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 hi stological, immunophenotypic, Epstein-Barr virus (EBV) in situ hybridi zation, and genotypic analyses is presented. The patient had a well-do cumented history of systemic lupus erythematosus (SLE) and was found a t autopsy to have massive lymphadenopathy, thymic enlargement, pulmona ry nodules, and polyclonal serum dysproteinemia. Histological examinat ion 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 d epletion. 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 proce ss, as well as the unusual histological features make this case a nota ble addition to the spectrum of atypical lymphoproliferative disorders associated with an autoimmune disorder. We conclude that although rem iniscent of angioimmunoblastic lymphadenopathy with dysproteinemia (AI LD), 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.