Frontiers in Medicine · Published 2026-08-03 · DOI 10.3389/fmed.2026.1918778
Zhaoxia He, Yan Zeng, Dongmei Zeng, Rong Zhao, Tao Wang
BackgroundDiffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma, whereas primary splenic DLBCL (PS-DLBCL) is exceedingly rare. Lymphomas that mimic systemic lupus erythematosus (SLE) are clinically heterogeneous and share overlapping manifestations with SLE, rendering diagnosis extremely challenging.Case presentationWe report a case of an elderly patient presenting with recurrent fever, a hyperinflammatory state, and cytopenias, along with positivity for multiple autoimmune antibodies and lupus anticoagulant, which strongly suggested a clinical diagnosis of SLE. However, the patient showed no response to adequate doses of glucocorticoid therapy. Subsequent bone marrow and peripheral blood flow cytometry revealed a minor population of aberrant phenotype B cells, and positron emission tomography-computed tomography demonstrated mild splenomegaly with diffuse increased fluorodeoxyglucose uptake in the splenic parenchyma. A definitive diagnosis of PS-DLBCL was established following splenectomy. Unfortunately, the patient suffered sudden cardiac death shortly after diagnosis.ConclusionThis case highlights that PS-DLBCL can present with lupus-like manifestations as an atypical clinical feature, and such variant presentation may herald a poor prognosis.
Abstract from DOAJ. Public domain (CC0 1.0).
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He, Z., Zeng, Y., Zeng, D., et al. (2026). Primary splenic diffuse large B-cell lymphoma masquerading as systemic lupus erythematosus: a case report. Frontiers in Medicine. https://doi.org/10.3389/fmed.2026.1918778