Quintanella-Martinez et al described the clinical features shown by patients with a T-cell lymphoproliferative disorder associated with an infection caused by Epstein-Barr Virus (EBV). The authors are from the National Cancer Institute, National Institute of Allergy and Infectious Diseases, Instituto Nacional de la Nutricion in Mexico City and GSF-Research Center for Environment and Health in Neuherberg, Germany.


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


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