective review of the cases of ulcerative colitis treated at our hospital between January 1984 and August 1993 showed that, among 210 patients, three presented autoimmune hemolytic anemia with a positive direct Coomb's test. They were two men and one woman suffering from a moderately active ulcerative colitis that affected the left colon. The hemolysis was diagnosed before the onset of colitis in two cases and after it in the other. In the only patient treated with sulphasalazine, this drug was stopped without improvement. All the patients were treated with steroids, with resolution of the anemia in one of them. Healing was achieved with splenectomy in the other two. Colectomy was not necessary in any case. After suppression of sulphasalazine and treatment with steroids, the next therapeutic option in patients with ulcerative colitis and autoimmune hemolytic anemia should be splenectomy, whereas colectomy should be only used with unresponsive patients, as well as with those affected by severe ulcerative colitis refractory to steroids. In patients presenting with ulcerative colitis and anemia, the possibility of autoimmune hemolytic anemia has to be considered since--in spite of being rare--it is responsive to proper treatment.