Update on the World Health Organization classification of peripheral T-cell lymphomas.
Update on the World Health Organization classification of peripheral T-cell lymphomas.
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DOI：
10.1007/s11899-009-0030-5
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发表时间：
2009-10-01
影响因子：
2.9
通讯作者：
Savage, Kerry J
中科院分区：
文献类型：
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作者：
O'Leary, Hilary M;Savage, Kerry J
外周T细胞淋巴瘤(PTCL)是一种罕见且异质性的胸腺后淋巴增生性疾病。在阐明这些肿瘤的病理生物学和适当的治疗方面进展缓慢，主要是因为它们很罕见，但也因为直到20世纪90年代初，它们通常被归类在一起并与B细胞淋巴瘤合并。现在人们了解到，大多数PTCL侵袭性很强，对标准化疗的反应很差，因此他们的预后比B细胞同行差得多。1994年，欧美修订的淋巴瘤分类提供了第一个统一的系统，根据形态、表型、遗传学和临床特征对淋巴增生性疾病进行分类。从那时起，随着更多信息的发展，世界卫生组织(WHO)完善了这一系统，以进一步阐明这些肿瘤的来源。最近，在世卫组织最新分类中定义了几个新的不同和临时类别。本文综述了世界卫生组织第四版淋巴系统肿瘤分类的变化，特别是侵袭性亚型。
Peripheral T-cell lymphomas (PTCLs) are a rare and heterogeneous group of lymphoproliferative disorders of postthymic origin. Progress in elucidating the pathobiology and appropriate therapy of these neoplasms has been slow, primarily because of their rarity, but also because until the early 1990s, they were generally grouped together and combined with B-cell lymphomas. It is now understood that most PTCLs are highly aggressive and respond poorly to standard chemo therapy, and thus they have a significantly poorer prognosis than their B-cell counterparts. In 1994, the Revised European and American Lymphoma classification provided the first uniform system to classify lymphoproliferative disorders on the basis of morphology, phenotype, genetics, and clinical features. Since then, the World Health Organization (WHO) has refined this system as additional information has evolved to further elucidate the origin of these neoplasms. More recently, several new distinct and provisional categories in the updated WHO classification have been defined. This review summarizes the changes in the fourth edition of the WHO classification of lymphoid tumors, with particular focus on the aggressive subtypes.