急性早幼粒细胞白血病形态学和细胞遗传学的临床研究

目的 探讨形态学和细胞遗传学(MIC)联合检测对急性早幼粒细胞白血病(M3)的临床意义。方法 将MIC技术用于32例M3患者的诊断、分型及预后评价。结果 形态学检查26例M3a，中2例和6例M3b中3例曾误诊为其它白血病，经核型分析确诊。骨髓染色体分析正常3例(2／32)，28例(28／32)有t(15：17)，1例(1／32)具有变易移位，可评价的20例中19例(19／21)达到CR，具有复杂核型和变易移位各1例未能取得缓解。结论 MIC联合检测对M3的诊断、分型、评价预后等有重要价值。     

急性髓细胞性白血病和急性早幼粒细胞性白血病的治疗进展

2003年12月5～9日第45届美国血液学年会(ASH) 在美国召开,与会代表2万多人,我国有100多位代表参加了会议.上海第二医科大学瑞金医院王振义院士(the first speaker from Asia to serve as the Ham-Wasserman Lecturer)在大会上作了"Treatment of Acute Leukemia by Inducing Differentiation and Apoptosis"的专题发言,引发了热烈的讨论.现将该次年会中有关急性髓细胞性白血病(AML)和急性早幼粒细胞性白血病(APL)治疗的进展作一介绍.     

急性早幼粒细胞白血病九例临床误诊分析

自上世纪80年代中期全反式维甲酸(ATRA)应用于治疗急性早幼粒细胞白血病(APL)以来,该病已由一凶险、难治性急性髓细胞性白血病(AML)转变为一预后最好的急性白血病(AL)类型之一。目前以ATRA联合砷剂及化疗,其治愈率可达70%~75%[1]。但APL确诊过晚时,疗效明显变差。现将我院近5年     

急性早幼粒细胞白血病缓解后继发急性淋巴细胞白血病一例

患者,男,32岁,2016年2月因血尿5天、口腔溃疡伴牙龈出血2天就诊于深圳市宝安区人民医院.患者5天前无明显诱因出现血尿,全程尿色皆鲜红,2天后尿色转为暗红.尿中无泡沫,不伴尿痛、腰痛、尿液淋沥不净,大便正常.2天前晨起发现下唇系带附近口腔溃疡,溃疡面出血结痂,伴牙龈出血,为求进一步治疗来我院就诊,门诊以血尿、...     

急性早幼粒细胞白血病两亚型的比较分析

目的探讨急性早幼粒细胞白血病（APL）两亚型（M3a和M3b）的鉴别方法并比较其特点。方法对39例APL的细胞形态学、化学染色及血常规进行回顾性比较分析。结果急性早幼粒细胞白血病M3a型的细胞形态特点以胞浆中含粗大、深染、密集的嗜苯胺蓝颗粒增生为主（〉30％），M3b型的异常早幼粒细胞主要以密集、细小的嗜苯胺蓝颗粒为主，具有单核细胞样的特征。过氧化物染色两型均为强阳性。非特异性酯酶染色均不被氯化钠抑制。外周血M3b型的白细胞明显高于M3a型（P〈0．01）。结论通过对急性早幼粒细胞白血病的细胞形态、细胞化学染色及血常规的统计学分析，可对M3a和M3b作出初步的鉴别，以细胞形态检查最为关键。     

急性早幼粒细胞白血病的治疗

急性早幼粒细胞白血病 (APL)因起病急、病情凶险、易出血 ,伴发播散性血管内凝血 (DIC) ,传统化疗效果差 ,其早期病死率极高。自 80年代中期全反式维甲酸 (ATRA)应用于临床治疗 APL后 ,其缓解率明显提高 ,病死率降低 ,尤其是近年来砷制剂的应用 ,更使APL患者的生存率增加。 APL的治疗有以下几个方面。1.诱导缓解1.1　ATRA　为目前治疗 APL的首选药物。其优点为缓解率高 ,不抑制骨髓 ,副反应少 ,使用方便。现已证实 ,ATRA可诱导早幼粒细胞分化成熟 ,使大多数患者异常的染色体 t(15,17)核型消失 ,PML - RARα融合基因分化。AT…     

急性早幼粒细胞白血病并发中枢神经系统浸润临床分析

白血病细胞的髓外浸润是急性髓性白血病的并发症 ,发生率为 1 0 %左右 ,常见于单核细胞系白血病亚型 ,而急性早幼粒细胞白血病 (ＡＰＬ)并发髓外浸润则极为少见〔1〕。应用全反式维Ａ酸 (ＡＴＲＡ)治疗ＡＰＬ虽然取得了很高的缓解率 ,但髓外浸润有增多的倾向 ,而且多见于皮肤浸润〔2〕。我们 1 993～2 0 0 0年收治ＡＰＬ患者 1 5 4例 ,其中 1 2例并发中枢神经系统浸润 ,现报告如下。1　资料与方法1 .1 　 临床资料1 5 4例ＡＰＬ患者均系本院住院患者 ,符合ＡＰＬ的诊断标准〔3〕。其中男 80例 ,女 74例 ,年龄 1 6～70岁 ,平均 3 3岁。 1 2例…     

三氧化二砷治疗急性早幼粒细胞白血病43例临床观察

１９９６年５月至１９９９年８月,我们采用三氧化二砷（Ａｓ２Ｏ３）治疗急性早幼粒细胞白血病（ＡＰＬ）患者４３例,取得较好疗效。现报告如下。１　资料与方法本组男２９例,女１４例;年龄１１～６５岁,平均３６岁。患者均符合ＡＰＬ的诊断标准。其中初治３８例,复...     

全反式维甲酸治疗急性早幼粒细胞白血病20例临床分析

单用全反式维甲酸(RA)或并用小剂量高三尖杉酯碱(HOM)治疗急性早幼粒细胞白血病(APL)20例,其完全缓解率(CR)为70%.单用RA15例,CR66.7%,CR 时间为56±17.1天;并用小剂量HOM 5例,其中4例完全缓解,达CR 时间为55.3±19.2天.     

A Case of T-Cell Acute Lymphoblastic Leukemia after Treatment of Acute Promyelocytic Leukemia

We diagnosed T-cell acute lymphoblastic leukemia (T-ALL) with multiple cytogenetic abnormalities in a 17-year-old girl a year after she had received a diagnosis of acute promyelocytic leukemia (APML). After the diagnosis of APML in June 2001, the patient was treated with idarubicin and all-trans-retinoic acid. In September 1999, her younger sister also received a diagnosis of APML and to date has remained well. T-ALL after remission of APML is very rare, and only 1 such case has been reported. Possible causes include therapy-related reasons, genetic susceptibility to leukemia, and environmental exposure.     

急性早幼粒细胞白血病治疗前血象与预后分析

目的:探讨急性早幼粒细胞白血病(APL)患者治疗前外周血白细胞计数变化规律与预后的关系。方法:总结12例首诊时被误诊为其他疾病(或急性白血病的其他类型),最后在我科确诊为APL的患者首诊及确诊时血象、凝血功能变化及转归。结果:首诊至确诊为APL的时间4~45d不等,平均13.4d。9例确诊时WBC计数较首诊时升高,3例下降。首诊和确诊时平均WBC计数分别为5.13×109/L、25.16×109/L。经治疗后5例死亡,均在诱导治疗期间死于颅内出血,其余7例至今均为持续完全缓解(CCR)状态。结论:APL发病早期,患者外周血WBC计数多低于正常,以后,WBC计数可逐渐升高,凝血功能紊乱及出血倾向亦渐加重,患者的预后也明显变差。     

Genotypic and Phenotypic Characteristics of Acute Promyelocytic Leukemia Translocation Variants

Acute promyelocytic leukemia (APL) is a special disease entity of acute myeloid leukemia (AML). The clinical use of all-trans retinoic acid (ATRA) has transformed APL into the most curable form of AML. The majority of APL cases are characterized by the fusion gene PML-RARA. Although the PML-RARA fusion gene can be detected in almost all APL cases, translocation variants of APL have been reported. To date, this is the most comprehensive review of these translocations, discussing 15 different variants. Reviewed genes involved in APL variants include: ZBTB16, NPM, NuMA, STAT5b, PRKAR1A, FIP1L1, BCOR, NABP1, TBLR1, GTF2I, IRF2BP2, FNDC3B, ADAMDTS17, STAT3, and TFG. The genotypic and phenotypic features of APL translocations are summarized. All reported studies were either case reports or case series indicating the rarity of these entities and limiting the ability to drive conclusions regarding their characteristics. However, reported variants have shown variable clinical and morphological features, with diverse responsiveness to ATRA.     

两种剂量维甲酸治疗早幼粒白血病的观察研究

采用小剂量全反式维甲酸（ATRA）治疗急性早幼粒细胞白血病（APL）30例，与常规量历史组进行比较。结果显示：①小剂量ATRA同样有较高的CR率（96．5％），达CR时间不延长。②小剂量ATRA不降低高白细胞血症的发生率。③小剂量ATRA治疗的副作用可减少、减轻，无1例发生RAS。这与其末梢血WBC的峰值明显低于常规量组相一致。     

急性早幼粒细胞性白血病:全反式维甲酸诱导分化治疗中出血并发症的处理

16例急性早幼粒细胞性白血病(APL)采用全反式维甲酸(RA)进行诱导分化治疗。入院时和病程中80%(13/16例)的患者发生弥散性血管内凝血(DIC),4例发生严重出血。经配合包括肝素抗凝、新鲜血及血小板输注,并用地塞米松、脱水和防治感染等积极的综合性治疗,渡过危险期,有效保障 RA 诱导分化作用的发挥,全部病例取得骨髓完全缓解.     

Ultrastructural Studies in Acute Promyelocytic Leukemia

In this investigation, the cells of eightpatients with acute promyelocytic leukemia were followed by light and electron microscopy. Promyelocytes fromuntreated patients were filled withlarge, splinter-shaped granules. Thegranules were lysosomes, with someshowing the ultrastructural features ofAuer bodies. In cases responsive tochemotherapy, promyelocytes contained only infrequently splinter-shaped lysosomes, while most lysosomes were of more uniform size andshape. The persistence of large numbers of splinter-shaped lysosomeswithin promyelocytes was associatedwith episodes of disseminated intravascular coagulation less responsiveto heparin treatment. Promyelocytesfrom patients in remission were similarto promyeloctyes from nonleukemicbone marrow. The ultrastructural differences in lysosome morphology provide a better criterion for distinguishing malignant from normal promyelocytes than previously detectable bylight microscopy alone.

Submitted on June 2, 1971 Revised on October 8, 1971 Accepted on November 1, 1971     

急性白血病合并DIC53例临床分析

对５３例ＡＬ合并ＤＩＣ作回顾分析，并发ＤＩＣ的ＡＬ类型为ＡＰＬ２８例，非Ｍ３型ＡＮＬＬ１５例、ＡＬＬ１０例，发生于初发期、诱导化疗和化疗间歇期、难治期的ＤＩＣ分别占２５、１１、１７例。结果显示，ＡＰＬ合并ＤＩＣ６８％发生于初发期、非Ｍ３期ＡＮＬＬ并ＤＩＣ发生于难治期占６０％，而ＡＬＬ并ＤＩＣ５０％与诱导化疗有关。４０例经全程治疗者，ＤＩＣ治愈８例，其中６例为初发期患者（６／１６）。讨论了ＤＩＣ发生     

Two Cases of Acute Promyelocytic Leukemia Complicated by Torsade de Pointes during Arsenic Trioxide Therapy

We describe 2 patients with acute promyelocytic leukemia (APL) in whom torsade de pointes (TdP) developed during treatment with arsenic trioxide. Patient 1 was a 23-year-old woman with second-relapse APL. Ventricular premature beat bigeminy developed on day 27 of treatment, and episodes of TdP developed on day 28. Patient 2 was a 51-year-old woman with second-relapse APL who had cardiomyopathy due to prior anthracycline treatment. TdP developed on day 17 of treatment. Arsenic trioxide is known to cause electrocardiographic abnormalities, such as ventricular tachycardia and prolongation of QT interval. Patient 1 was given fluconazole as a concomitant drug. Patient 2 had cardiomyopathy and hypokalemia. Careful management is needed during arsenic trioxide therapy because this treatment prolongs the QT interval, possibly inducing episodes of TdP.     

Successful Management of Chronic Refractory Immune Thrombocytopenia with Laparoscopic Splenectomy in a Patient with Acute Promyelocytic Leukemia

Acute promyelocytic leukemia (APL) is a particular type of acute myeloid leukemia with characteristic biological and clinical features, the frequent association at diagnosis of a severe hemorrhagic diathesis. Immune thrombocytopenia (ITP) is a common autoimmune disease characterized by low platelet counts and an increased risk of bleeding. Here we present a patient with the diagnosis of APL who achieved and maintained a remission with an induction consisting of idarubicin and ATRA, and then developed corticosteroid refractory ITP which is successfully treated with laparoscopic splenectomy.