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T-ALL presenting with i-TLP-like indolent clinical course with repeated spontaneous regressions.

Pathol Res Pract

November 2024

Division of Hematology, Department of Medicine, Jichi Medical University, Tochigi, Japan. Electronic address:

Rapidly progressing ALL, a potentially fatal disease, demands timely diagnosis and treatment. On the other hand, spontaneous remission/regression (SR) is reported in various cancers including aggressive tumors like ALL. Infection or trauma-mediated immune system activation is assumed to cause SR, with the duration in cases of ALL typically being short.

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Indolent T-lymphoblastic proliferation (iT-LBP) is a rare, non-clonal, extrathymic lymphoid proliferation with an immature T cell phenotype, indolent clinical course, and excellent prognosis. Although their pathogenesis is unclear, they are reported to be associated with Castleman disease, follicular dendritic cell tumors/sarcomas, angioimmunoblastic T cell lymphoma, hepatocellular carcinoma (HCC), myasthenia gravis, and acinic cell carcinoma. There are around 51 reported cases of iT-LBP in the literature.

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Indolent T-lymphoblastic proliferation (iT-LBP) consists of a proliferation of non-neoplastic TdT + T cells in extrathymic tissues, requiring no treatment. However, due to overlapping clinical and histologic features, distinguishing iT-LBP from T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma (T-ALL/LBL) can be challenging. Recently, flow cytometry-based evaluation of TRBC1 has been used to detect of T-cell clonality in TCRαβ + mature T-cell lymphomas and aid in the differential diagnosis between T-ALL and normal thymocytes.

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Indolent T-lymphoblastic proliferation (iT-LBP) is a non-neoplastic disease with an indolent clinical course, manifesting as hyperplasia of immature extrathymic T-lymphoblastic cells. Isolated iT-LBP has been observed, but the majority of iT-LBP cases has been seen in conjunction with other diseases. iT-LBP is easily misdiagnosed as T-lymphoblastic lymphoma/leukemia, and understanding the disease of indolent T-lymphoblastic proliferation may prevent misdiagnosis and missed diagnosis in pathological diagnosis.

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