Angioimmunoblastic T-cell lymphoma (AITL) originates from follicular helper T cells and shows variable biological and clinical presentations. The survival rate of patients with AITL did not correlate with T-cell clonality, the presence of EBV-infected cells, EBV-DNA copy number, or IgH rearrangements. However, tumor-associated macrophage/M2 macrophages significantly correlated with worse overall survival (OS). Additionally, patients with composite lymphoma with diffuse large B cell lymphoma and AITL showed worse OS. We reported mutations in TET2, DNMT3A, IDH2, and RHOA. TET2 and DNMT3A mutations were identified in both programmed cell death 1 (PD1) + T cells and CD20+ cells. All RHOA and IDH2 mutations were confined to PD1+ T cells, whereas several mutations, including NOTCH1 mutations, were detected only in CD20+ cells. TET2 and DNMT3A mutations may originate in hematopoietic progenitor cells. Adult T-cell leukemia/lymphoma (ATLL) expresses CCR4 and FOXP3 of the regulatory T-cell marker. The p40tax viral protein leads to the transcriptional activation of several genes. In addition, the HTLV-1 basic leucine zipper factor is considered important for T-cell proliferation and oncogenesis. The presence of Tax-specific cytotoxic T lymphocytes is inversely correlated with FOXP3 expression. M2 macrophages were associated with worse clinical prognosis in patients with ATLL. Programmed cell death ligand 1 (PD-L1) expression showed two patterns, namely neoplastic PD-L1 (nPD-L1) and microenvironmental PD-L1 (miPD-L1). Patients with nPD-L1-positive ATLL cells had inferior OS, whereas those with miPD-L1-positive ATLL cells had superior OS. Patients with a HLA+ beta2M+ phenotype had significantly better prognosis, and those with a HLAm+ beta2Mm+ miPD-L1high phenotype demonstrated the most favorable prognosis. Thus, our study demonstrated that understanding the microenvironment is critical in discerning the clinicopathological features of peripheral T-cell lymphoma.Keywords: AITL; ATLL; Immunity; Microenvironment.

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乐备实（上海优宁维生物科技股份有限公司旗下全资子公司），是国内专注于提供高质量蛋白检测以及组学分析服务的实验服务专家，自2018年成立以来，乐备实不断寻求突破，公司的服务技术平台已扩展到单细胞测序、空间多组学、流式检测、超敏电化学发光、Luminex多因子检测、抗体芯片、PCR Array、ELISA、Elispot、PLA蛋白互作、多色免疫组化、DSP空间多组学等30多个，建立起了一套涵盖基因、蛋白、细胞以及组织水平实验的完整检测体系。

 
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