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.
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.
Re
A
A
Division of Hematology, Spedali Civili di Brescia, Brescia, Italy.
Facchetti
F
F
Borlenghi
E
E
Cattaneo
C
C
Capucci
M A
MA
Ungari
M
M
Barozzi
P
P
Vallerini
D
D
Potenza
L
L
Torelli
G
G
Rossi
G
G
Luppi
M
M
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
D000305:Adrenal Cortex Hormones