Original contributions: Case reportsKidneyRapidly Fatal Hemophagocytic Lymphohistiocytosis Developing Within Six Days Following Deceased-Donor Renal Transplantation: Case Report

- Fatal hemophagocytic lymphohistiocytosis (HLH) may develop after kidney transplantation.
- HLH is suggested by exaggerated pancytopenia and/or hyperinflammation after transplantation.
- Prompt recognition and treatment of this syndrome may be life saving.

Hemophagocytic lymphohistiocytosis (HLH) is an often fatal hyperinflammatory syndrome that may complicate malignancy, infection, rheumatic disease, or immunosuppression. HLH after kidney transplantation is most often triggered by infection, usually Herpes viruses such as cytomegalovirus and Epstein-Barr virus (EBV). It usually occurs early after transplantation. We present a case of HLH triggered by reactivation of EBV that pursued a rapidly fatal course within 6 days of receiving a deceased-donor kidney transplant. This case serves to remind transplant clinicians to consider HLH when cytopenias and hyperinflammation are atypical for the usual post-transplantation course. We discuss pitfalls in diagnosis and suggestions for treatment in this setting.