骨髓增生异常综合征预后因素分析

目的:探讨骨髓增生异常综合征(MDS)的预后相关因素.方法:回顾性分析68例MDS患者的性别、年龄、WHO(2001)分类、诊断时的白细胞、血红蛋白、血小板水平、染色体核型和国际预后积分系统(IPSS)分组与生存期的关系,对相关因素进行多项Logistic回归分析.结果:68例患者总的中位生存期为20个月,不同性别患者...     

骨髓增生异常综合征骨髓活检的临床意义和预后价值

本文对82例初诊的骨髓增生异常综合征(MDS)进行骨髓活检,分析组织学特征与临床的关系。研究表明:未成熟前体细胞异常定位(ALIP)、原红细胞小岛、异形巨核细胞和红细胞是MDS的主要组织学特征,并伴有骨髓基质成分的病理改变。同时,对骨髓造血组织增生程度的判断有重要的预后价值,增生愈活跃,预后愈差。     

伴有骨髓纤维化的骨髓增生异常综合征

据文献报告，骨髓增生异常综合征（MDS）在诊断时骨髓纤维化（MF）的发生率为6％～70％“－”，说明MDS伴MF常见。1982年以来我们诊断15例，占同期MDS的13．82％，现将其实验室追踪随访的结果及预后进行探讨。三资料分析1．亚临床资料15例均为我院门诊及住院病人，男9例，女6例，年龄16～7O岁，平均年龄48．5岁。其中RA13例，RAEBI例，RAEB－ti例。15例均排除巨幼贫、溶血性贫血、缺铁、再障及原发性MF。初诊时均有不同程度的贫血，出血倾向IO例，感染发热6例，淋巴结肿大3例，胸骨压痛3例，肝肿大4例，脾轻度肿大3例。1．2实验…     

老年骨髓增生异常综合征患者的临床特点和预后分析

目的 探讨老年骨髓增生异常综合征(Myelodysplastic syndrome,MDS)患者的临床特点、临床转归及影响其预后的相关因素,评估国际预后积分系统(IPSS)在老年MDS预后中应用的意义.方法 选取2000年1月～2006年12月老年MDS患者(≥60岁)病例31例,因病采用不同治疗方案并追踪其临床病情变化情况.同期选取50例非老年性MDS患者(＜60岁)作为对照.所有生存率采用寿命表法,所有生存率函数曲线均采用Kaplan-Meier法分析.结果 31例老年MDS患者中位发病年龄为65岁.初诊时48.39%的患者有全血细胞减少,35.48%的患者合并感染.按WHO分型难治性白细胞减少伴多系增生异常(RCMD)和伴原始细胞增多的难治性蛋白-Ⅱ型(RAEB-Ⅱ)比例最高,共占84.62%.按1976年法、美、英三国制定的白血病分类法(FAB分型)RAEB患者比例最高,占61.29%.骨髓细胞形态学提示各系均有不同程度病态造血,以二系病态造血最多见(54.84%).19例骨髓活检患者中57.89%出现病态造血,47.37%出现幼稚前体细胞异常定位(ALIP)现象.13例患者进行细胞遗传学检查,染色体异常率为38.46%.截止随访结束,死亡26例(83.87%),中位生存期12.448个月,61.54%患者死于感染.老年组总存活期(overall survival,OS)显著低于非老年组患者(P＜0.000 1).IPSS不同危险组别的老年组(P＝0.004 5)和非老年组(P＜0.000 1)的总存活期均有显著差异.对各项临床指标的单因素分析结果表明,IPSS染色体分组(P＝0.002 4)和血小板计数(P＝0.041)是影响老年MDS预后的重要因素.不同治疗方法对老年MDS患者总存活期未见有明显区别(P＝0.912).结论 老年MDS患者以预后相对较差亚型常见,感染是其初诊及死亡的常见原因.相比非老年性患者,老年性MDS患者有显著较差的预后,IPSS及其染色体分组仍是判断老年MDS预后的重要指标.     

低危骨髓增生异常综合征初诊实验室检查结果与预后及转归的关系

为探讨低危骨髓增生异常综合征（ＭＤＳ）初诊时实验室检查结果与其预后及转归的关系，对９６例低危ＭＤＳ随访观察１２～５５个月。转为ＲＡＥＢ或ＲＡＥＢ－ｔ１０例，转为白血病１６例。死亡２７例，其中１５例死于骨髓衰竭，１２例死于白血病。初诊时外周血血红蛋白＜４０ｇ／Ｌ，血小板＜２０×１０９／Ｌ，中性粒细胞＜０５×１０９／Ｌ，骨髓涂片粒系增生活跃者预后不良；骨髓活检红系增生占优者转白率、病死率低于粒系增生占优者，骨髓造血细胞增生低下者易死于骨髓衰竭，ＡＬＩＰ阳性者转白率及病死率明显高于阴性者。单因素分析结果提示：初诊时实验室检查结果有助于判断低危ＭＤＳ的转归及预后     

骨髓增生异常综合征患者染色体变化与预后的关系

采用骨髓细胞短期培养法或直接法R带技术,对81例骨髓增生异常综合征(MDS)患者进行染色体核型分析.结果染色体核型异常37例,主要涉及8、5、7、9、11、20号染色体及Y染色体;正常44例.染色体核型正常与异常者的转白率分别为13.6%、54.1%,病死率分别为18.2%、64.9%,中位生存期分别为42.1、23.6个月,其差异均有统计学意义(P均<0.05).提示MDS患者的染色体变化对其预后判断有重要价值.     

骨髓增生异常综合征73例分析

本文分析我院近年所诊治的MDS73例,11例作骨髓造血祖细胞培养,均有红及(或)粒系祖细胞生长异常。35例作骨髓染色体检查,10例(28.6%)有克隆性核型异常。在追踪观察的58例中,1/3死于急性白血病,1/3死于感染及出血,尚存活者多为RA.RA较RAEB和RAEB-T生存期长。     

骨髓增生异常综合征与免疫异常

骨髓增生异常综合征与免疫异常徐世荣骨髓增生异常综合征（ＭＤＳ）为造血干细胞疾病，不仅在细胞遗传学、分子生物学、细胞生物学、血细胞酶学等方面有异常，而且在免疫学方面也存在异常。近１０余年来，研究进一步证实免疫功能异常在ＭＤＳ发病、发展，向急性白血病（Ａ...     

骨髓增生异常骨髓纤维化综合征5例

骨髓增生异常骨髓纤维化综合征５例福建省漳州市医院游慧萍，叶宝国，杨素美，游旭闽骨髓增生异常骨髓纤维化综合征（Ｍｙｅｌｏｄｙｓｐｌａｓｉａ／ＭｙｅｌｏｆｉｂｒｏｓｉｓＳｙｎｄｒｏｍｅＭＤ／ＭＦ）１９８１年由Ｓｕｌｔａｎ等首先报告，近来国外已陆续有报道，...     

骨髓增生异常综合征研究进展

骨髓增生异常综合征(MDS)是一组克隆性造血干细胞疾病,其特征是血细胞减少,髓系细胞一系或多系发育异常,无效造血及演变为急性髓系白血病(AML)的风险增高〔1-4〕。MDS的主要病理生理本质是:①起源于造血干细胞的克隆性疾病;②粒系、红系和巨核细胞系一系或多系发育异常;③无效造血。其临床表现主要是外周血一系或多系血细胞计数减少及由此导致的症状和体征,结局     

Evolving New Treatment for Myelodysplastic Syndromes

Over the last several years, there has been substantial progress in the definition, diagnosis, and management of myelodysplastic syndromes (MDSs). This progress includes the new World Health Organization classification and the revised standardized response criteria to be applicable to most new compounds, which, taken together with the International Prognostic Scoring System, provide a uniform basis for the management of individual patients. The recent introduction of certain new agents, as well as an apparent increase in the use of stem cell transplantation with a variety of so-called reduced-intensity settings, has indeed raised the hope that we are entering a new era of MDS treatment.     

Immunomodulatory Therapy for Myelodysplastic Syndromes

Thalidomide and lenalidomide belong to the proprietary group of immunomodulatory drugs (IMiDs) that display broad biologic and pharmacologic properties. Encouraging results of clinical studies that evaluated the efficacy of thalidomide in patients with myelodysplastic syndromes (MDSs) led to the investigation of its structural analogue, lenalidomide, in patients with lower-risk MDS. The cumulative results of studies that tested lenalidomide in patients with interstitial deletion of chromosome 5q, ie, del(5q), showed a high frequency of both erythroid and cytogenetic responses (approximately 75% of patients), which led to US Food and Drug Administration approval of this agent for this cytogenetically defined MDS subset. A multicenter phase III study (MDS-002) that investigated the frequency of transfusion response in lower-risk non-del(5q) MDS patients showed that lenalidomide had significant erythropoietic activity, albeit less robust in lower-risk MDS without del(5q). These studies established lenalidomide as an active erythropoietic-remitting agent with novel cytogenetic-remitting activity in lower-risk MDS patients who would not otherwise benefit from therapy with erythropoietic growth factors. The National Comprehensive Cancer Network Clinical Practice Guidelines recently added lenalidomide to the therapeutic algorithm for MDS as front-line therapy for lower-risk MDS patients with del(5q) and transfusion-dependent anemia.     

Oxidative Stress and the Myelodysplastic Syndromes

The evolution of higher organisms from anaerobic to aerobic living has promoted an elaborate mechanism of defense against potentially toxic oxidants. Many environmental toxicants implicated in the pathogenesis of myelodysplastic syndromes (MDS), including benzene and ionizing radiation, exert toxicity via pro-oxidant mechanisms. The emerging data suggest a probable genetic susceptibility to environmental carcinogenesis through functional polymorphic variants in enzymes that metabolize toxicants and/or protect against oxidative stress. The most studied enzyme is NAD(P)H:quinone oxidoreductase (NQO1). CD34+ cells from individuals homozygous for the NQO1 C609T nonfunctional allelic variant are incapable of enzyme induction following exposure to benzene, thus potentially increasing the hematotoxicity of benzene metabolites. Serologic and molecular markers of oxidative stress are present in many patients with MDS and include an increased concentration of the lipid peroxidation product malondialdehyde and the presence of oxidized bases in CD34+ cells. Potential mechanisms of oxidative stress include mitochondrial dysfunction via iron overload and mitochondrial DNA mutation, systemic inflammation, and bone marrow stromal defects. The biological activity of the antioxidant aminothiol amifostine in vivo suggests that these pathways may be meaningful targets for future therapy in MDS patients.     

骨髓增生异常综合征红细胞酶谱测定临床应用研究

本文比较了２３例ＭＤＳ和９例ＡＡ患者４种红细胞酶的活性，除醛缩酶外，丙酮酸激酶嘌呤核苷磷酸化酶、腺苷脱氨酸在ＡＡ患者均接近正常，和ＭＤＳ差异显著，尤其是ＰＫ和ＰＮＰ差异显著。据此联合检测红细胞４种酶活性，建立了ＭＤＳ和ＡＡ的差别函数式：ＰＭＤＳ＝－５．０４１０７＋０．２５６７４ＰＫ＋１．６７６５１ＡＬＤ＋０．０３４８１ＰＮＰ＋０．２５０２４ＡＤＡ，ＰＡＡ＝－７．９００３８＋０．３８４６ＰＫ＋１．４     

骨髓增生异常综合征患者血清GM-CSF水平测定及意义

目的 :了解 MDS患者血清 GM- CSF水平并探讨其意义。方法 :采用放射免疫法测定 32例MDS、35例健康人血清 GM- CSF水平 ,t检验行统计学处理。结果 :同正常人相比 ,MDS患者血清GM- CSF水平明显下降 ( 54.86± 17pg/ ml;34 .0 1± 14pg/ ml)。结论 :MDS患者 GM- CSF自分泌水平较正常人降低 ,GM- CSF分泌水平与 MDS分型无明显相关性 ,与疗效无肯定关系。     

Myelodysplastic syndromes

The authors review the epidemiological biological, diagnostic, prognostic and therapeutic aspects of myelodysplastic syndromes.     

Room for Improvement: A 20-Year Single Center Experience with Allogeneic Stem Cell Transplantation for Myelodysplastic Syndromes

Allogeneic stem cell transplantation (allo-SCT) remains the only curative therapeutic approach for patients with myelodysplastic syndromes (MDS). The aim of the study was to assess the efficacy/safety of allo-SCT as well as to identify factors influencing post-transplant survival. One hundred and two MDS patients (median age: 48 years; 57 males) who underwent allo-SCT were retrospectively evaluated. Twenty seven patients were transplanted from HLA-matched sibling and 75 patients received grafts from unrelated donors. Peripheral blood was a source of stem cell for 79 patients. Reduced intensity conditioning was used in 64 subjects. Acute and chronic graft versus host disease (GvHD) developed in 61 and 19 of patients, respectively. In total, 61 patients have died. The causes of deaths included infectious complications (n = 30), steroid-resistant GvHD (n = 17), MDS relapse (n = 9) and transformation to AML (n = 5). Non-relapse mortality and cumulative incidence of relapse at 2 years were 49.8% and 9%, respectively. 41 patients are alive at last contact and present full donor chimerism. 38 patients remain in complete hematological remission (CHR), 3 patients had CHR with incomplete platelet recovery. Median follow-up from diagnosis of MDS and transplantation are 27.1 months and 7 months respectively. Overall survival and relapse-free survival were 41% at 2 years. Increased serum ferritin level > 1000 ng/ml, presence of acute GvHD, grades III–IV acute GvHD and high hematopoietic cell transplantation-comorbidity index were found to negatively influenced survival. Allo-SCT for MDS is feasible procedure with a proportion of patients to be cured.     

伴骨髓纤维化的MDS的独特临床病理现象

目的：进一步加深对于伴骨髓纤维化的骨髓增生异常综合征（ＭＤＳ－ＭＦ）这一特殊亚型的认识。方法：从临床、病理形态角度总结１２ 例ＭＤＳ－ＭＦ患者的特征。结果：所有病例均具备全血细胞减少、病态造血、骨髓纤维组织增生及预后较差的特征。该型不同于无骨髓纤维化的ＭＤＳ和原发性骨髓纤维化（ＩＭＦ）,与前者的区别在于巨核细胞增多。对常规治疗反应不良,生存期明显缩短;与后者的区别在于无显著的肝脾肿大,骨髓较易抽取,有三系病态造血等。结论：ＭＤＳ－ＭＦ是一具有特色的亚型,有必要与ＩＭＦ和ＭＤＳ的其他亚型相区别。