骨髓增生异常综合征FAB和WHO分型的临床评价

目的:评价和分析骨髓增生异常综合征(MDS)从FAB分型到WHO分型的发展和临床意义。方法:对MDS患者分别用FAB分型及WHO分型进行分型,并对形态学、临床、实验室检查及预后资料进行对比分析。结果:MDS和急性髓性白血病(AML)均可出现病态造血。FAB分型中难治性贫血(RA)、原始细胞过多难治性贫血(RAEB)、转化中的原始细胞过多难治性贫血(RAEB-T)及AML之间生存率差异有统计学意义。WHO分型中RA与难治性血细胞减少伴多系增生异常(RCMD)之间生存率差异无统计学意义,RA与RAEB、RCMD与RAEB之间生存率差异有统计学意义,RAEB-Ⅰ与RAEB-Ⅱ之间生存率有显著差异。结论:WHO分型将FAB分型中的RA分为RA和RCMD并未显示出临床优越性。RAEB-T生存期比AML更短,因而将RAEB- T归为急性白血病,对临床治疗有好处。WHO分型按照原始细胞百分比将RAEB分为RAEB-Ⅰ和RAEB-Ⅱ,对临床诊断、治疗和预后有益。     

32例骨髓增生异常综合征临床治疗分析

<正>骨髓增生异常综合征(MDS)是一组起源于造血干细胞,以血细胞病态造血、高风险向急性髓系白血病(AML)转化为特征的难治性白细胞质、量异常的异质性疾病~([1])。法、美、英(FAB)协作组将MDS分为难治性贫血(RA)、环形铁粒幼细胞性难治性贫血(RAS)、难治性贫血伴原始细胞增多(RAEB)、难治性贫血伴原始细胞增多转化型(RAEB-T)及慢性粒-单核细     

原发骨髓增生异常综合征患者IPSS及染色体核型的临床分析

目的:比较原发性骨髓增生异常综合征(MDS)患者WHO(2001)分型与FAB分型的IPSS染色体核型分析及预后的相关性分析。方法:经FAB标准确诊的原发MDS的患者重新按WHO标准分型,对2种结果的IPSS及染色体异常与各亚型的关系进行分析。结果:按FAB分型各亚型的IPSS及染色体异常无显著性差异,按WHO分型的难治性细胞减少伴多系增生异常(RCMD)与难治性贫血(RA)患者染色体异常率有统计学意义(66.6%,41.7%,P<0.01),RAEB-2高危组比例明显高于RAEB-1组(25%,0%,P<0.01)。结论:原发MDS的WHO分型与FAB分型相比,前者与预后的相关性更好。     

难治性血细胞减少伴多系发育异常患者96例临床和实验室特征分析

骨髓增生异常综合征 (MDS)是一组异质性造血干细胞克隆性疾病。FAB分型标准中提出难治性贫血(RA)的形态学异常常仅限于红系,但形态学异常累及 2 ~3系血细胞的RA情况相当常见,而且预后与仅累及红系的RA有所不同,因此,在新近提出的WHO分型标准中将同时有粒系和 (或 )巨核系发     

结合WHO诊断标准浅谈骨髓细胞形态学在骨髓增生异常综合征诊断中的意义

按照WHO标准,主要依靠外周血细胞减少、细胞形态学(骨髓细胞病态造血、环形铁粒幼红细胞计数、原始细胞比例)、细胞遗传学核型分析,对骨髓增生异常综合征(MDS)进行诊断和分型。重要的辅助诊断包括骨髓病理活检或(和)免疫组织化学、流式细胞免疫表型和MDS相关基因突变的检测。病态造血是MDS诊断分型的形态学基础,原始细胞不增多的MDS主要依靠病态造血进行诊断分型。病态造血巨核细胞≥10%或环状铁粒幼红细胞≥15%,对MDS具有较确定性的诊断意义。部分MDS-SLD及MDS-MLD,巨核细胞系无病态造血,仅有红细胞系和(或)粒细胞系病态造血细胞的比例≥10%,属于真正意义上的MDS最低诊断标准,在实际操作起来难以准确把握,诊断MDS时应慎重,并需排除反应性病态造血。该文详细介绍了细胞形态学对MDS的诊断意义,详细解读了各系病态造血的细胞形态学特征以及笔者的经验和体会,分析总结了MDS的诊断思路,阐述了MDS诊断中应注意的问题,对于MDS的精准诊断及防止误诊具有建设性意义。     

骨髓增生异常综合征分型和预后因素的探讨

骨髓增生异常综合征(MDS)是一组异质性造血组织干细胞性疾病,现就其分型和预后因素的研究作一简介。一、细胞形态学分类与预后的关系FAB 协作组根据造血细胞形态学变化将 MDS分为5型:(1)难治性贫血(RA);(2)难治性贫血伴环形铁粒幼细胞增多(RARS);(3)难治性贫血伴原始细胞增多(RAEB);(4)难治性贫血伴原始细胞     

骨髓增生异常综合征临床研究近况

骨髓增生异常综合征(MDS)是一组克隆性造血干细胞或骨髓祖细胞疾病状态谱,至少包括难治性贫血(RA)、铁粒幼细胞性贫血(RAS)、伴有原始细胞增多的RA(RAEB)、慢性粒单细胞白血病(CMML)及转化中的RAEB(RAEB-t)等多个类型。虽然各型的表现不尽相同,但骨髓增生异常、血细胞减少和向急性髓性白血病(AML)转化的危险性则是共同的.提出MDS这一概念的时间不长,由于受到国内外广泛重视,研究进展迅速.现就临床研究近状作简要介绍. 流行病学与病因学     

骨髓异常增生综合征:具有预后意义的记分法

作者报道按 FAB 标准分类的141例骨髓异常增生综合征(MDS)。根据细胞形态学将其分为5型:转化中的伴原始细胞过多的难治性贫血(RAEBT);伴原始细胞过多的难治性贫血(RAEB);慢性粒-单核细胞白血病(CMML);难治性贫血(RA);伴铁粒幼细胞的难治性贫血(RAS)。随访4～192个月。平     

骨髓增生异常综合征细胞遗传学与生存期的研究

WHO 2001年根据外周血象和骨髓象提出了骨髓增生异常综合征(MDS)新的分型:难治性贫血(RA),难治性贫血伴环形铁粒幼细胞(RAILS),难治性血细胞减少伴多系增生异常(RCMD),难治性贫血伴原始细胞增多1型(RAEBI),难治性贫血伴原始细胞增多2型(RAE82),未归类的MDS(MDS.U),5q-综合征[1].     

地西他滨为主方案治疗中高危骨髓增生异常综合征患者的病态造血变化及预后价值分析

目的:探讨以地西他滨为主方案治疗中高危骨髓增生异常综合征(MDS)患者的病态造血变化及其预后影响因素。方法:回顾性分析60例中高危MDS患者的病态造血特点及其预后的影响因素。结果:χ~2或Fisher精确检验显示,骨髓原始细胞数、WHO 2016诊断分型、红系病态造血有无减少与以地西他滨为主方案治疗的中高危MDS患者中达总有效相关(P0.05);Kaplan-Meier检验单因素分析显示,WHO 2016诊断分型、IPSS-R评分、年龄、骨髓原始细胞数、染色体有无-7、初诊有无粒系病态造血、粒系病态造血有无变化各组间疗效差异有统计学意义(P0.05);COX多因素分析显示,骨髓原始细胞数、染色体有无-7、粒系病态造血变化是地西他滨为主方案治疗中高危MDS预后的独立危险因素(均P0.05)。结论:在以地西他滨为主方案治疗中高危MDS的治疗过程中,关注骨髓原始细胞数、染色体有无-7和粒系病态造血变化对于评估预后具有重要的意义。     

The myelodysplastic syndromes: diagnosis, molecular biology and risk assessment

Myelodysplastic syndromes (MDS) are heterogeneous group of neoplastic clonal stem cell diseases characterized by dysplastic morphological features and clinical bone marrow failure. The FAB (French-American-British) system served as the gold standard for MDS classification for more than two decades. The WHO classification, built on the backbone of FAB classification, is an attempt to further improve the prognostic value of MDS classification as well as establish its clinical utility as a tool to select different treatments. In this article we review the epidemiology, pathogenesis, molecular biology, diagnosis and classification of MDS. We highlight the major differences between the FAB classification and the WHO MDS classification. We discuss in more detail the experience of using the new WHO classification since its publication and review the studies that tried to validate the prognostic value of the new classification or apply it to predict clinical responses to various treatments.     

The clinical implications of the World Health Organization's classification of myelodysplastic syndromes

Myelodysplastic syndromes (MDS) are a heterogeneous group of neoplastic clonal stem cell diseases characterized by dysplastic morphological features with a varying percentage of leukemic blasts and clinical bone marrow failure. The French-American-British (FAB) system served as the gold standard of MDS classification for more than two decades. The World Health Organization (WHO) classification, built on the backbone of the FAB classification, is an attempt to further improve the prognostic value of MDS classification as well as to establish its clinical utility as a tool to select different treatments. In this article we highlight the major differences between the FAB classification and the WHO MDS classification. We discuss in more details the experience of using the new WHO classification since its publications and review the studies that tried to either validate the prognostic value of the new classification or apply it to predict clinical responses to various treatments.     

Myelodysplastic syndromes according to FAB and WHO classification. Single center experience

The results of clinical and laboratory observations of 119 MDS patients divided acc. to FAB, and - after excluding RAEB-t and CMML groups -- of 95 patients divided accordingly to WHO classification are presented. The diagnosis of MDS was based on medical interview, physical examination, blood biochemistry, peripheral blood (PB) and bone marrow (BM) cytomorphology and cytochemistry, trephine biopsy and cytogenetic examination. All hematologic examinations were done according to routine methods. Cytogenetic analyses were carried out on BM cells from 24-48 h cultures in standard conditions. At least 15-20 GTG-banded metaphases were analyzed in every patient. The survival time (ST) of patients differed significantly between the FAB or WHO groups, with p=0.0004 for FAB and p=0.02 for WHO. The progression to AML was more common in less favorable groups, with p=0.0001 for FAB and p=0.00016 for WHO. The distribution of IPSS prognostic index among the groups showed statistically significant difference (p=0.0004 for FAB, and p=0.0001 for WHO), whereas the distribution of karyotypic abnormalities did not. However, in univariate analysis statistically significant influence on ST showed, beside the both classification systems: cytogenetics, the presence of blasts in PB, age and IPSS index. In multivariate analysis the sole independent prognostic factors were: PB blasts and cytogenetics. The authors conclude that the WHO classification offers a good prognostic tool for MDS patients. However, the karyotype and the presence of blasts in PB should always be taken into account.     

World Health Organization classification in combination with cytogenetic markers improves the prognostic stratification of patients with de novo primary myelodysplastic syndromes

This study correlated chromosomal defects with French-American-British (FAB)/World Health Organization (WHO) classification subtypes, proposed a revised International Prognostic Scoring System (IPSS) cytogenetic grouping; and established which classification, when used with the IPSS cytogenetic categories, best predicted clinical outcome in the myelodysplastic syndromes (MDS). A higher prevalence of chromosomal defects and distinct defects were observed in patients with multi-lineage dysplasia and a blast cell percentage >10%. Abnormalities of the long arm of chromosome 3, del(7)(q31q35), trisomy 8, del(11)(q14q23), del(12p) and 20q- could be segregated from their respective IPSS cytogenetic categories and used to develop new cytogenetic subgroups. Clinical parameters, FAB/WHO classification, IPSS score and standard or revised cytogenetic categories were statistically relevant for overall survival (OS) and progression-free intervals (PFI) and were included within five distinct multivariate models compared by the Akaike Information Criterion. To predict OS, the best models included age, WHO classification and standard or revised IPSS cytogenetic categories; to predict PFI, the best model included the same variables and revised cytogenetic categories. In conclusion, (i) the WHO classification was associated with a more homogeneous cytogenetic pattern than the FAB classification, (ii) WHO classification and standard/revised IPSS cytogenetic categories were much more effective than IPSS for predicting MDS clinical outcome, (iii) revised cytogenetic subgroups predicted PFI more effectively than standard categories.     

上海地区805例成人急性白血病世界卫生组织亚型的临床分析

目的 分析上海地区24家医院急性白血病(AL)世界卫生组织(WHO)亚型的分布,并与国外资料进行比较,探讨我国AL患者WHO亚型的分布特点.方法 收集连续样本,对中美联合上海市白血病协作组805例AL患者同时采用FAB和WHO分型标准进行分型诊断.结果 本组AL中急性髓系白血病(AML)77.4%(623/805),急性淋巴细胞白血病(ALL)20.4%(164/805),AML和ALL之比为3.8:1.伴有重现性细胞遗传学异常AML占36.4%,AML伴多系病态造血占17.7%,不属于上述分类的AML占45.9%.FAB分型中M4最多,占33.9%,M3和M4比例高于国外,M1比例低于国外报道.与上海市1984-1994年AL资料相比,M4亚型比例增多,M1和M5亚型比例减少.AML染色体异常率61.7%,AML伴t(15;17)比例高于国外,染色体预后良好组占30.1%,预后中等组占51.6%,预后不良组占18.3%,预后良好组比例高于国外.ALL中前体B细胞ALL占绝大多数(82.3%),ALL中t(9;22)占28.2%.结论 AML伴t(15;17)和M4比例高于国外,染色体预后良好组比例高于国外,AML的WHO分型和染色体异常类型与国外有所不同.与上海市1984-1994年AL资料相比,AML的亚型分布有所变化.     

Clinical, haematological and histomorphological profile of adult myelodysplastic syndrome. Study of 96 cases in a single institute

Myelodysplastic syndromes (MDS) are clonal haematopoietic stem cell disorders characterised by ineffective and dyspoietic haematopoiesis. The natural history of these disorders is variable and ranges from a chronic to a rapid course towards leukaemic progression. Certain shortcomings have been encountered in the French-American-British (FAB) classification over the years, and therefore there is a need for an alternative method of classification. In 1999, the WHO published a revised classification of MDS. In the present study, we have analysed the clinical, haematological and histomorphological features in 96 cases of primary MDS seen in the department of haematology at the All India Institute of Medical Sciences (AIIMS) over a 6-yr period (1996-2001). Both FAB and WHO classifications have been incorporated and the Bournemouth scoring system applied in each case at presentation. The Bournemouth scoring system, in the absence of a cytogenetic study, offers a good prognostication and long-term survival estimate.     

Immunophenotyping of acute myeloid leukemia by immuno-alkaline phosphatase (APAAP) labeling with a panel of antibodies

A panel of eight antibodies was used by the alkaline-phosphatase/anti-alkaline phosphatase (APAAP) method to stain peripheral blood films, bone marrow smears, and cytocentrifuge preparations from 29 cases of acute myeloid leukemia. These findings were correlated with the French-American-British (FAB) classification. Leukemic cells from six cases of myeloblastic leukemia (FAB;M1) were predominantly labeled by the antimyeloperoxidase monoclonal antibody (MPO-7). Leukemic cells from the majority of eight cases of myeloblastic leukemia with maturation (FAB;M2) and progranulocytic leukemia (FAB;M3) stained with monoclonal antibodies MPO-7, NP57 (anti-elastase), and EBM11 (antimonocyte/macrophage). Leukemic cells from six cases of myelomonocytic (FAB;M4) and five cases of monocytic (FAB;M5) leukemia were most often labeled with antibodies MPO-7, NP57, and EBM11 as well as monoclonal antibodies Y1/82A (anti-monocyte) and KB90 (against the p150, 95 molecule, CD11c; a monocyte/granulocyte marker), but not with monoclonal antibody C17 (antiglycoprotein IIb/IIIa) and/or monoclonal antibody Y2/51 (antiglycoprotein IIIa). Erythroblasts from a single case of erythroleukemia (FAB;M6) were not labeled with any of the antibodies from this panel. Leukemic cells from two cases of acute megakaryocytic leukemia (FAB;M7) stained strongly with the monoclonal antiglycoprotein IIIa/IIb antibody (C17) and antiglycoprotein IIIa antibody (Y2/51). Staining by the APAAP method with this panel of antibodies was easy to perform, required no expensive instrumentation, and provided useful information in the classification of acute myeloid leukemia.     

Impact of FAB classification on predicting outcome in acute myeloid leukemia,not otherwise specified,patients undergoing allogeneic stem cell transplantation in CR1: An analysis of 1690 patients from the acute leukemia working party of EBMT

The French, American, and British (FAB) classification system for acute myeloid leukemia (AML) is extensively used and is incorporated into the AML, not otherwise specified (NOS) category in the 2016 WHO edition of myeloid neoplasm classification. While recent data proposes that FAB classification does not provide additional prognostic information for patients for whom NPM1 status is available, it is unknown whether FAB still retains a current prognostic role in predicting outcome of AML patients undergoing allogeneic stem cell transplantation. Using the European Society of Blood and Bone Marrow Transplantation registry we analyzed outcome of 1690 patients transplanted in CR1 to determine if FAB classification provides additional prognostic value. Multivariate analysis revealed that M6/M7 patients had decreased leukemia free survival (hazard ratio (HR) of 1.41, 95% confidence interval (CI), 1.01–1.99; P = .046) in addition to increased nonrelapse mortality (NRM) rates (HR, 1.79; 95% CI, 1.06–3.01; P = .028) compared with other FAB types. In the NPM1^wt AML, NOS cohort, FAB M6/M7 was also associated with increased NRM (HR, 2.17; 95% CI, 1.14–4.16; P = .019). Finally, in FLT3‐ITD⁺ patients, multivariate analyses revealed that specific FAB types were tightly associated with adverse outcome. In conclusion, FAB classification may predict outcome following transplantation in AML, NOS patients.     

Further correlations of morphology according to FAB and WHO classification to cytogenetics in de novo acute myeloid leukemia: a study on 2,235 patients

In routine diagnostic procedures of acute myeloid leukemia (AML), the French–American–British (FAB) and World Health Organization (WHO) classifications both play a central role. Some morphologic subtypes are specifically associated to distinct cytogenetic and molecular aberrations; however, such close correlations do not exist for the majority of entities. We evaluated cytogenetics in 2,235 patients at diagnosis of AML with the FAB subtypes M0–2, M4, and M5–7. The cytogenetic patterns of these subtypes showed differences with respect to the clonal aberration rate and the incidence of complex aberrant karyotypes. The frequency of numerical gains and losses and of structural losses and the incidence of 11q23/MLL rearrangements differed. Thus, cytomorphology of AML may be helpful to support or even initiate other diagnostic procedures, e.g., interphase fluorescence in situ hybridization and polymerase chain reaction. In conclusion, the central role of morphology as defined by the FAB and WHO classification in AML at diagnosis is still justified in combination with other techniques.