Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport

• Jorge Vega ^[1] ; María De Los Ángeles Rodríguez ^[1] ; Helmuth Goecke ^[1] ; Mario Santamarina ^[1]
  1. [1] Hospital Naval Almirante Nef

     Hospital Naval Almirante Nef

     Viña del Mar, Chile

     
• Localización: Revista Médica de Chile, ISSN-e 0034-9887, Vol. 141, Nº. 4, 2013, págs. 519-524
• Idioma: español
• Títulos paralelos:
  • Hemophagocytic syndrome after kidney transplant in a patient with hereditary nephritis. Report of one case
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• Resumen

  • 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 MMF and tacrolimus were withdrawn and CsA was reinstituted.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.