title: Haemophagocytic syndrome in a liver transplant patient during treatment with Telaprevir.
abstract: Haemophagocytic syndrome (HS) is a rare disease that is often fatal despite treatment. HS is characterized by fevers, lymphadenopathy, hepatosplenomegaly, cytopenias and hyperferritinaemia due to deregulated activation and proliferation of macrophages, leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes, and their hematopoietic precursors throughout the reticuloendothelial system. Mycobacterium tuberculosis-associated HS is a rare and underdiagnosed association with only 39 cases reported. We describe a case of HS associated with disseminated Mycobacterium tuberculosis in the setting of post-liver transplantation anti-hepatitis C therapy with pegylated interferon (pegIFN), ribavirin (RBV) and telaprevir (TVR). Despite the delay in the etiologic diagnosis, the patient was treated properly with corticosteroids, cyclosporine and tuberculostatic agents. It is unknown whether telaprevir, a drug that only recently has been started off-label in liver transplant recipients, may have contributed to the development of the HS. Unfortunately, as in many reported cases of HS, the outcome was unfavourable resulting in the death of the patient.
authors.affiliation: Liver Transplantation and Hepatology Unit, La Fe Hospital, Valencia, Spain.
authors.forename: Beatriz
authors.initials: B
authors.lastname: Rodríguez-Medina
authors.forename: Marino
authors.initials: M
authors.lastname: Blanes
authors.forename: Carmen
authors.initials: C
authors.lastname: Vinaixa
authors.forename: Victoria
authors.initials: V
authors.lastname: Aguilera
authors.forename: Angel
authors.initials: A
authors.lastname: Rubín
authors.forename: Martín
authors.initials: M
authors.lastname: Prieto
authors.forename: Marina
authors.initials: M
authors.lastname: Berenguer
mesh_terms: D000995:Antitubercular Agents / Q000627:therapeutic use; D000998:Antiviral Agents / Q000009:adverse effects*; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D016174:Hepacivirus / Q000187:drug effects / Q000472:pathogenicity; D006526:Hepatitis C / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects; D008103:Liver Cirrhosis / Q000601:surgery* / Q000821:virology; D016031:Liver Transplantation / Q000009:adverse effects*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000139:chemically induced / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000382:microbiology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009169:Mycobacterium tuberculosis / Q000187:drug effects / Q000276:immunology / Q000302:isolation & purification*; D009842:Oligopeptides / Q000009:adverse effects*; D012307:Risk Factors; D013997:Time Factors; D014376:Tuberculosis / Q000188:drug therapy / Q000276:immunology / Q000382:microbiology*; D014775:Virus Activation / Q000187:drug effects
chemical_list: D000995:Antitubercular Agents; D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D009842:Oligopeptides; C486464:telaprevir