Systemic lupus erythematosus-associated lymphoproliferative disorder: Report of a case and discussion in light of the literature

A case of autoimmune disease-associated lymphadenopathy (ADAL) with histological, immunophenotypic, Epstein-Barr virus (EBV) in situ hybridization, and genotypic analyses is presented. The patient had a well-documented history of systemic lupus erythematosus (SLE) and was found at autopsy to have massive lymphadenopathy, thymic enlargement, pulmonary nodules, and polyclonal serum dysproteinemia. Histological examination revealed a polymorphous lymphoid infiltrate containing many plasma cells, rare immunoblasts, and a pronounced arborizing vasculature. No foci of necrosis were found and there was no evidence of lymphocyte depletion. The plasma cells were immunophenotypically polyclonal and no EBV mRNA (EBER-1) or gene rearrangements were identified. The unusual gross features, which resembled a malignant lymphoproliferative process, as well as the unusual histological features make this case a notable addition to the spectrum of atypical lymphoproliferative disorders associated with an autoimmune disorder. We conclude that although reminiscent of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD), this case lacks: the diagnostic features of AILD, and is, perhaps, best classified as an autoimmune disease-associated lymphadenopathy (ADAL). (C) 1997 by W.B. Saunders Company.