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Blood, Vol. 96 No. 2 (July 15), 2000:
pp. 443-451
Fulminant EBV+ T-cell lymphoproliferative disorder
following acute/chronic EBV infection: a distinct clinicopathologic
syndrome
Leticia Quintanilla-Martinez,
Shimareet Kumar,
Falko Fend,
Edgardo Reyes,
Julie Teruya-Feldstein,
Douglas W. Kingma,
Lynn Sorbara,
Mark Raffeld,
Stephen E. Straus, and
Elaine S. Jaffe
From the Hematopathology Section, Laboratory
of Pathology, National Cancer Institute, and Laboratory of Clinical
Investigation, National Institute of Allergy and Infectious Diseases,
National Institutes of Health, Bethesda, MD; Department of Pathology,
Instituto Nacional de la Nutrición, México City,
México; Institute for Pathology, GSF-Research Center for
Environment and Health, Neuherberg, Germany.
This study describes the clinicopathologic features of 5 patients who developed a fulminant Epstein-Barr virus (EBV)-positive clonal T-cell lymphoproliferative disorder (LPD) after acute EBV infection. One additional patient developed a similar disorder in the
setting of long-standing chronic active EBV infection. Detailed
immunophenotyping, in situ hybridization for EBV early RNA-1 (EBER1)
and polymerase chain reaction (PCR) analyses for immunoglobulin (Ig)
heavy chain and T-cell receptor (TCR)-gamma gene rearrangements were
performed on paraffin-embedded tissue from all patients. In addition,
EBV strain typing and detection of the characteristic 30-bp deletion of
the latent membrane protein-1 (LMP-1) gene were performed by
PCR. Controls included 8 cases of uncomplicated infectious
mononucleosis (IM). Patients included 4 males and 2 females with a
median age of 18 years (2-37 years). Three patients were Mexican, 2 were white, and 1 was of Asian descent. All presented with
fever, hepatosplenomegaly, and pancytopenia; 5 were previously healthy,
but had a clinical history of a recent viral-like upper respiratory
illness (1 week to 2 months), and 1 patient had documented chronic
active EBV infection for 7 years. Serologic data for EBV were
incomplete but titers were either negative or only modestly elevated
in 3 cases. In 1 case serology was consistent with severe chronic
active EBV infection. In the remaining 2 cases serologic studies were
not performed. All patients died within 7 days to 8 months of
presentation with T-cell LPD. On histologic examination, the liver and
spleen showed prominent sinusoidal and portal lymphoid infiltrates
of CD3+,  F1+,
EBER1+ T cells lacking significant cytologic atypia. Two
cases were CD4+, 2 cases were CD8+, and 2 cases had admixed CD4+ and CD8+ cells
without clear subset predominance. All were TIA-1+,
CD56 . Only rare B cells were noted. Marked
erythrophagocytosis was present. Molecular analysis revealed identical
T-cell clones in 2 or more sites (liver, spleen, lymph node) in 5 cases. All patients carried type A EBV; 4 cases had wild-type EBV-LMP,
and 2 showed the 30-bp deletion. This fulminant T-cell LPD
after acute/chronic EBV infection is characterized by
hepatosplenomegaly, often without significant lymphadenopathy, fever,
liver failure, pancytopenia, and erythrophagocytosis indicative of a
hemophagocytic syndrome. EBV serology may be misleading, with lack of
elevated titers. The presence of an EBER1+ T-cell
infiltrate with scant B cells should alert one to this diagnosis.
Although cytologic atypia is minimal, studies for T-cell clonality
confirm the diagnosis.
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