2008年版“世界卫生组织造血及淋巴组织肿瘤分类”指要

The point of WHO classification of tumors of hematopoietic and lymphoid tissues（2008）

2008年第4版“世界卫生组织造血及淋巴组织肿瘤分类”把该类肿瘤分列为12个项目，对分子生物学进展结合较多，基因、染色体改变均加入分类中。慢性骨髓增生性疾病改为骨髓增生性肿瘤，并将肥大细胞增多症（mastocytosis）归于此栏中。新增了伴有嗜酸细胞增多的髓系及淋巴细胞系恶性肿瘤及异常的PDDFRA、PDDFRB或FGFR1基因一栏。在骨髓增生异常综合征／骨髓增生性肿瘤（MDS／MPN）一栏中增加了伴环形铁粒幼细胞再生障碍性贫血并显著的血小板增多（RARS—T）（可能为一单独性疾病）。在骨髓增生异常综合征（MDS）一栏中分为一系或多系增生异常伴有一系或多系血细胞减少，并增添了儿童MDS。在急性髓系白血病及其有关前体细胞恶性肿瘤中，把髓系肉瘤（myeloid sarcoma）单独分类；新增加了唐氏综合征（Down syndrome）伴有的髓系增生疾病；新增加了母细胞性浆细胞样树突细胞肿瘤（blastic plasmacytoid dendretic cell neoplasm）。把系列不明的白血病（acute leukemia of ambiguous lineage）单列为一个项目，含6种白血病。在前体淋巴系肿瘤中分为B系及T系，淋巴母细胞性白血病／淋巴母细胞性淋巴瘤，其区别点在于骨髓中淋巴母细胞〉25％，则诊断为淋巴细胞白血病[和急性髓系白血病（AML）不同，不设下限为20％]。成熟B淋巴细胞系肿瘤分为39种，包括慢性淋巴细胞白血病、骨髓瘤、重链病、Burkitt淋巴瘤等，但不包括移植后淋巴细胞增生性疾病（PTLD）。成熟T淋巴细胞系和NK细胞系肿瘤栏目下列举了22种成熟T淋巴细胞系和NK细胞系恶性肿瘤。霍奇金淋巴瘤栏目下列举了6种霍奇金淋巴瘤。组织细胞和树突状细胞恶性肿瘤包括7种恶性肿瘤及播散性幼年型黄肉芽肿。移植后淋巴细胞增生性疾病（PTLD）被单独分类在一个栏目中，又分为5种类型。

In introduced the 4th edition （2008） of WHO classification of tumors of haematopoietic and lymphoid tissues. They are divided into 12-columns, which includes myeloproliferative neoplasms; myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDDFRA, PDDFRB or FGFR1; myelodysplastic/myeloproliferative neoplasms; myelodysplastic syndromes; acute myeloid leukemia （AML） and related precursor neoplasms; acute leukemia of ambiguous lineage; precursor lymphoid neoplasms; mature B- cell neoplasms; mature T-celt and NK-cell neoplasms; Hodgkin lymphoma; histocytic and dendritic cell neoplasms; post-transplant lymphoproliferative disorders （PTLD）. Accrodiug to the progression of the molecular biology, the new classifications combines a lot of the new knowledge with geue, chromosome, immunophenotypes, morphology, histocytology et al. The chronic myeloproliferative disorders changes into myeloproliferative neoplasms（MPN）, mastocytosis included in this column of MPN. There are three subdivisions in the column of myeloid and lymphoid neoplasms with eosinophilia and abnormalities of genes. Refractory anemia with ring sideroblasts associated with marked thrombocytosis（RARS-T） in the column of MDS/MPN. In the column of MDS, MDS in children is added. Myeloid sarcoma, Down syndrome with myeloid proliferation and blastic plasmacytoid dendritic cell neoplasm were added in the column of AML and related precursor neoplasms. Acute leukemia of ambiguous lineage includes 6 kinds neoplasms. Precursor lymphoid neoplasms （B ＆ T ceils） includes leukemias and lymphomas, the bone marrow lymphoblasts 〉 25 % is defined as leukemia （no lower limit 20 % as AML）. Mature B-cell （39 neoplasms） , T/NK cell neoplasms （22 neoplasms）, Hodgkin lymphoma （6 neoplasms）, histocytic and dendritic cell neoplasms （7 neoplasms）. Post transplant lymphoproliferative disorders （PTLD） forms a new columns （5 neoplasms）.