Abstract：
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. Recognition and accurate diagnosis of this entity is critical as it shares morphologic and immunophenotypic features with an aggressive malignancy-acute T cell leukemia/lymphoma (T-ALL). IT-LBP in HCC post-liver transplant and in metastatic sites has not been reported in the literature. Two case reports of patients presenting with recurrent and metastatic HCC in post-liver transplant settings are described. A 50-year-old man with an end-stage liver disease with HCC underwent liver transplant. A year later, he developed pulmonary metastasis with associated iT-LBP. A 69-year-old man underwent liver transplant for end-stage liver disease and HCC. Eighteen months later, he developed recurrent HCC in the transplanted liver and omental metastasis; both sites showed HCC with iT-LBP. iT-LBP in both patients expressed TdT, CD3, and CD4 and lacked CD34 and clonal T cell receptor gene rearrangements. On retrospective review, the pre-transplant HCC specimens lacked iT-LBP. We present two cases of iT-LBP associated with HCC in novel settings-in post-liver transplant patients and in recurrent/metastatic sites of HCC. In addition, a comprehensive literature review of clinical, histological, and immunophenotypic characteristics of reported cases of iT-LBP is presented.
摘要：
惰性T淋巴细胞增殖（iT-LBP）是一种罕见的，非克隆，具有未成熟T细胞表型的胸腺外淋巴样增殖，惰性临床过程，和良好的预后。虽然其发病机制尚不清楚,据报道，它们与Castleman病有关，滤泡树突状细胞肿瘤/肉瘤，血管免疫母细胞性T细胞淋巴瘤,肝细胞癌（HCC），重症肌无力,和腺泡细胞癌。文献中报道了约51例iT-LBP。该实体的识别和准确诊断至关重要，因为它与侵袭性恶性肿瘤-急性T细胞白血病/淋巴瘤（T-ALL）具有形态学和免疫表型特征。在肝癌肝移植后和转移部位的IT-LBP尚未在文献中报道。描述了在肝移植后环境中出现复发性和转移性HCC的患者的两个病例报告。一名50岁患有晚期肝病的肝癌患者接受了肝移植。一年后,他出现肺转移并伴有iT-LBP。一名69岁的男子接受了终末期肝病和HCC的肝移植。十八个月后,他在移植肝和网膜转移中发展为复发性HCC；两个部位均显示iT-LBP的HCC。两名患者的iT-LBP均表达TdT，CD3、CD4和缺乏CD34和克隆性T细胞受体基因重排。在回顾性审查中，移植前肝癌标本缺乏iT-LBP。我们介绍了在肝移植后患者和HCC复发/转移部位的新环境中与HCC相关的2例iT-LBP。此外,全面的临床文献综述，组织学,并介绍了iT-LBP报告病例的免疫表型特征。
