Hemophagocytic syndrome after kidney transplant in a patient with hereditary nephritis. Report of one case

作者：Vega Jorge^*; de los Angeles Rodriguez Maria; Goecke Helmuth; Santamarina Mario

来源：Revista Medica de Chile, 2013, 141(4): 519-524.

• 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 scan showed splenomegaly, ascites, bilateral pleural effusion and bowel edema. Laboratory showed hypoalbuminemia, increased C reactive protein (CRP) and panhypogam-maglobulinemia. 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 MMF and tacrolimus were withdrawn and CsA was reinstituted.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.

• 出版日期2013-4