title: Fatal hemophagocytic syndrome after living-related liver transplantation: a report of two cases.
abstract: BACKGROUND
  Hemophagocytic syndrome (HPS) is a serious hematological disorder caused by activated T lymphocytes in immunologically compromised patients. There is no report of HPS in liver transplant recipients.
  
  
  METHODS
  Among 135 patients who underwent living-related liver transplantation between June 1990 and October 2000, HPS developed in two pediatric patients (1.5%) on the 15th and 134th postoperative day, respectively. The courses of these patients were evaluated.
  
  
  RESULTS
  The cause of HPS was unknown in patient 1 and suspected to be Epstein-Barr virus infection in patient 2. The course of patient 2 was also complicated by posttransplant lymphoproliferative disorder. Both patients had high fever, pancytopenia, coagulopathy, and marked elevation of serum-soluble interleukin 2 receptor, serum ferritin, and urine beta2-microglobulin levels. The diagnosis was established based on clinical findings, laboratory data, and bone marrow biopsy. Both patients died in an acute course despite intensive care.
  
  
  CONCLUSIONS
  HPS should be recognized as a severe hematological complication in liver transplant patients. Prompt institution of adequate treatment is necessary to prevent fatality.
authors.affiliation: Firs Department of Surgery, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Chisuwa
authors.forename: Y
authors.initials: Y
authors.lastname: Hashikura
authors.forename: Y
authors.initials: Y
authors.lastname: Nakazawa
authors.forename: T
authors.initials: T
authors.lastname: Kamijo
authors.forename: K
authors.initials: K
authors.lastname: Nakazawa
authors.forename: J
authors.initials: J
authors.lastname: Nakayama
authors.forename: T
authors.initials: T
authors.lastname: Oh-Ishi
authors.forename: T
authors.initials: T
authors.lastname: Ikegami
authors.forename: M
authors.initials: M
authors.lastname: Terada
authors.forename: S
authors.initials: S
authors.lastname: Kawasaki
mesh_terms: D003422:Critical Care; D020031:Epstein-Barr Virus Infections / Q000150:complications; D017809:Fatal Outcome; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications / Q000209:etiology* / Q000628:therapy / Q000821:virology; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000009:adverse effects*; D019520:Living Donors; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000209:etiology; D008297:Male