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Association of lymphomatoid granulomatosis with Epstein-Barr viral
infection of B lymphocytes and response to interferon-alpha 2b
WH Wilson, DW Kingma, M Raffeld, RE Wittes and ES Jaffe
Medicine Branch, National Cancer Institute, Bethesda, MD 20892, USA.
Lymphomatoid granulomatosis (LyG) is an angiodestructive
lymphoproliferative disorder (LPD) often involving the lungs. Its etiology
is uncertain, but a number of previous studies had suggested it is a T-cell
LPD associated with Epstein-Barr virus (EBV). Because of the similarity
between LYG and nasal angiocentric lymphoma, the term angiocentric
immunoproliferative lesion was proposed for both entities. Optimal therapy
is unknown, but chemotherapy is often used. We studied four patients with
LYG over a 5-year period. Biopsy samples were analyzed by
immunohistochemistry, EBV in situ hybridization, and for Ig heavy-chain
(IgH) gene rearrangements, Clinically, we assessed EBV serology, lymphocyte
subsets, and the efficacy of interferon-alpha2b (IFN-alpha2b), All biopsy
samples showed an exuberant T-cell infiltrate with scattered atypical large
B cells. Double labeling showed EBV in the B cells but not T cells. Clonal
IgH gene rearrangements were detected in 2 of 3 patients studied, 1 of whom
had three distinct clones, and light-chain restriction showed two clones in
an additional patient. All patients had positive EBV serologies. and
markedly abnormal lymphocyte subsets. With IFN, 3 patients are alive and
disease free at 36, 43, and 60 months; 1 patient achieved a partial
response for 16 months but discontinued therapy and died with lymphoma.
These results indicate that LYG is a T-cell-rich EBV-associated B-cell LPD
in which the infiltrating T cells are numerous but reactive. IgH gene
rearrangements may be polyclonal, monoclonal, or oligoclonal. Its
association with immune defects suggests it is related to posttransplant
LPD. However, LYG and nasal angiocentric lymphoma are distinct entities and
should no longer be included together under the term angiocentric
immunoproliferative lesion. IFN is effective therapy and should be studied
further.
Volume 87,
Issue 11,
pp. 4531-4537,
06/01/1996
Copyright © 1996 by The American Society of Hematology
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