Adult T-cell leukemia/lymphoma coexistent with immune dysregulation–associated EBV⁺ lymphoproliferative disorder Free

A 54-year-old woman presented with generalized lymphadenopathy and positive human T-lymphotropic virus 1 serology. Bone marrow and peripheral blood flow cytometry, along with cervical and inguinal lymph node biopsies, revealed CD25⁺ atypical T cells, leading to a diagnosis of adult T-cell leukemia/lymphoma (ATLL). One month later, the patient experienced diarrhea and abdominal pain. A colonoscopy revealed diffuse ulcerative lesions throughout the colon (panel A). Colonoscopic biopsy revealed infiltration of atypical lymphoid cells in the mucosa and submucosa, with medium to large cells exhibiting irregular morphology and occasional nucleoli. The background comprised small lymphocytes, immunoblasts, plasmacytoid cells, and eosinophils (panels B-D, hematoxylin and eosin stain; ×10 original magnification [B]; ×40 original magnification [C]; ×60 original magnification [D]). The atypical cells were positive for CD3, CD4, and CD25 with CD7 loss. Concurrently, plasmacytoid cells expressed CD79a (panel E, ×60 original magnification, CD25 [red]/CD79a [brown] double-stain), MUM1, and CD38, with λ light chain restriction confirmed by κ (brown)/λ (red) double staining (panel F, ×60 original magnification). The Ki67 was high (panel G, ×40 original magnification). EBNA2 and LMP1 were focally expressed, and EBER was positive (panels H-J, ×40 original magnification). Concurrently, the patient exhibited monoclonal IGH and TRG rearrangements.

The colonic lesion was diagnosed as ATLL concurrent with immune dysregulation-associated EBV⁺ lymphoproliferative disorder. The patient succumbed to the disease within 4 months of diagnosis. To our knowledge, this is the first reported case of ATLL coexistent with polymorphic lymphoproliferative disorder.

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