title: Fatal hemophagocytic syndrome related to active human herpesvirus-8/Kaposi sarcoma-associated herpesvirus infection in human immunodeficiency virus-negative, non-transplant patients without related malignancies.
abstract: Hemophagocytic syndrome (HS) may occur as a consequence of herpes viral infections. Human herpesvirus 8 (HHV-8)/Kaposi sarcoma-associated herpesvirus has so far been recognized as a trigger of HS only in immunosuppressed subjects or in patients with Kaposi sarcoma and/or HHV-8-related lymphoproliferative diseases. We report two Italian human immunodeficiency virus (HIV)-negative elderly men who developed an HS with a rapidly fatal course, following treatment with corticosteroids for autoimmune hemolytic anemia. An overwhelming active infection with HHV-8 was unequivocally documented by molecular and immunohistochemical methods, in the absence of HHV-8-related tumors. The occurrence of HHV-8-associated HS, although rare, may be considered, even out of the HIV or the transplantation settings, at least in areas endemic for HHV-8 infection.
authors.affiliation: Division of Hematology, Spedali Civili di Brescia, Brescia, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Re
authors.forename: F
authors.initials: F
authors.lastname: Facchetti
authors.forename: E
authors.initials: E
authors.lastname: Borlenghi
authors.forename: C
authors.initials: C
authors.lastname: Cattaneo
authors.forename: M A
authors.initials: MA
authors.lastname: Capucci
authors.forename: M
authors.initials: M
authors.lastname: Ungari
authors.forename: P
authors.initials: P
authors.lastname: Barozzi
authors.forename: D
authors.initials: D
authors.lastname: Vallerini
authors.forename: L
authors.initials: L
authors.lastname: Potenza
authors.forename: G
authors.initials: G
authors.lastname: Torelli
authors.forename: G
authors.initials: G
authors.lastname: Rossi
authors.forename: M
authors.initials: M
authors.lastname: Luppi
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D017809:Fatal Outcome; D005260:Female; D006566:Herpesviridae Infections / Q000150:complications / Q000175:diagnosis*; D019288:Herpesvirus 8, Human / Q000302:isolation & purification*; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000601:surgery; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000188:drug therapy / Q000821:virology*; D008297:Male; D008875:Middle Aged
chemical_list: D000305:Adrenal Cortex Hormones