Yonsei Med J.  1997 Aug;38(4):245-248. 10.3349/ymj.1997.38.4.245.

EBV-elicited familial hemophagocytic lymphohistiocytosis

• Cho HS
• Park YN
• Lyu CJ
• Park SM
• Oh SH
• Yang CH
• Yang WI
• Kim KY

• KMID: 699958
• DOI: http://doi.org/10.3349/ymj.1997.38.4.245

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.

Keyword

Familial hemophagocytic lymphohistiocytosis; EBV infection

MeSH Terms

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*