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惰性T淋巴细胞增殖:一例未经细胞毒性治疗病程达16年的病例报告。

Indolent T-lymphoblastic proliferation: report of a case with a 16-year course without cytotoxic therapy.

作者信息

Velankar M M, Nathwani B N, Schlutz M J, Bain L A, Arber D A, Slovak M L, Weiss L M

机构信息

Department of Pathology, University of Southern California School of Medicine, USA.

出版信息

Am J Surg Pathol. 1999 Aug;23(8):977-81. doi: 10.1097/00000478-199908000-00017.

DOI:10.1097/00000478-199908000-00017
PMID:10435569
Abstract

T-lymphoblastic lymphoma is a high-grade malignant lymphoma. Clinically indolent T-lymphoblastic proliferations have not been described. We present a case report of an indolent T-cell lymphoblastic proliferation studied by histopathology, immunohistochemistry, flow cytometry, antigen receptor gene rearrangement studies, and cytogenetics. The patient had recurrent masses in the upper aerodigestive tract over a 16-year period, was treated by multiple surgical excisions, and never received either chemotherapy or radiotherapy. A proliferation of lymphoblasts was present histologically. The cells were positive for terminal deoxynucleotidyl transferase, CD1, and CD3, and coexpressed CD4 and CD8. No clonal rearrangements of the T-cell receptor beta or gamma chain genes were identified. Cytogenetic studies revealed a questionable inversion of the short arm of chromosome 9, affecting the 9p21-22 region. Although ectopic thymic tissue was considered, the case was considered to be an indolent lymphoblastic proliferation. It should be recognized that rare lymphoblastic proliferations may not behave in a high grade fashion as typically seen in T-lymphoblastic lymphoma.

摘要

T淋巴细胞母细胞淋巴瘤是一种高度恶性的淋巴瘤。临床上尚未描述过惰性T淋巴细胞母细胞增殖情况。我们报告一例惰性T细胞淋巴细胞母细胞增殖的病例,该病例通过组织病理学、免疫组织化学、流式细胞术、抗原受体基因重排研究和细胞遗传学进行研究。患者在16年期间上呼吸消化道反复出现肿块,接受了多次手术切除,从未接受过化疗或放疗。组织学检查显示有淋巴细胞母细胞增殖。这些细胞末端脱氧核苷酸转移酶、CD1和CD3呈阳性,并共同表达CD4和CD8。未发现T细胞受体β或γ链基因的克隆重排。细胞遗传学研究显示9号染色体短臂有可疑倒位,累及9p21-22区域。尽管考虑了异位胸腺组织,但该病例被认为是惰性淋巴细胞母细胞增殖。应该认识到,罕见的淋巴细胞母细胞增殖可能不会表现出T淋巴细胞母细胞淋巴瘤中常见的高度恶性行为。

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