造血和淋巴组织肿瘤WHO分类形态学诊断——附571例应用体会

The morphological diagnosis of tumours of the hematopoietic and lymphoidtissue on WHO classification——the application experiences in 571 cases

目的介绍造血和淋巴组织肿瘤WHO分类的应用体会,促进在我国的应用和普及。方法用血片,骨髓涂片、印片和切片(简称四片联检)加细胞免疫化学染色进行形态学诊断。结果按WHO的基本要求,近四年来诊断髓系肿瘤370例,淋系肿瘤201例。应用体会之一,开展四片联检,可显著提高WHO的形态学诊断水平,尤其是慢性骨髓增殖性疾病(MPD)和骨髓增生异常-MPD(MD-MPD)及伴骨髓纤维化的造血和淋巴组织肿瘤。体会之二,WHO的AML五分类中,第一类重现性染色体异常AML,形态学典型的M3、M2(原始细胞较大伴成熟特征或M2b)、M4Eo和M5,可提示这一类型并建议(分子)遗传学检查;第二类伴多系病态造血和第三类治疗相关AML,结合临床可以作出诊断;第四类不另作分类AML(M0、M1、M6和M7,以及不包括前述三类的M2、M4和M5),即仍按FAB诊断,还有形态学诊断的急性嗜碱粒细胞白血病、全髓增殖症伴骨髓纤维化和髓系肉瘤;第五类系列未明或双系AML,为形态学结合免疫表型诊断。体会之三,WHO分类的MDS八型,除5q-综合征外,以形态学为主要诊断,有核细胞量、原始细胞量和病态造血程度的评估是诊断和鉴别的根本指标。体会之四,WHO分类的MPD和MD-MPD,主要为形态学诊断,外周血细胞增多和骨髓增殖是MPD评估的最重要异常,骨髓增殖加病态造血是诊断MD-MPD的最有力证据。体会之五,ALL形态学诊断(尤其L1和L2)中,需要开展免疫化学染色,以区分B、T细胞;成熟B细胞肿瘤主要是形态学诊断。结论以WHO理念,摆正形态学与其他检查在诊断中的位置,按条件应用WHO分类的深度和广度,提高诊断水平以及对治疗和预后的评估力。

Objective To introduce our application experiences in the hematopoietic and lymphoid tissue tumours on WHO classification,to accelerate application and popularization of the WHO classification in our country.Methods Morphological diagnosis by examination of blood films,bone marrow smears,imprints and sections(called "four slides").Results According to the essential requests of WHO,we had diagnosed myeloid neoplasms 370 cases and lymphoid neoplasms 201 cases at recent 4 years,and summarized our application experience as follows.Firstly,to apply with combined examination of "four slides" can obviously improve the level of morphological diagnosis,especially the tumours of hematopoietic and lymphoid tissue with myelofibrosis,chronic myeloproliferative diseases(MPD)and myelodysplastic /myeloproliferative diseases(MD-MPD).Secondly,in the five categories of WHO classification to AML,the first category associates with the recurrent chromosome abnormalities,which include M3,M2(large blasts with maturation character or M2b),M4Eo and M5 with typical morphology,and suggests molecular genetics examination;the second category of AML with multilineage dysplasia and the third category of therapy-related AML,which can be diagnosed by morphology combining with clinic;the fourth category of AML not otherwise categorized,including M0,M1,M6,M7 which are diagnosed as FAB classification(including M2,M4,M5 which are not classified in the former three categories),and containing the acute basophilic leukemia,acute panmyelosis with myelofibrosis and myeloid sarcoma which are only diagnosed by morphology;the fifth category of AML of ambiguous lineage can be mainly diagnosed by immunophenotype combined with morphology.Thirdly,the MDS encompasses eight categories by WHO classification,except 5q-syndrome,the rest are diagnosed by morphology;the accurated evaluation of bone marrow cellularity,blasts and dysplasia are the most fundamental guide line in MDS diagnosis.Fourthly,WHO classification of MPD and MD-MPD are mainly diagnosed by morphology;periferal hematocytosis and myeloproliferation are the most important guide line in morphology evaluation of MPD,myeloproliferation and dysplasia are the most powerful morphology evidence in diagnosing MD-MPD.Fifthly,in the morphological diagnosis of ALL(especially L1 and L2),we should carry out immunocytochemistry staining in order to separate B from T cell ALL;mature B-cell neoplasms are diagnosed by morphology in the majority.Conclusion According to the WHO theory,setting the right position of morphology and other examination in diagnosis,depending on the condition of different laboratory to apply with the depth and extent of WHO classification,will upgrade diagnosic level and improve the evaluation of therapy and prognosis.