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Triple-hit B-cell Lymphoma With MYC, BCL2, and BCL6 Translocations/Rearrangements: Clinicopathologic Features of 11 Cases.

有MYC、BCL2和BCL6易位/重排的三重打击B细胞淋巴瘤:11例临床病理特点

Wang W,Hu S,Lu X,Young KH,Medeiros LJ

Abstract

Lymphomas with translocations/rearrangements of MYC, BCL2, and BCL6, so-called triple-hit B-cell lymphoma, are rare, and few studies on these tumors are available in the literature. We report 11 cases of triple-hit B-cell lymphoma and characterize their clinicopathologic findings. All patients were men, with a median age of 64 years (range, 45 to 80 y), and 4 patients had antecedent or concurrent follicular lymphoma. Using the 2008 World Health Organization classification, these cases were classified as: 5 B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma; 4 DLBCL; 1 DLBCL with concurrent follicular lymphoma; and 1 low-grade follicular lymphoma. All cases were positive for CD10, BCL2, and FOXP1. Ten of 11 cases were positive for CD20. MYC expression was high in 10/11 (91%), BCL6 was positive in 8/11 (73%), and MUM1/IRF4 was positive in 6/11 (55%) cases. T-cell antigens, TdT, and Epstein-Barr virus-encoded RNA were negative in all cases. Ten of 11 cases showed a high proliferation index-70% to 100%, and the follicular lymphoma had a 30% proliferation rate. Using most algorithms, all cases belonged to germinal center B-cell-like group. All patients received standard or more aggressive immunochemotherapy regimens. Three patients had no response to chemotherapy; 4 patients showed a partial response; 2 patients had complete remission after chemotherapy; and 2 patients had just begun chemotherapy. Three patients underwent a stem cell transplant. The median follow-up time was 5.3 months. Five patients died, and 6 patients were alive at last follow-up. Two patients who underwent stem cell transplant after complete response to chemotherapy were in remission with 16 to 19 months of clinical follow-up. In summary, triple-hit lymphomas are clinically aggressive tumors associated with a poor prognosis. Patients often respond poorly to chemotherapy, but a subset may completely respond to chemotherapy followed by stem cell transplant.

摘要

具有MYC、BCL2和 BCL6易位/重排的淋巴瘤即所谓的三重打击B细胞淋巴瘤少见,并且该肿瘤的相关文献报道较少。我们报道了11例三重打击B细胞淋巴瘤以及它们的临床病理特点。患者均为男性,中位年龄64岁(45-80岁),4例有滤泡性淋巴瘤前驱病变或同时出现。依据2008年WHO分类,这些肿瘤分为:5例B细胞淋巴瘤,未分类,具有介于弥漫大B细胞淋巴瘤(DLBCL)和Burkitt淋巴瘤之间的特点;4例DLBCL;1例 DLBCL伴滤泡淋巴瘤;1例低级别滤泡淋巴瘤。全部病例均表达CD10、BCL2和FOXP1。10/11表达CD20。10/11 (91%)表达MYC,8/11 (73%)表达BCL6,6/11 (55%)表达MUM1/IRF4。全部病例均不表达T细胞谱系抗原、TdT和Epstein-Barr病毒编码RNA。10/11病例增殖指数高,为70% --100%,并且滤泡性淋巴瘤增殖率30%。全部病例均属于生发中心B细胞样组。患者均经标准或更为激进的免疫化疗,3例对化疗无效,4例部分有效,2例化疗后完全消退,2例刚刚开始化疗。3例行干细胞移植。中位随访时间为5.3个月。5例患者死亡,6例在最后随访时健在。2例患者在化疗完全应答后行干细胞移植后缓解,临床随访时间16--19个月。总之,三重打击淋巴瘤为临床侵袭性肿瘤,预后差。患者通常对化疗反应差,但有一部分可以在化疗继之以干细胞移植后完全反应。
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