History may include:
(1) chronic EBV infection
(2) acute EBV infection
(3) recent viral-like upper respiratory tract infection
Clinical features:
(1) acute onset of fever and malaise
(2) hepatosplenomegaly
(3) variable lymphadenopathy (may be absent)
(4) hepatic failure with jaundice
(5) fulminant clinical course
Laboratory features:
(1) pancytopenia
(2) abnormal liver function tests
(3) EBV serologic results are variable, with low titers possible (false negatives may delay diagnosis)
Pathologic features:
(1) lymphoproliferative disorder with prominent sinusoidal and portal lymphoid infiltrates in the liver and spleen
(2) lymphocytes are clonal T cells (CD3 positive, CD56 negative) that lack significant cytologic atypia
(3) erythrophagocytosis consistent with the hemophagocytic syndrome
(4) evidence of active EBV infection, with either in situ hybridization for EBV early RNA-1 (EBER1) or PCR
