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.
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.
Kojima
Masaru
M
Department of Pathology and Clinical Laboratories, Gunma Cancer Center Hospital, Ohta, Japan. mkojima@gunma-cc.jp
Sugiura
Isamu
I
Itoh
Hideaki
H
Shimizu
Kazuhiko
K
Murayama
Kayoko
K
Motoori
Tadashi
T
Shimano
Shunichi
S
Masawa
Nobuhide
N
Nakamura
Shigeo
S
D000368:Aged; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology*; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology*; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications / Q000473:pathology; D007124:Immunoenzyme Techniques; D017403:In Situ Hybridization; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008198:Lymph Nodes / Q000473:pathology* / Q000821:virology; D008206:Lymphatic Diseases / Q000150:complications / Q000473:pathology; D008218:Lymphocytosis / Q000150:complications / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged
D001323:Autoantibodies; D007770:L-Lactate Dehydrogenase