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霍奇金淋巴瘤:病理学家的观点。

Hodgkin's lymphoma: the pathologist's viewpoint.

作者信息

Pileri S A, Ascani S, Leoncini L, Sabattini E, Zinzani P L, Piccaluga P P, Pileri A, Giunti M, Falini B, Bolis G B, Stein H

机构信息

Pathologic Anatomy and Haematopathology, Bologna University, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy.

出版信息

J Clin Pathol. 2002 Mar;55(3):162-76. doi: 10.1136/jcp.55.3.162.

Abstract

Despite its well known histological and clinical features, Hodgkin's lymphoma (HL) has recently been the object of intense research activity, leading to a better understanding of its phenotype, molecular characteristics, histogenesis, and possible mechanisms of lymphomagenesis. There is complete consensus on the B cell derivation of the tumour in most cases, and on the relevance of Epstein-Barr virus infection and defective cytokinesis in at least a proportion of patients. The REAL/WHO classification recognises a basic distinction between lymphocyte predominance HL (LP-HL) and classic HL (CHL), reflecting the differences in clinical presentation and behaviour, morphology, phenotype, and molecular features. CHL has been classified into four subtypes: lymphocyte rich, nodular sclerosing, with mixed cellularity, and lymphocyte depleted. The borders between CHL and anaplastic large cell lymphoma have become sharper, whereas those between LP-HL and T cell rich B cell lymphoma remain ill defined. Treatments adjusted to the pathobiological characteristics of the tumour in at risk patients have been proposed and are on the way to being applied.

摘要

尽管霍奇金淋巴瘤(HL)具有广为人知的组织学和临床特征,但它最近仍是深入研究的对象,这使得人们对其表型、分子特征、组织发生以及淋巴瘤发生的可能机制有了更好的理解。在大多数情况下,对于肿瘤的B细胞起源以及至少一部分患者中爱泼斯坦-巴尔病毒感染和细胞分裂缺陷的相关性已达成完全共识。REAL/WHO分类法认识到淋巴细胞为主型HL(LP-HL)和经典HL(CHL)之间的基本区别,这反映了临床表现和行为、形态、表型及分子特征方面的差异。CHL已被分为四种亚型:富于淋巴细胞型、结节硬化型、混合细胞型和淋巴细胞消减型。CHL与间变性大细胞淋巴瘤之间的界限变得更加清晰,而LP-HL与富于T细胞的B细胞淋巴瘤之间的界限仍不明确。针对高危患者肿瘤病理生物学特征调整的治疗方法已被提出并正在应用中。

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