EBV-elicited familial hemophagocytic lymphohistiocytosis.
Familial hemophagocytic lymphohistiocytosis (FHL) is a rapidly fatal illness, usually encountered in infancy, characterized by fever, hepatosplenomegaly, pancytopenia, and central nervous system involvement. Microscopic examination of tissue shows a non-malignant lymphohistiocytic infiltrate, with prominent erythrophagocytosis. FHL is an autosomal recessive hereditary disorder but may develop secondarily to other conditions such as immunosuppression, malignancies, fat overload and certain infections. We recently experienced a case of siblings developing FHL, which may be associated with EBV infection.
Cho
H S
HS
Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
Park
Y N
YN
Lyu
C J
CJ
Park
S M
SM
Oh
S H
SH
Yang
C H
CH
Yang
W I
WI
Kim
K Y
KY
D002675:Child, Preschool; D005260:Female; D006566:Herpesviridae Infections / Q000150:complications*; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000235:genetics* / Q000821:virology*; D006801:Humans; D007223:Infant; D008297:Male; D014412:Tumor Virus Infections / Q000150:complications*