Clinical and pathological analysis of indolent T-cell lymphoproliferative disease of the gastrointestinal tract
ObjectiveThis 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.MethodsA retrospective analysis was conducted on eight ITLPD-GI patients treated...
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Frontiers Media S.A.
2025-05-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1530149/full |
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| author | Dan Yuan Na Liang Dong-Yue Wang Jin-Jing Wang Cong-Wei Jia |
| author_facet | Dan Yuan Na Liang Dong-Yue Wang Jin-Jing Wang Cong-Wei Jia |
| author_sort | Dan Yuan |
| collection | DOAJ |
| description | ObjectiveThis 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.MethodsA 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.ResultsClinical 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.ConclusionITLPD-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. |
| format | Article |
| id | doaj-art-fe9b522f5d014b5490dfca570db7bd56 |
| institution | OA Journals |
| issn | 1664-3224 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Immunology |
| spelling | doaj-art-fe9b522f5d014b5490dfca570db7bd562025-08-20T01:55:21ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-05-011610.3389/fimmu.2025.15301491530149Clinical and pathological analysis of indolent T-cell lymphoproliferative disease of the gastrointestinal tractDan Yuan0Na Liang1Dong-Yue Wang2Jin-Jing Wang3Cong-Wei Jia4Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi, ChinaDepartment of Histology and Embryology, School of Basic Medicine, Zunyi Medical University, Zunyi, ChinaDepartment of Pathology, Jilin Municipal People’s Hospital, Jilin, ChinaDepartment of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi, ChinaDepartment of Pathology, Peking Union Medical College Hospital, Beijing, ChinaObjectiveThis 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.MethodsA 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.ResultsClinical 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.ConclusionITLPD-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.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1530149/fullIndolent T-cell lymphoproliferative disease of the gastrointestinal tract (ITLPD-GT)clinical and pathological featuresabnormal positive expression of CD20TCR monoclonal rearrangementtreatment |
| spellingShingle | Dan Yuan Na Liang Dong-Yue Wang Jin-Jing Wang Cong-Wei Jia Clinical and pathological analysis of indolent T-cell lymphoproliferative disease of the gastrointestinal tract Frontiers in Immunology Indolent T-cell lymphoproliferative disease of the gastrointestinal tract (ITLPD-GT) clinical and pathological features abnormal positive expression of CD20 TCR monoclonal rearrangement treatment |
| title | Clinical and pathological analysis of indolent T-cell lymphoproliferative disease of the gastrointestinal tract |
| title_full | Clinical and pathological analysis of indolent T-cell lymphoproliferative disease of the gastrointestinal tract |
| title_fullStr | Clinical and pathological analysis of indolent T-cell lymphoproliferative disease of the gastrointestinal tract |
| title_full_unstemmed | Clinical and pathological analysis of indolent T-cell lymphoproliferative disease of the gastrointestinal tract |
| title_short | Clinical and pathological analysis of indolent T-cell lymphoproliferative disease of the gastrointestinal tract |
| title_sort | clinical and pathological analysis of indolent t cell lymphoproliferative disease of the gastrointestinal tract |
| topic | Indolent T-cell lymphoproliferative disease of the gastrointestinal tract (ITLPD-GT) clinical and pathological features abnormal positive expression of CD20 TCR monoclonal rearrangement treatment |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1530149/full |
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