Autoimmune disease-associated lymphadenopathy with histological appearanceof T-zone dysplasia with hyperplastic follicles. A clinicopathological analysis of nine cases

Autoimmune disease-associated lymphadenopathy shows marked histopathologica l and clinical diversity. We describe the clinicopathological and immunohis tochemical findings of nine cases of autoimmune disease-associated lymphade nopathy, which posed a serious differential diagnostic problem regarding T- zone dysplasia with hyperplastic follicles. There were two males and seven females aged 25 to 65 years (median 37 years). The patients had multicentri c lymphadenopathy in association with clinical and laboratory findings sugg estive of an "autoimmune disease". Four patients were diagnosed to have sys temic lupus erythematosus (SLE), and the remaining five patients had antiph ospholipid antibody syndrome and Sjogren's syndrome (SS), rheumatoid arthri tis (RA), chronic thyroiditis, RA and SS, and SLE and SS, respectively. Non e of the nine patients developed malignant lymphomas during the follow-up p eriods from 44 to 225 months (median 103 months). The lesions were characte rized by paracortical hyperplasia with prominent vascular proliferation and many lymphoid follicles with germinal centers. The paracortical area usual ly contained numerous small T-lymphocytes without cytological atypia, accom panied by a variable number of plasma cells, B-immunoblasts, and histiocyte s. Polymerase chain reaction analysis demonstrated no clonal rearrangement of the T-cell receptor chain gene in four cases examined, although immunogl obulin heavy chain rearrangement was detected in only one case. These findi ngs suggest that autoimmune disease-associated lymphadenopathy, especially SLE, shares the histological features with T-zone dysplasia with hyperplast ic follicles. The nine cases presented here should be differentiated from T -zone lymphoma with follicles and angioimmunoblastic lymphoma with hyperpla stic germinal centers. To avoid overdiagnosis and overtreatment, we emphasi ze the need to turn attention to these clinical and laboratory findings as well as to the morphological features.