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Ki-1阳性大细胞淋巴瘤,一组异质性肿瘤。24例病例的形态学、免疫表型、基因型及临床特征

Ki-1-positive large cell lymphomas, a heterogenous group of neoplasms. Morphologic, immunophenotypic, genotypic, and clinical features of 24 cases.

作者信息

Penny R J, Blaustein J C, Longtine J A, Pinkus G S

机构信息

Pathology Department of Brigham and Women's Hospital, Boston, Massachusetts 02115.

出版信息

Cancer. 1991 Jul 15;68(2):362-73. doi: 10.1002/1097-0142(19910715)68:2<362::aid-cncr2820680226>3.0.co;2-c.

DOI:10.1002/1097-0142(19910715)68:2<362::aid-cncr2820680226>3.0.co;2-c
PMID:1648995
Abstract

Clinical and pathologic features of 24 patients with large cell lymphomas that expressed the activation antigen Ki-1 are described. Phenotypic and/or genotypic studies characterized these neoplasms as T-cell (16 cases), B-cell (six cases), or null cell (two cases) type. Males predominantly were affected. Age of patients ranged from 19 to 73 years, with a bimodal distribution, with peaks in the third and seventh decades. Lymphadenopathy was present in nearly all patients. Extranodal involvement, including skin, soft tissue, bone, central nervous system, lung, or small intestine was observed in a total of 54% of the patients, either at presentation or during the course of disease. "Prototypic" features of large cell anaplastic lymphomas were observed for eight T-cell lymphomas, with morphologic heterogeneity noted for the remainder. Eight patients, all with T-cell neoplasms (only one with prototypic morphology), have died of lymphoma (median survival, 5 months). An antecedent history of a lymphoproliferative disorder (mycosis fungoides, B-cell lymphoma, immunoblastic lymphadenopathy) was apparent in seven patients. An 8-year history of Crohn's disease occurred in one patient with a T-cell lymphoma involving small intestine. Phenotypically, loss of one or more markers was typically noted for T-cell neoplasms. Leukocyte common antigen was detected in all cases, although partial loss of immunoreactivity was noticed in some cases. Nearly all cases evaluated for Ia antigen or alpha-1-antichymotrysin were reactive. Eleven of 16 T-cell, two of six B-cell, and two null cell lymphomas expressed epithelial membrane antigen. Ki-1-positive large cell lymphomas are characterized by clinical, morphologic, and immunophenotypic heterogeneity.

摘要

本文描述了24例表达激活抗原Ki-1的大细胞淋巴瘤患者的临床和病理特征。表型和/或基因型研究将这些肿瘤分为T细胞型(16例)、B细胞型(6例)或无细胞型(2例)。男性受累为主。患者年龄范围为19至73岁,呈双峰分布,高峰在第三个和第七个十年。几乎所有患者都有淋巴结病。共有54%的患者在就诊时或疾病过程中出现结外受累,包括皮肤、软组织、骨骼、中枢神经系统、肺或小肠。8例T细胞淋巴瘤观察到了大细胞间变性淋巴瘤的“典型”特征,其余病例形态学具有异质性。8例患者(均为T细胞肿瘤,仅1例具有典型形态)死于淋巴瘤(中位生存期5个月)。7例患者有淋巴增殖性疾病(蕈样霉菌病、B细胞淋巴瘤、免疫母细胞性淋巴结病)的既往史。1例患有累及小肠的T细胞淋巴瘤的患者有8年克罗恩病病史。表型上,T细胞肿瘤通常会出现一种或多种标志物的缺失。所有病例均检测到白细胞共同抗原,尽管在某些病例中发现免疫反应性部分丧失。几乎所有检测Ia抗原或α-1抗糜蛋白酶的病例均呈阳性反应。16例T细胞淋巴瘤中的11例、6例B细胞淋巴瘤中的2例以及2例无细胞淋巴瘤表达上皮膜抗原。Ki-1阳性大细胞淋巴瘤具有临床、形态学和免疫表型异质性。

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