Department of Laboratory Medicine, University of California San Francisco, CA, USA.
Department of Laboratory Medicine, University of California San Francisco, CA, USA.
Pathology. 2021 Apr;53(3):349-366. doi: 10.1016/j.pathol.2021.01.002. Epub 2021 Mar 5.
The revised fourth edition of the World Health Organization (WHO) Classification of Tumours of Haematopoietic and Lymphoid Tissues (2017) reflects significant advances in understanding the biology, genetic basis and behaviour of haematopoietic neoplasms. This review focuses on some of the major changes in B-cell and T-cell non-Hodgkin lymphomas in the 2017 WHO and includes more recent updates. The 2017 WHO saw a shift towards conservatism in the classification of precursor lesions of small B-cell lymphomas such as monoclonal B-cell lymphocytosis, in situ follicular and in situ mantle cell neoplasms. With more widespread use of next generation sequencing (NGS), special entities within follicular lymphoma and mantle cell lymphoma were recognised with recurrent genetic aberrations and unique clinicopathological features. The diagnostic workup of lymphoplasmacytic lymphoma and hairy cell leukaemia has been refined with the discovery of MYD88 L265P and BRAF V600E mutations, respectively, in these entities. Recommendations in the immunohistochemical evaluation of diffuse large B-cell lymphoma include determining cell of origin and expression of MYC and BCL2, so called 'double-expressor' phenotype. EBV-positive large B-cell lymphoma of the elderly has been renamed to recognise its occurrence amongst a wider age group. EBV-positive mucocutaneous ulcer is a newly recognised entity with indolent clinical behaviour that occurs in the setting of immunosuppression. Two lymphomas with recurrent genetic aberrations are newly included provisional entities: Burkitt-like lymphoma with 11q aberration and large B-cell lymphoma with IRF4 rearrangement. Aggressive B-cell lymphomas with MYC, BCL2 and/or BCL6 rearrangements, so called 'double-hit/triple-hit' lymphomas are now a distinct entity. Much progress has been made in understanding intestinal T-cell lymphomas. Enteropathy-associated T-cell lymphoma, type II, is now known to not be associated with coeliac disease and is hence renamed monomorphic epitheliotropic T-cell lymphoma. An indolent clonal T-cell lymphoproliferative disorder of the GI tract is a newly included provisional entity. Angioimmunoblastic T-cell lymphoma and nodal T-cell lymphomas with T-follicular helper phenotype are included in a single broad category, emphasising their shared genetic and phenotypic features. Anaplastic large cell lymphoma, ALK- is upgraded to a definitive entity with subsets carrying recurrent rearrangements in DUSP22 or TP63. Breast implant-associated anaplastic large cell lymphoma is a new provisional entity with indolent behaviour. Finally, cutaneous T-cell proliferations include a new provisional entity, primary cutaneous acral CD8-positive T-cell lymphoma, and reclassification of primary small/medium CD4-positive T-cell lymphoma as lymphoproliferative disorder.
世界卫生组织(世卫组织)造血和淋巴组织肿瘤分类的第四版修订本(2017 年)反映了在理解造血肿瘤的生物学、遗传基础和行为方面的重大进展。本综述重点介绍了 2017 年世卫组织中非霍奇金淋巴瘤中 B 细胞和 T 细胞的一些重大变化,并包括了最近的更新。2017 年世卫组织在小 B 细胞淋巴瘤如单克隆 B 细胞淋巴增生症、滤泡内和套细胞内肿瘤的前体病变的分类中向保守主义转变。随着下一代测序(NGS)的广泛应用,在滤泡性淋巴瘤和套细胞淋巴瘤中发现了具有复发性遗传异常和独特临床病理特征的特殊实体。随着 MYD88 L265P 和 BRAF V600E 突变的发现,淋巴浆细胞淋巴瘤和毛细胞白血病的诊断工作得到了改进。弥漫性大 B 细胞淋巴瘤的免疫组织化学评估建议包括确定细胞起源和 MYC 和 BCL2 的表达,即所谓的“双表达”表型。EBV 阳性老年人弥漫性大 B 细胞淋巴瘤已更名为认识到它发生在更广泛的年龄组。EBV 阳性粘膜溃疡是一种新认识的具有惰性临床行为的实体,发生在免疫抑制的情况下。两种具有复发性遗传异常的淋巴瘤是新的暂定实体:具有 11q 异常的 Burkitt 样淋巴瘤和具有 IRF4 重排的大 B 细胞淋巴瘤。具有 MYC、BCL2 和/或 BCL6 重排的侵袭性 B 细胞淋巴瘤,即所谓的“双打击/三打击”淋巴瘤,现在是一个明确的实体。在理解肠道 T 细胞淋巴瘤方面取得了很大进展。肠病相关 T 细胞淋巴瘤,II 型,现在已知与乳糜泻无关,因此更名为单形上皮嗜性 T 细胞淋巴瘤。一种新的暂定实体是胃肠道克隆性 T 细胞淋巴增生性疾病。血管免疫母细胞性 T 细胞淋巴瘤和具有 T 滤泡辅助表型的结内 T 细胞淋巴瘤被归入一个广泛的类别,强调了它们共同的遗传和表型特征。间变性大细胞淋巴瘤,ALK- 被升级为一个明确的实体,亚组具有 DUSP22 或 TP63 重复重排。乳房植入物相关间变性大细胞淋巴瘤是一种新的暂定实体,具有惰性行为。最后,皮肤 T 细胞增生包括一个新的暂定实体,原发性皮肤肢端 CD8 阳性 T 细胞淋巴瘤,以及原发性小/中 CD4 阳性 T 细胞淋巴瘤作为淋巴增生性疾病的重新分类。
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