title: Rapidly Fatal Hemophagocytic Lymphohistiocytosis Developing Within Six Days Following Deceased-Donor Renal Transplantation: Case Report.
abstract: 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.
authors.affiliation: Division of Nephrology, Department of Medicine, Sydney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address: kidneys@comcast.net.
authors.forename: E J
authors.initials: EJ
authors.lastname: Filippone
authors.affiliation: Division of Nephrology, Department of Medicine, Sydney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: P
authors.initials: P
authors.lastname: Singh
authors.affiliation: Department of Surgery, Sydney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: A M
authors.initials: AM
authors.lastname: Frank
authors.affiliation: Department of Medicine, Albert Einstein Medical Center, Philadelphia, Pennsylvania.
authors.forename: A
authors.initials: A
authors.lastname: Gupta
authors.affiliation: Department of Pathology, Sydney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: J L
authors.initials: JL
authors.lastname: Farber
mesh_terms: D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017809:Fatal Outcome; D006801:Humans; D016030:Kidney Transplantation / Q000009:adverse effects*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D008297:Male; D011183:Postoperative Complications / Q000821:virology*