Ohshima Koichi, Suzumiya Junji, Kikuchi Masahiro
Department of Pathology, School of Medicine, Fukuoka University, Fukuoka, Japan.
Pathol Int. 2002 Jan;52(1):1-12. doi: 10.1046/j.1440-1827.2002.01308.x.
New insights into the pathogenesis of lymphoid malignancies have been gained through novel genetic, molecular and immunological techniques. A new classification system for lymphoid malignancies, known as the new World Health Organization (WHO) classification, has been proposed recently based on these findings. The relative incidence of the subtypes of malignant lymphoma is known to differ according to geographic location. Adult T-cell leukemia/lymphoma (ATLL) is a human malignancy associated with human T-cell leukemia virus type 1 (HTLV-1), and the Kyushu islands are an HTLV-1 endemic area. To clarify the relationship between the histological classification and prognosis of lymphoid malignancies, we reclassified previous cases in our department and summarized our previous reports using the WHO classification. Of 933 cases of lymphoid malignancies, 471 (50%) were B-cell lymphoma, 396 (42%) T/natural killer (NK)-cell lymphoma and 41 (4%) Hodgkin lymphoma (HL). Analysis of clinical outcome showed favorable prognosis for HL, intermediate for B-cell lymphoma and poor prognosis for T-cell lymphoma. Among B-cell lymphomas, the commonest type was diffuse large B-cell lymphoma (n = 281; 60%). Marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) was diagnosed in 82 cases (17%), follicular lymphoma in 52 (11%) and mantle cell lymphoma in 24 (5%). Other less common lymphomas were Burkitt lymphoma (n = 9; 2%) and lymphoblastic lymphoma (n = 5; 1%). Using overall survival rates, the various B-cell lymphoma types could be divided into three broad groups for prognostic purposes: (i) low-risk group comprising follicular lymphoma and MALT; (ii) intermediate-risk group comprising diffuse large B-cell lymphoma and Burkitt lymphoma; and (iii) high-risk group comprising mantle cell lymphoma and lymphoblastic lymphoma. Among the T/NK-cell lymphomas, the commonest type was ATLL (n = 191; 48%), followed by peripheral T-cell lymphoma, unspecified (n = 83; 21%), angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) (n = 38; 10%), anaplastic large cell lymphoma (ALCL) (n = 22; 6%). Less common types were lymphoblastic lymphoma (n = 17; 4%), nasal and nasal-type NK/T-cell lymphoma (n = 17; 4%), mycosis fungoides (MF) (n = 9; 2%) and other rare types. With respect to clinical prognosis, T/NK-cell lymphomas fell into three groups: (i) relative low-risk group comprising ALCL, AILD, MF and lymphoblastic lymphoma; (ii) relative intermediate-risk group comprising NK/T-cell lymphoma and unspecified lymphoma; and (iii) extremely high-risk group comprising ATLL. Among the lymphoblastic lymphomas, B-cell type and T-cell type lymphomas exhibited different clinical outcomes. We conclude that the histological, phenotypic and genotypic classification of the new WHO system should be beneficial for the clinical approach to these tumors.
通过新颖的遗传、分子和免疫技术，人们对淋巴系统恶性肿瘤的发病机制有了新的认识。基于这些发现，最近提出了一种新的淋巴系统恶性肿瘤分类系统，即世界卫生组织（WHO）新分类。已知恶性淋巴瘤各亚型的相对发病率因地理位置而异。成人T细胞白血病/淋巴瘤（ATLL）是一种与1型人类T细胞白血病病毒（HTLV-1）相关的人类恶性肿瘤，九州岛是HTLV-1的流行地区。为了阐明淋巴系统恶性肿瘤的组织学分类与预后之间的关系，我们对本部门之前的病例进行了重新分类，并使用WHO分类总结了我们之前的报告。在933例淋巴系统恶性肿瘤病例中，471例（50%）为B细胞淋巴瘤，396例（42%）为T/自然杀伤（NK）细胞淋巴瘤，41例（4%）为霍奇金淋巴瘤（HL）。临床结果分析显示，HL预后良好，B细胞淋巴瘤预后中等，T细胞淋巴瘤预后较差。在B细胞淋巴瘤中，最常见的类型是弥漫性大B细胞淋巴瘤（n = 281；60%）。黏膜相关淋巴组织（MALT）边缘区淋巴瘤82例（17%），滤泡性淋巴瘤52例（11%），套细胞淋巴瘤24例（5%）。其他较罕见的淋巴瘤包括伯基特淋巴瘤（n = 9；2%）和淋巴母细胞淋巴瘤（n = 5；1%）。为了预后目的，根据总生存率，各种B细胞淋巴瘤类型可分为三大组：（i）低风险组，包括滤泡性淋巴瘤和MALT；（ii）中等风险组，包括弥漫性大B细胞淋巴瘤和伯基特淋巴瘤；（iii）高风险组，包括套细胞淋巴瘤和淋巴母细胞淋巴瘤。在T/NK细胞淋巴瘤中，最常见的类型是ATLL（n = 191；48%），其次是外周T细胞淋巴瘤，未特指型（n = 83；21%），血管免疫母细胞性淋巴结病伴蛋白异常血症（AILD）（n = 38；10%），间变性大细胞淋巴瘤（ALCL）（n = 22；6%）。较罕见的类型包括淋巴母细胞淋巴瘤（n = 17；4%），鼻及鼻型NK/T细胞淋巴瘤（n = 17；4%），蕈样霉菌病（MF）（n = 9；2%）及其他罕见类型。关于临床预后，T/NK细胞淋巴瘤分为三组：（i）相对低风险组，包括ALCL、AILD、MF和淋巴母细胞淋巴瘤；（ii）相对中等风险组，包括NK/T细胞淋巴瘤和未特指淋巴瘤；（iii）极高风险组，包括ATLL。在淋巴母细胞淋巴瘤中，B细胞型和T细胞型淋巴瘤表现出不同的临床结果。我们得出结论，WHO新系统的组织学、表型和基因型分类对这些肿瘤的临床处理应是有益的。
