Hemophagocytic lymphohistiocytosis in a pancreas-kidney transplant recipient: response to dexamethasone and cyclosporine

• Published in: Clinical nephrology Vol. 70; no. 1; p. 82
• Main Authors: González-Posada, J M, Hernández, D, Martin, A, Raya, J M, Pitti, S, Bonilla, A, Astigarraga, I, Alarcó, A
• Format: Journal Article
• Language: English
• Published: Germany 01.07.2008
• Subjects: Adult; Anti-Inflammatory Agents - therapeutic use; Antifungal Agents - therapeutic use; Candida glabrata; Candidiasis - complications; Candidiasis - diagnosis; Candidiasis - drug therapy; Cyclosporine - therapeutic use; Dexamethasone - therapeutic use; Humans; Kidney Transplantation - adverse effects; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - drug therapy; Lymphohistiocytosis, Hemophagocytic - etiology; Male; Pancreas Transplantation - adverse effects
• ISSN: 0301-0430
• DOI: 10.5414/cnp70082

We describe a severe case of hemophagocytic lymphohistiocytosis (HLH), secondary to a candida glabrata retroperitoneal abscess in a 41-year-old simultaneous pancreas-kidney transplantation (SPKT) recipient. Despite percutaneous abscess drainage and antifungal therapy, general status deteriorated with persistent fever, severe pancytopenia and liver dysfunction. Presence of hypertriglyceridemia, very high serum levels of ferritin and hemophagocytosis in a bone-marrow aspirate gave the diagnosis of HLH. Of note, change from tacrolimus to cyclosporine together with dexamethasone produced rapid response with status improvement. We concluded that HLH, a rare but often fatal condition characterized by excessive activation of lymphocytes and macrophages, is a diagnostic and therapeutic challenge in solid-organ transplanted patients and must be suspected in the presence of fever, blood cytopenia and liver dysfunction. Specific antiinfectious therapy together with cyclosporine and dexamethasone may be a therapeutic approach.