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In this study, we conducted integrated molecular analyses of the transcriptome and tumor genome in 24 newly diagnosed patients with angioimmunoblastic T-cell lymphoma (AITL). Gene expression profiling revealed significant enrichment of B cell receptor signaling and innate immune-related pathways in the response group. CIBERSORT-based deconvolution analysis showed that the proportions of tumor-infiltrating B cells and M1 macrophages were significantly higher in the response group compared to the non-response group (B cells: 17.4% vs. 7.8%, P = 0.012; M1 macrophages: 11.3% vs. 6.1%, P = 0.005). The abundance of these immune cells was associated with favorable progression-free survival and overall survival. Conversely, T follicular helper (TFH) cells, which reflect tumor burden, were inversely correlated with these favorable immune subsets. The most frequently mutated genes in this cohort included TET2 (73.9%), RHOA (47.8%), IDH2 (34.7%), and DNMT3A (26.1%). Mutations in RHOA, IDH2, and DNMT3A were negatively correlated with tumor-infiltrating B cells and were associated with poorer survival outcomes. Furthermore, higher variant allele frequencies (VAFs) of TET2 mutations were also negatively correlated with B cell infiltration, while RHOA VAFs were positively associated with TFH cell abundance, suggesting a link between mutational clonality and immune suppression. In conclusion, our study highlights the interplay between tumor genetic alterations and the immune microenvironment in AITL. We identified a favorable immune profile, characterized by increased infiltration of B cells and M1 macrophages, that correlates with chemosensitivity and improved prognosis. In contrast, mutations in RHOA, IDH2, DNMT3A, and high VAFs of TET2 were associated with adverse clinical outcomes and unfavorable immune contexture.
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http://dx.doi.org/10.1007/s00277-025-06589-3 | DOI Listing |
Background: Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive form of peripheral T-cell lymphoma, accounting for 1 - 2% of non-Hodgkin lymphomas. Diagnosis is challenging, and there is no established standard first-line treatment. This case report highlights a rare progression from AITL to therapy-related acute myeloid leukemia (AML-pCT) following cytotoxic chemotherapy.
View Article and Find Full Text PDFAnn Hematol
September 2025
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Wangfujing Shuaifuyuan, Dongdan, Beijing, 100730, China.
In this study, we conducted integrated molecular analyses of the transcriptome and tumor genome in 24 newly diagnosed patients with angioimmunoblastic T-cell lymphoma (AITL). Gene expression profiling revealed significant enrichment of B cell receptor signaling and innate immune-related pathways in the response group. CIBERSORT-based deconvolution analysis showed that the proportions of tumor-infiltrating B cells and M1 macrophages were significantly higher in the response group compared to the non-response group (B cells: 17.
View Article and Find Full Text PDFAm J Dermatopathol
September 2025
Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY.
Primary cutaneous Epstein-Barr virus (EBV)-positive polymorphic B-cell lymphoproliferative disorder (LPD) is a rare LPD associated with iatrogenic and endogenous immune dysregulation with the commonest risk factor being immunosuppressive therapy. We present a 55-year-old woman with rheumatoid arthritis, previously on methotrexate, who developed a waxing and waning papulonodular eruption on the chest and neck. Histopathology revealed a lymphohistiocytic infiltrate with atypical EBV+/CD30+ B cells, consistent with EBV+ polymorphic B-cell LPD.
View Article and Find Full Text PDFVirchows Arch
September 2025
Pathology Department, Health Research Institute-Fundación Jiménez Díaz University Hospital, Universidad Autónoma de Madrid (IIS-FJD, UAM), 28040, Madrid, Spain.
In the context of immunosuppression, most lymphoproliferative disorders are of B-cell origin and associated with Epstein-Barr virus (EBV). Follicular Helper T-cell Lymphoma, Angioimmunoblastic type (AITL) is the most frequently encountered T-cell lymphoma subtype. We present a challenging AITL case, initially misinterpreted as a polymorphic EBV + B-cell lymphoma, in a 62-year-old woman with a history of rheumatoid arthritis treated with multiple immunosuppressive agents.
View Article and Find Full Text PDFCureus
July 2025
Division of Hematology and Oncology, Kyoto Prefectural University of Medicine, Kyoto, JPN.
Immune checkpoint inhibitors (ICIs) are innovative immunotherapeutic agents used to treat various types of cancer by enhancing T-cell-mediated antitumor activity. These agents have distinct adverse events, known as immune-related adverse events, which can affect multiple organ systems and typically occur within a year after initiating ICI therapy. Another concern regarding ICI therapy is the potential development of T-cell lymphomas due to prolonged activation of T-cell activity.
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