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Splenic manifestations of chronic autoimmune disorder: a report of five cases with histiocytic necrotizing change in four cases
Author(s) -
Auerbach Aaron,
Summers Thomas A,
Zhang Binxue,
Aguilera Nadine S
Publication year - 2013
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.12143
Subject(s) - pathology , spleen , histiocyte , medicine , necrosis , histiocytosis , immunology , biology , disease
Aims Autoimmune diseases ( AD ) are associated with lymphadenopathy and splenomegaly. Changes in the spleen have not been characterized completely in AD ; we describe splenectomy specimens from five patients with chronic AD , highlighting the presence of necrotizing histiocytosis. Methods and results Of the patients (three males and two females; mean 40 years), four had systemic lupus erythematosus; one had rheumatoid arthritis. All had moderate splenomegaly (213–803 g, mean 421 g). Four cases exhibited necrosis with apoptosis and karyorrhectic debris occurring in the white pulp and minimal acute inflammation; one showed florid follicular hyperplasia. Splenic involvement ranged from focal to extensive. Plasma cells were negative for Ig G 4. Haematoxylin bodies were not identified. Stains for infectious organisms were negative. Immunohistochemical studies showed that lymphocytes surrounding the necrosis were a mixture of CD 4 + and CD 8 + T cells; CD 123‐positive plasmacytoid dendritic cells were not present, and staining for kappa and lambda light chains showed no clonality. 16 S r DNA PCR was performed; no amplification was seen in three of four cases tested for bacteria specific r DNA . E pstein– B arr virus‐encoded RNA ( EBER ) in situ hybridization studies highlighted rare positive cells in four cases. Conclusions Splenomegaly in AD is thought to be hyperplasic, but we present four cases showing histiocytic necrosis, a finding which should be considered part of the spectrum of AD in the spleen.