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黏膜相关淋巴组织结外边缘区B细胞淋巴瘤向弥漫性大B细胞淋巴瘤组织学转化的临床病理特征:467例患者分析

Clinicopathological features of histological transformation from extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue to diffuse large B-cell lymphoma: an analysis of 467 patients.

作者信息

Maeshima Akiko Miyagi, Taniguchi Hirokazu, Toyoda Kosuke, Yamauchi Nobuhiko, Makita Shinichi, Fukuhara Suguru, Munakata Wataru, Maruyama Dai, Kobayashi Yukio, Tobinai Kensei

机构信息

Department of Pathology, National Cancer Centre Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.

Department of Hematology, National Cancer Centre Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.

出版信息

Br J Haematol. 2016 Sep;174(6):923-31. doi: 10.1111/bjh.14153. Epub 2016 Jul 27.

DOI:10.1111/bjh.14153
PMID:27460179
Abstract

This study analysed incidence, patient outcome, immunophenotype and prognostic factors of histological transformation (HT) from extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) to diffuse large B-cell lymphoma (DLBCL) in 467 patients (median age, 61 years). The primary sites of MALT lymphoma were the stomach (43%), ocular adnexa (25%), lung (8%), systemic (8%) and other tissues (16%). HT occurred in 8% of MALT lymphomas. Risk of HT by 15 years was 5%: 4% in limited-stage diseases (n = 385) and 16% in advanced-stage diseases (n = 56) (P = 0·02). The median time to HT was 48 months (range, 4-139). Five-year progression-free survival (PFS) and overall survival (OS) rates after HT were 80% and 94%, respectively. Immunohistochemical results of DLBCL were as follows: germinal centre B-cell (GCB)/non-GCB, 37%/63%; CD10, 9%; BCL6, 59%; MUM1, 38%; MYC, 42%; BCL2, 35%; Ki67 ≥ 90%, 23%; and CD5, 3%. The majority (75%, 9/12) of GCB-type DLBCLs exhibited CD10(-) , BCL6(+) and MUM1(-) immunophenotypes; the remainder had CD10(+) immunophenotypes. Multivariate analysis revealed that only advanced stage at HT was a significant adverse factor for PFS (P = 0·037). Thus, overall risk of HT was low and prognosis after HT was favourable; however, in advanced-stage cases, risk of HT was relatively high and prognosis was unfavourable.

摘要

本研究分析了467例(中位年龄61岁)黏膜相关淋巴组织结外边缘区B细胞淋巴瘤(MALT淋巴瘤)转化为弥漫性大B细胞淋巴瘤(DLBCL)的组织学转化(HT)的发生率、患者结局、免疫表型及预后因素。MALT淋巴瘤的原发部位为胃(43%)、眼附属器(25%)、肺(8%)、全身(8%)和其他组织(16%)。8%的MALT淋巴瘤发生了HT。15年时HT的风险为5%:局限期疾病(n = 385)为4%,晚期疾病(n = 56)为16%(P = 0.02)。HT的中位时间为48个月(范围4 - 139个月)。HT后5年无进展生存(PFS)率和总生存(OS)率分别为80%和94%。DLBCL的免疫组化结果如下:生发中心B细胞(GCB)/非GCB,37%/63%;CD10,9%;BCL6,59%;MUM1,38%;MYC,42%;BCL2,35%;Ki67≥90%,23%;CD5,3%。大多数(75%,9/12)GCB型DLBCL表现为CD10(-)、BCL6(+)和MUM1(-)免疫表型;其余具有CD10(+)免疫表型。多变量分析显示,仅HT时的晚期是PFS的显著不良因素(P = 0.037)。因此,HT的总体风险较低,HT后的预后良好;然而,在晚期病例中,HT的风险相对较高且预后不良。

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