Yuan Dan, Liang Na, Wang Dong-Yue, Wang Jin-Jing, Jia Cong-Wei
Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi, China.
Department of Histology and Embryology, School of Basic Medicine, Zunyi Medical University, Zunyi, China.
Front Immunol. 2025 May 21;16:1530149. doi: 10.3389/fimmu.2025.1530149. eCollection 2025.
This study aimed to investigate the clinicopathological features of indolent T-cell lymphoproliferative disease of the gastrointestinal tract (ITLPD-GI) and to improve its diagnostic and therapeutic approaches.
A retrospective analysis was conducted on eight ITLPD-GI patients treated between January 2018 and January 2024. Clinical data, pathological features, immunophenotypes, molecular testing results, and follow-up records were reviewed.
Clinical characteristics: Male-to-female ratio 3:5; mean age at onset 42 years. Symptoms: Predominantly diarrhea and abdominal pain. Endoscopic findings: Erosions, multiple shallow ulcers, and small polypoid lesions. Pathological features: Histology: Atrophy of gastric/intestinal glands with diffuse infiltration of small lymphocytes (round/irregular nuclei, dense chromatin) in the lamina propria; rare mitoses; absence of angioinvasion or necrosis. Notably, two cases showed prominent plasma cell infiltration in the superficial mucosa. Immunophenotype: Pan-T-cell markers positive (5/8); CD4-/CD8+ (5/8), CD4+/CD8+ (2/8), CD4+/CD8- (1/8); aberrant CD20 expression (2/8); low Ki-67 index. TCR rearrangement: Monoclonal in all four tested cases. Treatment and prognosis: Supportive therapy (five cases): Dietary modification, immunosuppression, immunomodulation, and anti-infective agents. Symptoms resolved in one case but persisted in four. Targeted therapy (one CD20+ case): Rituximab added, with no improvement after 14 months of follow-up. Chemotherapy (two cases): Prednisone + thalidomide; one achieved significant remission at 9 months, while the other showed no response (persistent diarrhea/anxiety) at 35 months. No disease progression was observed during follow-up.
ITLPD-GI is a rare indolent monoclonal T-cell proliferation with non-specific clinical/endoscopic features, necessitating differentiation from aggressive lymphomas to avoid misdiagnosis and overtreatment. Diagnosis relies on histomorphology, immunohistochemistry, and TCR clonality assessment (critical for atypical cases, e.g., CD20+). The majority of patients have favorable outcomes with conservative management. Enhanced clinical awareness and novel therapeutic targets warrant further exploration.
本研究旨在探讨胃肠道惰性T细胞淋巴增殖性疾病（ITLPD-GI）的临床病理特征，并改进其诊断和治疗方法。
对2018年1月至2024年1月期间接受治疗的8例ITLPD-GI患者进行回顾性分析。回顾临床资料、病理特征、免疫表型、分子检测结果及随访记录。
临床特征：男女比例为3:5；平均发病年龄42岁。症状：以腹泻和腹痛为主。内镜检查结果：糜烂、多发浅溃疡及小息肉样病变。病理特征：组织学：胃/肠腺萎缩，固有层有小淋巴细胞弥漫浸润（核圆形/不规则，染色质致密）；罕见有丝分裂；无血管侵犯或坏死。值得注意的是，2例在浅表黏膜有显著浆细胞浸润。免疫表型：泛T细胞标志物阳性（5/8）；CD4-/CD8+（5/8），CD4+/CD8+（2/8），CD4+/CD8-（1/8）；异常CD20表达（2/8）；Ki-67指数低。TCR重排：4例检测病例均为单克隆。治疗与预后：支持治疗（5例）：饮食调整、免疫抑制、免疫调节及抗感染药物。1例症状缓解，4例持续存在。靶向治疗（1例CD20+病例）：加用利妥昔单抗，随访14个月无改善。化疗（2例）：泼尼松+沙利度胺；1例在9个月时显著缓解，另1例在35个月时无反应（持续腹泻/焦虑）。随访期间未观察到疾病进展。
ITLPD-GI是一种罕见的惰性单克隆T细胞增殖性疾病，具有非特异性临床/内镜特征，需要与侵袭性淋巴瘤相鉴别以避免误诊和过度治疗。诊断依赖于组织形态学、免疫组化及TCR克隆性评估（对非典型病例如CD20+至关重要）。大多数患者采用保守治疗预后良好。提高临床认识和探索新的治疗靶点值得进一步研究。
