Six cases were studied to further clarify clinicopathological findings of Epstein-Barr virus (EBV)-related lymph node lesions showing
autoimmune disease-like clinicopathological findings (EBVAID) in middle-aged and elderly patients. The patients, four males and two females, ranged in age from 53 to 74 years, with a median age of 62 years. Clinically, they were characterized by systemic
lymphadenopathy, "B"symptoms, polyclonal
hypergammaglobulinemia, elevated serum
lactate dehydrogenase and a transient presence of various
autoantibodies, as well as an infrequent presence of atypical
lymphocytosis in peripheral blood. Two cases were associated with
idiopathic thrombocytopenic purpura. The
clinical course was self-limiting. Histologically, three patterns could be delineated: pattern A, follicular
hyperplasia with pronounced arborizing vasculature in the expanded paracortex (n=3); pattern B, follicular
hyperplasia with pronounced interfollicular B-immunoblastic/plasma cell proliferation (n=2); and pattern C, paracortical
hyperplasia containing numerous large transformed lymphocytes (n=1). In situ hybridization demonstrated a varying number of EBV-infected lymphocytes in the germinal center and in the interfollicular area. Polymerase chain reaction analysis demonstrated that neither clonal rearrangement of
T-cell receptor gamma-chain nor
immunoglobulin heavy-chain rearrangement was detected in the three cases examined. Although EBVAID appears to be rare in middle-aged and older adults, EBVAID exhibits histological variations and should be added to the differential diagnosis of various atypical or malignant
lymphoproliferative disorders, in particular
autoimmune-disease-associated
lymphadenopathy and angioimmunoblastic
T-cell lymphoma with a hyperplastic germinal center in middle-aged and elderly patients.