Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport

We report a 28-year-old male with a hereditary nephritis (Alport Syndrome) on hemodialysis for 5 years, who received a kidney graft from a deceased donor. Cyclosporine (CsA), mycophenolate mofetil (MMF) and steroids were prescribed. In the postoperative period the patient had thrombo...

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Autores principales: Vega,Jorge, Rodríguez,María De Los Ángeles, Goecke,Helmuth, Santamarina,Mario
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2013
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013000400014
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spelling oai:scielo:S0034-988720130004000142013-09-11Síndrome hemofagocítico en un trasplantado renal con síndrome de AlportVega,JorgeRodríguez,María De Los ÁngelesGoecke,HelmuthSantamarina,Mario Lymphohistiocytosis, hemophagocytic Mycophenolate mofetil Nephritis, hereditary Organ transplantation We report a 28-year-old male with a hereditary nephritis (Alport Syndrome) on hemodialysis for 5 years, who received a kidney graft from a deceased donor. Cyclosporine (CsA), mycophenolate mofetil (MMF) and steroids were prescribed. In the postoperative period the patient had thrombophlebitis and diarrhea. A CT sean showed splenomegaly, ascites, bilateral pleural effusion and bowel edema. Laboratory showed hypoalbuminemia, increased C reactive protein (CRP) and panhypogammaglobulinemia. At day 32 after transplantation, an acute rejection (Banff II b) was diagnosed and treated with methylprednisolone, replacing CsA by tacrolimus. The acute rejection was controlled but six days later, high fever, pancytopenia and hyperferritinemia appeared. A bone marrow smear showed numerous histiocytes and hemophagocytosis. Hemophagocytic syndrome was diagnosed. MMF and tacrolimus were withdrawn and CsA was reinstituted. Fever fell quickly, CPR normalized at 24 hours and white blood cell count at 72 hours. Days later, the concentrations of albumin, immunoglobulins and hematological parameters normalized. The patient was discharged on day 57 after admission in good condition.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.141 n.4 20132013-04-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013000400014es10.4067/S0034-98872013000400014
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Lymphohistiocytosis, hemophagocytic
Mycophenolate mofetil
Nephritis, hereditary
Organ transplantation
spellingShingle Lymphohistiocytosis, hemophagocytic
Mycophenolate mofetil
Nephritis, hereditary
Organ transplantation
Vega,Jorge
Rodríguez,María De Los Ángeles
Goecke,Helmuth
Santamarina,Mario
Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport
description We report a 28-year-old male with a hereditary nephritis (Alport Syndrome) on hemodialysis for 5 years, who received a kidney graft from a deceased donor. Cyclosporine (CsA), mycophenolate mofetil (MMF) and steroids were prescribed. In the postoperative period the patient had thrombophlebitis and diarrhea. A CT sean showed splenomegaly, ascites, bilateral pleural effusion and bowel edema. Laboratory showed hypoalbuminemia, increased C reactive protein (CRP) and panhypogammaglobulinemia. At day 32 after transplantation, an acute rejection (Banff II b) was diagnosed and treated with methylprednisolone, replacing CsA by tacrolimus. The acute rejection was controlled but six days later, high fever, pancytopenia and hyperferritinemia appeared. A bone marrow smear showed numerous histiocytes and hemophagocytosis. Hemophagocytic syndrome was diagnosed. MMF and tacrolimus were withdrawn and CsA was reinstituted. Fever fell quickly, CPR normalized at 24 hours and white blood cell count at 72 hours. Days later, the concentrations of albumin, immunoglobulins and hematological parameters normalized. The patient was discharged on day 57 after admission in good condition.
author Vega,Jorge
Rodríguez,María De Los Ángeles
Goecke,Helmuth
Santamarina,Mario
author_facet Vega,Jorge
Rodríguez,María De Los Ángeles
Goecke,Helmuth
Santamarina,Mario
author_sort Vega,Jorge
title Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport
title_short Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport
title_full Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport
title_fullStr Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport
title_full_unstemmed Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport
title_sort síndrome hemofagocítico en un trasplantado renal con síndrome de alport
publisher Sociedad Médica de Santiago
publishDate 2013
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013000400014
work_keys_str_mv AT vegajorge sindromehemofagociticoenuntrasplantadorenalconsindromedealport
AT rodriguezmariadelosangeles sindromehemofagociticoenuntrasplantadorenalconsindromedealport
AT goeckehelmuth sindromehemofagociticoenuntrasplantadorenalconsindromedealport
AT santamarinamario sindromehemofagociticoenuntrasplantadorenalconsindromedealport
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