Harris N L, Jaffe E S, Diebold J, Flandrin G, Muller-Hermelink H K, Vardiman J, Lister T A, Bloomfield C D
Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.
Hematol J. 2000;1(1):53-66. doi: 10.1038/sj.thj.6200013.
Since 1995, the European Association of Pathologists and the Society for Hematopathology have been developing a new World Health Organization (WHO) classification of hematologic malignancies. The classification includes lymphoid, myeloid, histiocytic, and mast cell neoplasms.
The WHO project involves ten committees of pathologists, who have developed lists and definitions of disease entities. A Clinical Advisory Committee (CAC) of international hematologists and oncologists was formed to ensure that the classification will be useful to clinicians. A meeting was held in November 1997 to discuss clinical issues related to the classification.
WHO has adopted the 'Revised European-American Classification of Lymphoid Neoplasms' (REAL), published in 1994 by the International Lymphoma Study Group (ILSG), as the classification of lymphoid neoplasms. This approach to classification is based on the principle that a classification is a list of 'real' disease entities, which are defined by a combination of morphology, immunophenotype, genetic features, and clinical features. The relative importance of each of these features varies among diseases, and there is no one 'gold standard'. The WHO classification has applied the principles of the REAL classification to myeloid and histiocytic neoplasms. The classification of myeloid neoplasms recognizes distinct entities defined by a combination of morphology and cytogenetic abnormalities. The CAC meeting, which was organized around a series of clinical questions, was able to reach a consensus on most of the questions posed. The questions and the consensus are discussed in detail below. Among other things, the CAC concluded that clinical groupings of lymphoid neoplasms were neither necessary nor desirable. Patient treatment is determined by the specific type of lymphoma, with the addition of grade within the tumor type, if applicable, and clinical prognostic factors such as the international prognostic index (IPI).
The experience of developing the WHO classification has produced a new and exciting degree of cooperation and communication between oncologists and pathologists from around the world, which should facilitate progress in the understanding and treatment of hematologic malignancies.
自1995年以来，欧洲病理学家协会和血液病理学协会一直在制定世界卫生组织（WHO）关于血液系统恶性肿瘤的新分类。该分类包括淋巴、髓系、组织细胞和肥大细胞肿瘤。
WHO项目涉及十个病理学家委员会，他们制定了疾病实体的列表和定义。成立了一个由国际血液学家和肿瘤学家组成的临床咨询委员会（CAC），以确保该分类对临床医生有用。1997年11月召开了一次会议，讨论与该分类相关的临床问题。
WHO采用了国际淋巴瘤研究组（ILSG）1994年发表的“欧美淋巴肿瘤修订分类”（REAL）作为淋巴肿瘤的分类。这种分类方法基于这样一个原则，即分类是一份“真实”疾病实体的列表，这些实体由形态学、免疫表型、遗传特征和临床特征的组合来定义。这些特征中每个特征的相对重要性因疾病而异，不存在单一的“金标准”。WHO分类已将REAL分类的原则应用于髓系和组织细胞肿瘤。髓系肿瘤的分类识别由形态学和细胞遗传学异常组合定义的不同实体。围绕一系列临床问题组织的CAC会议能够就提出的大多数问题达成共识。下面将详细讨论这些问题和共识。此外，CAC得出结论，淋巴肿瘤的临床分组既无必要也不可取。患者的治疗取决于淋巴瘤的具体类型，如有必要，还需加上肿瘤类型内的分级以及国际预后指数（IPI）等临床预后因素。
制定WHO分类的经验在全球肿瘤学家和病理学家之间产生了新的、令人兴奋的合作与交流程度，这应有助于在血液系统恶性肿瘤的理解和治疗方面取得进展。
