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Histological varieties of Epstein-Barr virus-related lymph node lesion resembling autoimmune disease-like clinicopathological findings in middle-aged and elderly patients: a study of six cases.

Abstract
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.
AuthorsMasaru Kojima, Isamu Sugiura, Hideaki Itoh, Kazuhiko Shimizu, Kayoko Murayama, Tadashi Motoori, Shunichi Shimano, Nobuhide Masawa, Shigeo Nakamura
JournalPathology, research and practice (Pathol Res Pract) Vol. 202 Issue 8 Pg. 609-15 ( 2006) ISSN: 0344-0338 [Print] Germany
PMID16824704 (Publication Type: Case Reports, Journal Article, Multicenter Study)
Chemical References
  • Autoantibodies
  • L-Lactate Dehydrogenase
Topics
  • Aged
  • Autoantibodies (blood)
  • Autoimmune Diseases (immunology, pathology)
  • Diagnosis, Differential
  • Epstein-Barr Virus Infections (complications, pathology)
  • Female
  • Herpesvirus 4, Human (genetics, isolation & purification)
  • Humans
  • Hypergammaglobulinemia (complications, pathology)
  • Immunoenzyme Techniques
  • In Situ Hybridization
  • L-Lactate Dehydrogenase (blood)
  • Lymph Nodes (pathology, virology)
  • Lymphatic Diseases (complications, pathology)
  • Lymphocytosis (complications, pathology)
  • Lymphoproliferative Disorders (complications, pathology, virology)
  • Male
  • Middle Aged

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