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伴发播散性多结节累及的惰性T淋巴母细胞增殖并且部分表达CD33
尽管惰性T淋巴母细胞增殖(iT-LBP)罕见,对任何患者作出这个诊断之前,都应该排除幼稚的TdT阳性T细胞的胸腺外增殖。与T淋巴母细胞性白血病/淋巴瘤不同,iT-LBP患者不需要化疗。我们报道一例发生在49岁健康女性患者的伴发播散性多结节累及的iT-LBP。数月中我们对多个淋巴结进行了活检,但持续性的有不同部位的淋巴结受累。长达18个月之久,我们对她没有做任何治疗,该患者仍然很健康,并且她的淋巴结病有了显著的改善。骨髓和外周血均未受累。通过流式细胞学和免疫组织化学技术发现不典型的T细胞表达T细胞抗原谱系及部分表达CD33.对淋巴结活检标本,对其进行T细胞克隆能力和人类雄激素受体含量测(HUMARA)的研究均为阴性。本文首先详细的从临床特点、形态学、分子学及免疫表型方面描述了伴发播散性多结节累及的非克隆性iT-LBP,部分T细胞表达CD33.                                            Am J Surg Pathol 2014;00:000–000
Abstract: Although indolent T-lymphoblastic proliferations(iT-LBP) are rare, this diagnosis should be excluded in any patient with an extrathymic proliferation of immature TdT+T cells. Unlike T-lymphoblastic leukemia/lymphoma, patients with iT-LBP do not require chemotherapy. We report a case of iT-LBP with disseminated multinodal involvement in an other-wise healthy 49-year-old woman. Multiple lymph node biopsies were performed over the course of several months demonstrat-ing persistent and anatomically diffuse involvement. Over 18 months, and without therapy, she has remained healthy, and her lymphadenopathy significantly improved. No bone marrow or peripheral blood involvement was ever identified. Atypical T cells showed an immunophenotypic spectrum of T-cell antigen expression with partial CD33 on a subset of T cells detected by both flow cytometry and immunohistochemistry. Both T-cell clonality and Human Androgen Receptor Assay (HUMARA) studies, performed on lymph node biopsy specimens, were negative. This case represents the first detailed clinical, mor-phologic, molecular, and immunophenotypic description of disseminated multinodal involvement by nonclonal iT-LBP with partial CD33 expression on T cells.             

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