原发性皮肤弥漫性大 B 细胞淋巴瘤中的双表达和双/三打击状态：腿型与非特指亚型的比较。
Double expressor and double/triple hit status among primary cutaneous diffuse large B-cell lymphoma: a comparison between leg type and not otherwise specified subtypes.
机构信息
Anatomic Pathology, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, 2700, Italy.
Anatomic Pathology, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, 2700, Italy.
出版信息
Hum Pathol. 2021 May;111:1-9. doi: 10.1016/j.humpath.2021.01.006. Epub 2021 Feb 3.
Primary cutaneous diffuse large B-cell lymphomas (pcDLBCLs) are rare hematological neoplasms. The pcDLBCL category includes primary cutaneous large B-cell lymphoma leg type (pcDLBCL-LT), characterized by a particularly unfavorable outcome, and primary cutaneous large B-cell lymphoma not otherwise specified (pcDLBCL-NOS), a widely debated subentity with a more indolent course. The negative prognostic impact of double expressor status (DE status, given by coexpression of MYC and BCL2) and double hit/triple hit status (DH/TH status, given by translocations of MYC and BCL2 and/or BCL6) in nodal DLBCL is well known; however, no unanimous conclusions regarding relevance of DE and DH/TH status have been reached in pcDLBCL. Therefore, our purpose has been to investigate the presence and prognostic relevance of DE and DH/TH status among a retrospective multicentric cohort of 16 cases of pcDLBCL-LT and 17 cases of pcDLBCL-NOS. All cases were thoroughly reevaluated, both on a morphological and immunohistochemical level, and tested by means of fluorescence in situ hybridization for MYC, BCL2 and BCL6 rearrangements. DE status was observed in 69% of pcDLBCL-LT cases and in 24% of pcDLBCL-NOS cases; however, it did not impact prognosis in any of the groups examined. Combining molecular results, we highlighted a relevant fraction of DH pcDLBCL cases (three pcDLBCL-LT cases and one pcDLBCL-NOS case) and the very first case of TH pcDLBCL-LT reported to date. All DH cases were characterized by MYC and BCL6 rearrangements. Overall, DH/TH cases represented 15% (5/33) of all pcDLBCLs and were mostly pcDLBCL-LT. DH/TH status and DH status alone were associated with poorer overall survival and disease-specific survival (both p < 0.05) among all pcDLBCLs, without reaching statistical significance in the pcDLBCL-LT and pcDLBCL-NOS groups. In conclusion, MYC, BCL2, and BCL6 cytogenetical testing could be useful in identifying a putative subset of more aggressive pcDLBCLs, although this observation has to be confirmed by further studies.
原发性皮肤弥漫性大 B 细胞淋巴瘤（pcDLBCL）是一种罕见的血液系统肿瘤。pcDLBCL 包括原发性皮肤大 B 细胞淋巴瘤腿型（pcDLBCL-LT），其预后特别差，以及未特指的原发性皮肤大 B 细胞淋巴瘤（pcDLBCL-NOS），这是一种具有更惰性病程的广泛争论的亚实体。在结内 DLBCL 中，双表达状态（DE 状态，由 MYC 和 BCL2 的共表达表示）和双打击/三打击状态（DH/TH 状态，由 MYC 和 BCL2 及/或 BCL6 的易位表示）的阴性预后影响是众所周知的；然而，在 pcDLBCL 中，关于 DE 和 DH/TH 状态相关性的一致结论尚未达成。因此，我们的目的是在 16 例原发性皮肤弥漫性大 B 细胞淋巴瘤腿型（pcDLBCL-LT）和 17 例原发性皮肤弥漫性大 B 细胞淋巴瘤非特指型（pcDLBCL-NOS）的回顾性多中心队列中研究 DE 和 DH/TH 状态的存在及其预后相关性。所有病例均进行了彻底的重新评估，包括形态学和免疫组织化学水平，并通过荧光原位杂交检测 MYC、BCL2 和 BCL6 重排。pcDLBCL-LT 病例中有 69%存在 DE 状态，pcDLBCL-NOS 病例中有 24%存在 DE 状态；然而，在任何检查的组中，它都没有影响预后。结合分子结果，我们突出了具有相关 DH pcDLBCL 病例的比例（3 例 pcDLBCL-LT 病例和 1 例 pcDLBCL-NOS 病例），以及迄今为止报告的首例 TH pcDLBCL-LT 病例。所有 DH 病例均表现为 MYC 和 BCL6 易位。总体而言，DH/TH 病例占所有 pcDLBCL 的 15%（33 例中的 5 例），且主要为 pcDLBCL-LT。DH/TH 状态和 DH 状态单独与所有 pcDLBCL 的总生存和疾病特异性生存较差相关（均 p<0.05），但在 pcDLBCL-LT 和 pcDLBCL-NOS 组中未达到统计学意义。总之，MYC、BCL2 和 BCL6 细胞遗传学检测可用于识别更具侵袭性的 pcDLBCL 的潜在亚群，尽管这一观察结果需要进一步研究证实。
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