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Training Level

Resident PGY 3


Henry Ford Hospital


Introduction: Polymorphic B-cell lymphoproliferative disorders (B-LPDs) are a rare, morphologically heterogeneous, and diagnostically challenging group of neoplasms that occur in the setting of immunosuppression. Associated lesions are almost uniformly Epstein-Barr virus-positive (EBV+) and can cause destructive masses that may mimic more aggressive lymphomas. Clinical outcomes are highly variable, ranging from resolution with withdrawal of immunosuppression to fatal dissemination of disease.

Clinical case: A 77-year-old white male with a history of advanced chronic lymphocytic leukemia (CLL) presented with a six-week history of a tender sore on his right lower lip. He had previously been treated with acyclovir without improvement. Physical exam revealed a 1 cm firm tender nodule with overlying crusted erosion on the right lower lip. A deep saucerization biopsy was performed which showed a dense lymphoplasmacytic infiltrate composed primarily of plasmacytoid cells extending into the subcutis. In-situ hybridization for EBV (EBER) was diffusely positive, while CD30, CD20, and Pax-5 immunostains showed only rare clusters of positive cells. The kappa/lambda ratio was 1:10, consistent with lambda light chain restriction. IGH clonality assay failed to detect a monoclonal population. On the basis of these results, a diagnosis of EBV+ polymorphic B-LPD was favored. Unfortunately, the patient expired a few weeks after presentation as a result of complications from his CLL.

Conclusion: We report a rare case of cutaneous EBV+ polymorphic B-LPD. Prompt and accurate diagnosis of this entity is important to avoid pursuit of unnecessarily aggressive therapies, prompt workup for undiagnosed immunodeficiency, and minimize the risk of fatal progression.

Presentation Date


EBV+ B-cell polymorphic lymphoproliferative disorder of the lip in a patient with advanced chronic lymphocytic leukemia