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Indolent T-lymphoblastic proliferation with disseminated multinodal involvement and partial CD33 expression.

伴播散性多结节累及和部分表达CD33 的惰性T 淋巴母细胞增生

Ohgami RS,Sendamarai AK,Atwater SK,Liedtke M,Fleming MD,Natkunam Y,Warnke RA

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 otherwise healthy 49-year-old woman. Multiple lymph node biopsies were performed over the course of several months demonstrating 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, morphologic, molecular, and immunophenotypic description of disseminated multinodal involvement by nonclonal iT-LBP with partial CD33 expression on T cells.

摘要

尽管惰性T淋巴母细胞增生(iT-LBP)罕见,但在胸腺外未成熟性TdT阳性T细胞增殖的患者应注意排除该疾病。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.本研究报道1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.数月中对多个淋巴结进行了活检,但仍持续性的有不同部位的淋巴结受累.病程长达18个月之久,患者没有做任何治疗,但无碍健康,并且淋巴结肿大有显着改善,骨髓和外周血均未受累,流式细胞检测和免疫组织化学染色发现,不典型的T细胞表达T细胞抗原谱系且部分表达CD33.对淋巴结活检标本,进行T细胞克隆性分析和人类雄激素受体测定(HUMARA),均为阴性.本研究首次详细的从临床特点、形态学、分子及免疫表型方面描述了伴播散性多结节累及的非克隆性iT-LBP,部分T细胞表达CD33.

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