临床血液学杂志
Journal of Clinical Hematology 림상혈액학잡지
- 主管单位： 中华人民共和国教育部
- 主办单位： 华中科技大学同济医学院附属协和医院 北京大学医学院血研所
- 影响因子： 1.06
- 审稿时间： 1-3个月
- 国际刊号： 1004-2806
- 国内刊号： 42-1284/R
- 论文标题 期刊级别 审稿状态
-
乳腺癌化疗后继发急性早幼粒细胞白血病2例并文献复习
We reported 2 cases of secondary APL after breast cancer chemotherapy and conducted a literatures review.Two patients with secondary APL had a history of breast cancer chemotherapy with 2 years of incubation period.The treatment was effective and 2 patients were in a state of complete remission after the follow-up of 40 and 7 months respectively.Alkylating agent or anthracycline for treating breast cancer patients may increase the risk of secondary APL.But the clinical features of secondary APL are similar to that of primary APL.The prognosis of secondary APL is good,which is different from other types of secondary leukemia.
-
SIL-TAL1基因阳性的急性T淋巴细胞白血病合并肿瘤溶解综合征1例报告
Acute T lymphoblastic leukemia is characterized by T cells malignant clone proliferation.Abnormal karyotypes can be detected among 50％ to 70 ％ patients with acute T lymphoblastic leukemia.Cytogenetics detection has become an important index in the diagnosis of acute T lymphoblastic leukemia.It is also closely related to prognosis.SIL-TAL1 (+) acute T lymphoblastic leukemia patient is in a critical condition,with rapid progression and poor prognosis.Here we report a SIL-TAL1 gene positive acute T lymphoblastic leukemia patient.
-
国产达沙替尼治疗慢性髓系白血病急髓变伴Y253H突变1例报告
To observe the curative effect and security of the generic dasatinib for treatment of the chronic myeloid leukemia (CML) in blastic phase with Y253H gene mutation and to improve the understanding of the disease.We treated an elderly patient of CML in blastic phase with Y253H gene mutation with generic dasatinib (100 to 140 mg daily),the patient appeared to be resistant with imatinib mesylate and nilotinib after treatment.His blood regular reminded leukocytosis,peripheral blood basophils amounted to 71％,and there were different phase of immature cells in his peripheral blood,the results of bone marrow examination returned that the patient got Y253 H gene mutation positive,BCR-ABL fusion gene quantification was 2 496.11 copies/10 000 abl copies,and he had additional chromosome abnormality.We diagnosed the patient with CML in blastic phase by analyzing the results of his examination.Considering his age and tolerance,he was treated with domestic dasatinib.During the treatment analysis of blood,bone marrow,genetics and molecular examinations were taken regularly to evaluate the efficacy.Getting the information in details about the patient and learning pertinent literature to analyze this kind of disease.The patient's peripheral blood basophils descended to 5％ and immature cells disappeared in 1.5 months,getting complete hematologic response.His gene of Y253H turned to be negative,chromosomes were back to normal and BCR-ABL fusion gene quantification dropped to 21.04 copies/10 000 abl copies in 3 months,getting complete cytogenetic response and major molecular response.The patient's BCR-ABL fusion gene quantification dropped to 1.6 copies/10 000 abl copies in 8 months,getting the newly defined very deep molecular responses MR 4.0.During the treatment,there was no adverse event besides a transient pancytopenia.Patients of CML in blastic phase have a poor prognosis and some of them can get remission and live for a longer time by using TKI alone.Domestic dasatinib is effective and well tolerant for patients of CML in blastic phase with Y253H gene mutation.
-
骨髓增生异常综合征相关基因研究进展
Myelodysplastic syndrome (MDS) is a cluster of heterogeneous clonal hematopoietic neoplasm which is manifested by peripheral cytopenias,lineage dysplasia,and a substantial risk of progression to acute myeloid leukemia (AML).Approximately more than 80％ of MDS patients have been shown to harbor gene mutations.The mutations have been found to be related to epigenetic alterations (including DNA methylation and histone modification TET2,DNMT3A,IDH1/2,WT1,EZH2 and ASXL1),RNA splicing (SF3B1,SRSF2,U2AF1,ZRSR and PRPF8),transcription regulation (RUNX1,TP53),signal transduction (NRAS,KRAS,CBL,JAK2).Recent advances in the molecular pathogenesis of MDS may contribute to the clinical diagnosis,risk stratification,prognostic assessment and therapeutic insights of this disease.This paper will review these genes involved in MDS patients.
-
miR-29在白血病中的研究进展
MicroRNA is a class of small no-coding RNA comprising of 21-22 nucleotides,which plays a significant role in cell proliferation,differentiation,apoptosis and other physiological phases.A lot of researches indicate that abnormal expression of microRNA is associated with tumorigenesis and progression.Studies about microRNA in leukemia grow fast in recent years.The dysregulated expression of microRNA directly contributes to hematological malignancies,such as acute myeloid leukemia (AML),chronic lymphocytic leukemia (CLL) and so on.The miR-29 family contains miR-29a,miR-29b and miR-29c.Studies have shown that the aberrantly expression of miR-29 family influence the tumorigenesis,progression and prognosis.It plays a role of oncogenesis or tumor supression and may serve as the potential therapy targets of leukemia.In this review,the genetic structure of miR-29 and its regulation mechanism in leukemia are discussed.
-
二代酪氨酸激酶抑制剂达沙替尼治疗慢性髓性白血病安全性研究进展
Chronic myeloid leukemia (CML) is a malignant hematopoietic myeloproliferative neoplasm characterized in 95 ％ of cases by t(9:22)(q34;q11),the fusion of Abelson leukemia virus (ABL) gene on chromosome 9 with the breakpoint cluster region (BCR) gene on chromosome 22,resulting in BCR-ABL fusion gene (Philadelphia chromosome,Ph) and protein,which account for the molecular pathogenesis of CML occurrence and development.As the first generation of tyrosine kinase inhibitor (TKI) targeting BCR-ABL activity,imatinib mesylate has revolutionized the treatment of CML and transformed CML into a manageable'chronic'disorder,but resistance as well as intolerance has emerged as a significant clinical concern.Dasatinib is one of the second-generation TKIs that can achieve faster and deeper molecular responses with a favorable safety profile,and its related hematologic and non-hematologic adverse events (AE) are predominantly mild to moderate,self-limiting,reversible or manageable.While choosing a TKI treatment,balancing expected benefits with tolerance and resistance should be taken into consideration.Early detection and proper intervention of AE can minimize the potential risk and damage,increase patients adherence and persistence,as well as quality of life,which is important for patients with lifelong CML treatment.
-
WT1基因与造血系统月中瘤研究现状
Wilms tumor gene 1(WT1) manifests both tumour suppressor and oncogenic activities,and WT1 can either activate or repress numerous target genes resulting in disparate biological effects.There are many researchs of WT1 gene in growth,development and tumor,in recent years WT1 gene on hematopoietic neoplasias research attracted the attention of people and some of the research results have been used in clinical work.This paper reviews the expression regulation,target genes and clinical application value study of WT1.
-
多发性骨髓瘤累及中枢神经系统8例的临床观察
多发性骨髓瘤(multiple myeloma,MM)是单克隆浆细胞在骨髓中异常增生的恶性肿瘤,通常局限于骨髓.约5％的MM患者会累及髓外软组织器官(淋巴结、肝、鼻咽、喉及上呼吸道、胃肠道、皮肤、会阴、内分泌腺等)[1],称为髓外浆细胞瘤.高黏滞综合征、M蛋白相关的神经病变、高钙血症或脊髓压迫引起MM患者神经系统症状并不少见,但直接侵犯中枢神经系统(central nervous system,CNS)的病例很少,仅见于约1％的MM患者.本研究回顾性分析2002-01-2015-12我院诊治的8例累及CNS的MM患者的临床和实验室资料,现报告如下,并复习相关文献.
-
HAML诱导治疗前阿糖胞苷持续静脉滴注临床疗效观察
高白细胞急性髓系白血病(hyperleukocytic acute myeloid leukemia,HAML)属于血液科的危重症,其病情进展迅速,早期死亡率高,预后差.治疗的关键在于尽快降低外周血白细胞负荷,以接受诱导化疗,如处理得当,仍可达到缓解并能获得长期生存.目前治疗性血细胞分离和小剂量化疗是HAML诱导化疗前降白细胞治疗比较公认的治疗选择,但是部分患者由于心血管合并症、凝血功能障碍等原因不能采用治疗性血细胞分离.无论是否进行治疗性血细胞分离,都需要选用一种化疗药物降低白血病细胞负荷,目前选择羟基脲口服、小剂量阿糖胞苷(Ara-C)静脉滴注或肌肉注射的较多,应用减低剂量的化疗药物联合治疗也有报道,但是少有以Ara-C持续静脉滴注方式给药降白细胞治疗的报道.我们观察了60例HAML患者诱导治疗前单独应用Ara-C持续静脉滴注或Ara-C持续静脉滴注联合白细胞分离术作为HAML诱导前治疗的效果.
-
X线修复交叉互补基因3Thr241Met(rs861539)基因多态性与淋巴瘤易感性Meta分析
目的:通过Meta分析方法探讨X线修复交叉互补基因3 XRCC3 Thr241Met(rs861539)多态性与淋巴瘤易感性的关系.方法:通过检索2016年4月之前PubMed,Embase,Cochrane Library,Web of Science andthe Chinese Biomedical Literature Database的相关文献,我们进行了一项荟萃分析以研究它们之间的关系.结果:在这项荟萃分析中,后我们纳入了854例病例组和1 081例对照组.结果显示XRCC3的5种基因模型与淋巴瘤的风险之间无统计学意义(TT vs.CC,OR=1.05,95％CI=0.79～1.38,P=0.75;TT+CT vs.CC,OR=1.01,95％CI=0.84～1.22,P=0.88;TT vs.CT+CC,OR=1.02,95％CI=0.80～1.32,P=0.86;T vs.C,OR=1.01,95％CI=0.89～1.16,P=0.84;CT vs.CC,OR=1.08,95％ CI=0.79～1.48,P=0.62).结论:Meta分析结果表明:尚没有足够的证据证明XRCC3 Thr241Met(rs861539)多态性与淋巴瘤的风险之间有联系.
-
基于Bayes判别分析法的慢性再生障碍性贫血中医证候诊断研究
目的:探讨Bayes判别分析方法在慢性再生障碍性贫血(CAA)中医辨证分型中的应用,为其规范化中医证候诊断提供科学依据.方法:利用“全国中医医疗与临床科研信息共享系统”收集我院372例CAA患者症状评估量表信息,涵盖CAA常见的12种症状.将患者中医辨证分型分为肾阳虚型、肾阴虚型以及肾阴阳两虚型3组,以Bayes判别分析方法建立CAA辨证分型的判别方程,并采用自身验证回代法对函数式进行检验.结果:根据12项症状建立CAA中医证型判别函数,回代法判别肾阳虚证的正确率为94.93％,肾阴虚证的正确率为59.26％,肾阴阳两虚证的正确率为82.81％,总体正确率为88.17％.结论:将判别分析等多元统计方法用于中医证候的研究中,有利于提高临床诊断的标准化、客观性以及准确性.
-
超小剂量地西他滨治疗骨髓增生异常综合征疗效分析
目的:总结超小剂量地西他滨治疗骨髓增生异常综合征(MDS)临床疗效与安全性.方法:回顾性分析皮下注射地西他滨(5～7 mg· m-2·d-1,d1～3,8,15,22,6周为1个疗程)治疗16例MDS患者的疗效和不良反应.结果:2例(12.5％)获完全缓解,2例(12.5％)获部分缓解并脱离成分血输注,5例(31.3％)达血液学改善,5例(31.3％)病情稳定,总反应率87.5％.Ⅳ级血液学毒性发生率2例(12.5％),Ⅲ～Ⅳ级感染发生率4例(25.0％),无Ⅲ～Ⅳ级出血、恶心呕吐和肝功能损伤.中位随访时间15.5(6～27)个月,随访期间1例死亡.结论:超小剂量地西他滨可以有效治疗MDS,严重血液学毒性和早期病死发生率低.
-
4个积分系统在弥散性血管内凝血诊断与预后评估中的作用
目的:评价弥散性血管内凝血(DIC)4个积分系统:JMHW评分、ISTH显性评分、JAAM评分和M-JAAM评分在DIC诊断与预后评估中的作用;并分析各凝血指标对DIC的诊断价值及预后的影响.方法:搜集疑诊为DIC的非恶性血液病患者共579例,并对搜集患者的临床资料进行回顾性分析.分别根据4个积分系统进行评分并比较各评分标准对非恶性血液病DIC的诊断效能及死亡预判效果;分析各凝血指标对DIC的诊断价值及预后的影响.结果:M-JAAM评分对DIC的诊断阳性率高(74.96％),与JAAM评分的诊断符合率为97.75％,32例(16.67％)先于ISTH评分被诊断为DIC,16例(6.45％)先于JMHW评分被诊断为DIC,M-JAAM评分对28 d死亡预判效果差(AUC=0.690),与AT活性的取值较高有关.JMHW评分对DIC的死亡预判佳(AUC=0.775),但JMHW评分诊断率(42.83％)不如JAAM评分(74.09％)和M-JAAM评分(74.96％).ISTH评分相对于其他标准,诊断阳性率(33.16％)及死亡预判(AUC=0.721)均没有优势.JAAM评分诊断阳性率略低于M-JAAM评分,但死亡预判效果(AUC=0.705)高于M-JAAM评分(AUC=0.690).临床表现的发生率、血小板计数(PLT)、凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)、抗凝血酶(AT)、D-二聚体(D-D)和纤维蛋白(原)降解产物(FDP)在DIC阳性组和DIC阴性组间均差异有统计学意义(P＜0.05).PLT、PT、APTT、D-D、FDP、AT与DIC的预后及积分值相关性较好.结论:为了早期发现DIC,JAAM评分和M-JAAM评分更有利,如已达到JMHW评分诊断为DIC时,则患者死亡风险高,应予以高度重视;PLT、PT、APTT、AT、D-D、FDP、AT对DIC的诊断及预后评估均有一定价值.
-
免疫球蛋白重/轻链检测在IgA型多发性骨髓瘤患者预后判断中的作用
目的:观察疗效评估为非常好的部分缓解(VGPR)及以上的IgA型多发性骨髓瘤(MM)患者中血清免疫球蛋白重/轻链(HLC)和游离轻链(FLC)及免疫固定电泳(IFE)的结果,探讨HLC在IgA型MM患者疗效和预后评估中的作用.方法:收集54例治疗后疗效评估为VGPR及以上IgA型MM患者血清样本,采用散射免疫比浊法在全自动SPA plus特定蛋白分析仪上进行HLC及FLC检测.结合同期IFE检测结果,评价HLC在IgA型MM疗效及预后判断中的价值.结果:①54例IgA型MM患者中,22例rHLC(HLC IgA-κ/IgA-λ比值)异常,其中IgA-κ型13例,IgA-λ型9例,rHLC检测结果的中位数分别为4.30、0.29;rHLC异常组与rHLC正常组中位无进展生存期(PFS)分别为7.8个月与13.0个月(P=0.018);中位总生存期(OS)分别为11.2个月和13.2个月(P=0.048).②54例患者中,15例IFE、rHLC、rFLC 3项指标均正常为A组;12例有1项指标异常为B组;18例有2项指标异常为C组;9例3项指标均异常为D组;A、B、C、D组中位PFS分别为16.6个月、14.2个月、7.8个月、7.0个月(P=0.019);中位OS分别为18.7个月、16.5个月、9.4个月、9.3个月(P=0.016).结论:达到VGPR及以上疗效的MM患者仍能检出rHLC的异常,rHLC异常提示患者预后不良;HLC与FLC及IFE检测的联合应用可以更好地对IgA型MM患者进行预后监测.
-
重型再生障碍性贫血并发感染的临床分析
目的:探讨重型再生障碍性贫血(SAA)患者并发感染的临床特征,以指导治疗.方法:回顾性分析50例SAA患者,总结SAA所并发感染的特点.结果:SAA患者并发感染的患病率为100％.粒细胞缺乏(粒缺)时间越长,容易并发感染的部位数越多(P=0.034).感染好发部位常见的是肺部和上呼吸道,其次为口腔,皮肤软组织,血流,胃肠道,泌尿系等.肺感染与年龄相关(P=0.026),血流感染与粒缺持续时间相关(P=0.032).感染部位取得标本培养结果以革兰阴性细菌为主,占68.9％,革兰阳性细菌占27.3％,真菌占3.8％.革兰阳性细菌中常见的是凝固酶阴性葡萄球菌.革兰阴性细菌中常见的依次为铜绿假单胞菌,肺炎克雷伯杆菌,阴沟肠杆菌,鲍曼不动杆菌,嗜麦芽窄食单胞菌,大肠埃希菌.血培养中常见的是肠杆菌科,凝固酶阴性的葡萄球菌,痰培养中常见的依次是非发酵菌,肠杆菌科.50例患者中,19例(38％)合并有病毒感染,常见的是单纯疱疹.1例患者合并肺结核,症状体征均不典型.粒缺时间越长,感染持续时间越长.SAA并发感染患者好转27例,自动出院18例,死亡5例.血流感染组病死率高于无血流感染组(P=0.004).结论:SAA患者是感染的高危人群.经验性治疗必须覆盖革兰阴性细菌.同时应积极促进中性粒细胞水平恢复,缩短粒缺时间,以达到控制感染,提高疗效,减少死亡率的目的.
-
《外显子测序方法鉴定急性红白血病中GATA2及CEBPA突变高再现性》解读
1 研究背景急性红白血病(AEL)是急性髓系白血病(AML)的一个特殊亚群,以红系显著异常增生为特征.法英美协会(FAB)将AEL定义为骨髓红系异常比例≥50％骨髓有核细胞,原始细胞占非红系有核细胞比例≥30％[1],2008年世界卫生组织(WHO)对其进行修订:骨髓红系异常比例≥50％骨髓有核细胞,原始细胞占非红系有核细胞比例≥20％[2].AEL在成人AML中的发病率不到5％,且随着年龄的增长而升高[3].据以往文献报道,AEL不同于AML的其他亚型,以复杂核型为主,预后较差[4].
-
vdac-1基因调控多发性骨髓瘤细胞表达髓系分化抗原CD33实验研究
目的:探讨线粒体外膜电压依赖阴离子通道-1(vdac-1)基因对多发性骨髓瘤(MM)细胞表达髓系分化抗原CD33的影响,为MM的治疗提供新的思路.方法:应用细胞转染技术将携带vdac-1基因的质粒转染不表达Pax5和CD45的骨髓瘤细胞系U266细胞中,蛋白印迹检测其vdac-1蛋白及髓系细胞重要转录因子C/EBPα的表达;显微镜观察细胞形态的改变;流式细胞术检测细胞表面CD45及CD33分子的表达.结果:CD45-的U266细胞转染vdac-1基因后,高水平表达vdac-1基因和蛋白,并且髓系细胞重要转录因子C/EBPα表达增加;显微镜下观察发现细胞发生类似于髓系细胞形态学改变,如胞浆突起、胞核折叠;流式细胞术检测发现转染vdac-1基因的U266细胞不表达CD45,而CD33表达增加,其中CD33阳性细胞占细胞总数的30％以上.结论:vdac-1基因能够调控MM细胞表达髓系分化抗原CD33,使细胞发生髓系细胞形态学改变,CD33有望成为MM治疗新靶点.
-
地西他滨联合阿糖胞苷对HL60细胞的增殖抑制作用研究
目的:研究地西他滨(DAC)和阿糖胞苷(Ara-C)单用或联合作用对人急性髓细胞白血病HL60细胞增殖的影响,并探讨佳的联合给药方式.方法:将不同浓度的DAC和Ara-C分别单独或联合作用于HL60细胞,采用CCK8法检测2种药物单独或联合作用对HL60细胞增殖的影响,采用金(正均)氏公式计算协同系数Q值分析两药的协同作用.结果:DAC和Ara-C单独作用时对HL60细胞增殖的抑制作用均呈剂量依赖关系,DAC(0.13～2.00 μmol/L)序贯Ara-C联用对HL60细胞增殖抑制有协同作用(Q值为1.20～1.54),并呈剂量依赖性.DAC同时联合Ara-C或Ara-C序贯DAC无协同作用,甚至为拮抗作用(Q值＜1.15).且DAC序贯Ara-C对HL60细胞的增殖抑制率明显高于DAC同时联合Ara-C或Ara-C序贯DAC(P＜0.05).结论:DAC序贯Ara-C可协同抑制HL60细胞增殖,该方案明显优于DAC与Ara-C同时用药组或Ara-C序贯DAC组.DAC48 h后序贯Ara-C联合方案在急性髓细胞白血病的治疗中可能具有重要临床意义.
-
未来精准治疗白血病的卫士——新一代高通量测序技术
近几年,新一代高通量测序技术又称下一代测序技术(next-generation sequencing technology,NGS)在白血病克隆演变、诊断、预后分析、疗效评价、复发预测以及药物开发等方面得到广泛应用,新的进展层出不穷.本文仅就应用NGS技术在白血病克隆演变、预后分析和疗效评价等方面取得的新进展作一介绍.
-
急性淋巴细胞白血病的分子遗传学研究进展
急性淋巴细胞白血病(acute lymphoblastic leukemia,ALL)是一类起源于髓或淋巴细胞前体细胞的恶性克隆性肿瘤.ALL是儿童常见的恶性肿瘤之一,约占儿童急性白血病的80％.成人ALL患者的发病率较低,约占成人急性白血病的20％.随着对ALL致病机制的深入了解和个体化分层治疗,儿童ALL患者的临床疗效有了显著提高,其5年总生存率超过90％[1];而成人ALL患者对化疗不敏感,容易复发,预后较差.大多数ALL患者可检测出克隆性染色体异常,54％的儿童ALL患者检测到至少一种融合基因[2].细胞和分子遗传学异常的改变对于指导ALL患者的诊断、分型和治疗有着重要的意义,一些特殊的分子遗传学异常在WHO分型建议中已被列为特殊亚型.随着全基因组测序、全外显子测序、转录组测序等高通量分子生物学技术的广泛应用,逐渐揭示了ALL中一些新的分子遗传学异常,不但丰富了我们对ALL发病机制的理解,同时也为发展新的靶向治疗提供了契机.
-
血液系统恶性肿瘤精准诊疗现状与进展
1 概述本世纪初,人类基因组计划(H GP)的完成为基础向临床转化医学研究奠定了基础,随后癌症基因组计划(CGP)对上万例肿瘤样本进行测序.通过深入挖掘基因组学数据,探索并构建基于遗传与表观遗传学背景的信息网络,对恶性肿瘤进行精准分型和预后分层,进而实现其精准诊疗.
-
骨髓增生异常综合征的精准诊断与治疗:现况与问题
精准医疗(precision medicine)基于基因组、蛋白质组和药物组学等组学技术,精准寻找到疾病的原因和治疗靶点,实现对于疾病和特定患者进行精准诊断和精准治疗.骨髓增生异常综合征(MDS)是一组起源于造血干细胞的异质性髓系肿瘤,近年应用2代测序技术剖析了MDS的基因突变谱系,从而开启了MDS的精准诊断和精准治疗的新征程[1-2].本文拟对该领域的认识现况和存在的问题做一简述.
| 年 | 期数 |
| 2018 | 01 02 03 04 05 06 07 08 09 10 11 12 |
| 2017 | 01 02 03 04 05 06 07 08 09 10 11 12 |
| 2016 | 01 02 03 04 05 06 |
| 2015 | 01 02 03 04 05 06 |
| 2014 | 01 02 03 04 05 06 |
| 2013 | 01 02 03 04 05 06 |
| 2012 | 01 02 03 04 05 06 |
| 2011 | 01 02 03 04 05 06 |
| 2010 | 01 02 03 04 05 06 |
| 2009 | 01 02 03 04 05 06 |
| 2008 | 01 02 03 04 05 06 |
| 2007 | 01 02 03 04 05 06 |
| 2006 | 01 02 03 04 05 06 |
| 2005 | 01 02 03 04 05 06 |
| 2004 | 01 02 03 04 05 06 |
| 2003 | 01 02 03 04 05 06 |
| 2002 | 01 02 03 04 05 06 |
| 2001 | 01 02 03 04 05 06 |
| 2000 | 01 02 03 04 05 06 |
| 1999 | 01 02 03 04 05 06 |
