EBV-elicited familial hemophagocytic lymphohistiocytosis.
10.3349/ymj.1997.38.4.245
- Author:
Hyun Sang CHO
1
;
Young Nyun PARK
;
Chuhl Joo LYU
;
Sae Myung PARK
;
Seung Hwan OH
;
Chang Hyun YANG
;
Woo Ick YANG
;
Kir Young KIM
Author Information
1. Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
- Publication Type:Case Reports
- Keywords:
Familial hemophagocytic lymphohistiocytosis;
EBV infection
- MeSH:
Case Report;
Child, Preschool;
Female;
Herpesviridae Infections/complications*;
Herpesvirus 4, Human*;
Histiocytosis, Non-Langerhans-Cell/virology*;
Histiocytosis, Non-Langerhans-Cell/genetics*;
Human;
Infant;
Male;
Tumor Virus Infections/complications*
- From:Yonsei Medical Journal
1997;38(4):245-248
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
