成人中枢神经系统白血病151例

报告成人中枢神经系统白血病（ＣＮＳＬ）１５１例，结果表明：ＣＮＳＬ与白血病的细胞类型、患者年龄、存活期及治疗方法有关。在急性淋巴细胞性白血病和急性非淋巴细胞白血病中，存活期＜１年时，ＣＮＳＬ的发生率以前者为高，存活期＞１年时，两者无显著性差异（ｘ２＝０．３５，Ｐ＞０．０５）。该组有５７例（３７．７％）ＣＮＳＬ缓解后出现复发，说明目前的治疗方法仍难阻止ＣＮＳＬ的出现和复发。比较鞘内化疗加局部放疗和仅用鞘内化疗的疗效，两者无显著性差异（ｒ＝０．０２，Ｐ＞０．０５）。     

The Role of Decreased Retinoblastoma Protein Expression in Acute Myelomonocytic and Monoblastic Leukemias

The results of different investigators show that lack of p 105 expression is relatively common in human myeloid leukemias, especially in monocytic leukemias. This suggests that loss of p105 expression could contribute to the altered growth control of these cells. So far no clear data exist which show that low p105 levels in AML blasts predict a poor therapy outcome. Therefore it is not very likely that p105 expression will become a strong prognostic factor for the different treatment strategies in AML.     

Serological detection and partial characterization of the common-ALL-cell associated antigen in the serum of cALL-patients.

Sera of patients with common-ALL were found to contain a glycoprotein reacting with antibodies directed to common-ALL associated antigen (Ag cALL). The identification of this protein which has an apparent molecular weight of 125,000 and a binding specificity to lens culinaris lectin is reported. These data are in good accordance with those estimated for the corresponding antigen solubilized from cALL cells as well as those obtained from cultured cell-lines established from leukemic cells and indicate an in vivo shedding or secretion of the molecule.     

Recent Trends in the Treatment and Prognosis of Adult Leukemia with Characteristics of Patients in Japan: Transition during the Fifteen Years from 1971 to 1985

Patients with acute (2,569) and chronic (957) leukemia diagnosedat 19 institutes took part in the study on the "MultidisciplinaryTreatment of Leukemia" between 1971 and 1985 and were investigatedretrospectively. By dividing the 15 years into three five-yearperiods, we were able to compare patient ratios in the differentperiods. The proportions of acute to chronic leukemia casesshowed no obvious change; however, the proportions of casesdiagnosed as acute lymphocytic leukemia in acute leukemia showeda significant increase. The main chemotherapeutic drugs usedduring the three time periods were cytarabine or its analogues,the anthracyclines, 6-mercaputopurine and prednisolone, againstacute myelogenous leukemia, and the vinca alkaloids, prednisoloneand the anthracyclines against acute lymphocytic leukemia. Therate of complete remission from acute myelogenous leukemia mademarked progress, from 45.1% during 1971–1975 to 62.3%during 1981–1985, but that of acute lymphocytic leukemiashowed no significant progress, being 65% during 1971–1975and 69.7% during 1981–1985. The durations of remission,however, and the survival times for patients with acute lymphocyticleukemia, as well as for those with acute myelogenous leukemia,became significantly longer over the three periods. Median survivaltimes from chronic myelocytic leukemia were 37–40 mo inall three periods, showing no progress. There was a better prognosisin cases of chronic myelocytic leukemia with, than without,Philadelphia chromosome. Except for a low incidence of chroniclymphocytic leukemia in Japan, adult leukemia patients' characteristicsand prognoses seem to be almost the same in Japan as in theU.S.A. and Europe.     

重庆渝东地区1997年-2006年白血病分型及现况分析

目的：总结渝东地区各类型白血病的分布情况，为该地区白血病的防治提供依据。方法：回顾分析1997至2006年，以万州为中心长江上游段三峡库区主要位置的渝东片区八个县一个区以及毗邻湖北、陕西省的部分县区范围，收集医院收治的各类型白血病病人的临床资料，病人按FAB形态学分型标准及组化染色进行确诊分型。结果：1997至2006年间共收治血液病病人并行骨髓穿刺12993例，确诊白血病1525例。急性非淋巴细胞白血病（ANLL）756例占49．4％，其次为急性淋巴细胞白血病（ALL）406例占27．4％和慢性白血病326例占21．4％；白血病在各年龄段均有发病，急性原始粒细胞白血病部分分化型（ANLL—M2）和慢性粒细胞白血病（CGL）为中老年人常见，而急性淋巴细胞白血病（ALL）在青少年时期为常见；一年内以10月份发病和收治人数最高。结论：调查从地域、性别、年龄段、季节性多方面反映了重庆渝东地区白血病的分布及构成情况，为该地区白血病的防治提供了依据。     

成人高白细胞急性白血病95例的临床分析

本文报告初诊时外周血白细胞数等于或大于100×10~9/L成人急性白血病93例的临床及治疗结果。显示,在确诊后的14天内,易发生致死性颅内出血,且常见于其血小板数仍高于10×10~9/L(10～58×10~9/L),凝血-抗凝血指标正常者。本组患者完全缓解率(46.9％)与同期治疗的702例非商白细胞急性白血病患者的(57.6％)相似,但中位生存时间较短,且与其白细胞数呈负相关。高龄、肝和(或)脾肿大、中枢神经系统和肺、肾等白血病细胞侵犯以及骨髓中白血病细胞高值均对急性非淋巴细胞白血病疗效有不利影响。此外,对诱导化疗失败的类型作了讨论。     

The Prognostic Implications of an Immunological Classification of Acute Lymphoblastic Leukemia

Summary

Thirty-one patients with newly diagnosed acute lymphoblastic leukemia were examined before receiving any treatment and their clinical and laboratory data were analyzed in order to determine the possible correlation between clinical presentation, morphologic sub-classes, cytochemical reactions, immunological phenotypes and cytogenetic findings. Each of the previous parameters and response to therapy were also examined for correlation.

The analysis of clinical and laboratory characteristics of patients according to their immunological phenotype did not show any significant male sex bias, age distribution, hepatomegaly or splenomegaly at diagnosis.

The analysis of clinical response of patients did not demonstrate any significant influence of sex, age, initial WBC count or the presence of a big tumor mass at diagnosis.

There were no significant differences between our two major immunological subclasses Non-T CALLA⁺ ALL, and Pre-T ALL regarding proportions of patients in continuous remission, and relapse-free survival durations.

The analysis of clinical and laboratory characteristics of patients on the basis of their chromosome categories did not show any significant sex bias, age distribution, initial WBC count, tumoral presentation or morphological subtypes at diagnosis, although there was an apparent male predominance in the pseudodiploid category and female predominance in the hyperdiploid category.

Our results concerning the prognostic implication of CALLA were contradictory to those of several other investigators.     

Where does transformation occur in acute leukemia?

We studied 43 consecutive cases of acute leukemia for evidence of hybrid leukemia including biphenotypic or bilineal involvement. Twenty-two were initially diagnosed as acute lymphoblastic leukemia (ALL) and 21 as acute myelogenous leukemia (AML). Techniques included morphology, cytochemistry, immune phenotyping and cytogenetics. Thirty-one cases seemed restricted to one lineage. Twelve cases showed involvement of both lymphoid and myeloid cells. Dual staining immune phenotyping showed coexpression of diverse lineage markers. These data indicate a considerable proportion of unselected cases of acute leukemia are hybrid leukemias. These data are consistent with the notion that transformation frequently occurs in a stem or progenitor cell.     

急性髓系白血病合并慢性淋巴细胞白血病一例报道及文献复习

 目的 探讨急性髓系白血病（AML）合并慢性淋巴细胞白血病（CLL）的临床特点、病因、诊断、治疗及预后。方法 临床诊断1例AML合并CLL,并就相关文献进行复习。结果 患者经MA方案（米托蒽醌10 mg／d第1 ~ 3天,阿糖胞苷150 mg／d第1,3,5,7天,200 mg第2,4,6天）化疗后取得完全缓解,但CD19阳性的淋巴细胞（表达CD20,CD23,SIgM,部分表达CD5,CD22,CD25）仍然存在,于9个月后AML复发未能再次缓解而死亡。结论 AML合并CLL为一种具有特殊生物学特征的罕见疾病,免疫分型和细胞遗传学技术在疾病的诊断和认识中发挥重要作用,治疗应以AML为主。     

成人急性白血病强化治疗67例预后分析

目的：观察成人急性白血病（ＡＬ） 患者完全缓解（ＣＲ） 后强化治疗的效果。方法：对６７ 例ＣＲ后的成人ＡＬ患者进行强化治疗,急性髓细胞性白血病（ＡＭＬ）以中剂量阿糖胞苷（ＩＤ－ Ａｒａ－ Ｃ）方案为主,急性淋巴细胞性白血病（ＡＬＬ）以中剂量氨甲蝶呤（ＩＤ－ ＭＴＸ）方案为主。结果：４８ 例ＡＭＬ患者中位ＣＲ 期１６ 个月,预期３ 年和４ 年无病生存（ＤＦＳ）为３６９％ 和２１１ ％ ;２３ 例（４７９ ％） 患者复发。１９ 例ＡＬＬ 患者中位ＣＲ 期１４ 个月,预期４ 年ＤＦＳ 为３１５％ ;１０ 例（５２６％ ） 患者复发。结论：以ＩＤ－ Ａｒａ－ Ｃ 为主的强化方案及以ＩＤ－ ＭＴＸ 为主的强化方案分别能延长ＡＭＬ及ＡＬＬ患者的ＤＦＳ,降低复发率     

Evaluation of radiation therapy factors in prophylactic central nervous system irradiation for childhood leukemia: A report from the Children's Cancer Study Group

The results of six different types of central nervous system (CNS) prophylaxis were studied in two successive Children's Cancer Study Group clinical trials of children with acute leukemia. Radiation therapy doses and technical factors were analyzed in relation to survival, relapse-free survival, bone marrow and CNS relapse rates, and the toxicities encountered in 656 study children. They were randomized among: (1) 2400 rad to the craniospinal axis (CS) and 1200 rad to the abdomen and gonad (2) 2400 rad CS, (3) 2400 rad to the cranium (Cr) + intrathecal methotrexate (IT/MTX), (4) IT/MTX alone, (5) 1800 rad CS, and (6) 1800 rad Cr + IT/MTX. Hematologic, gastrointestinal and infectious disease complications were highest in group 1. The patients were divided into low and high risk categories, defined as those with initial white blood cell counts below and above 20,000/cumm. for outcome analyses. No statistically significant differences were detected in the five-year rates for relapse-free survival or survival, nor for CNS or bone marrow relapse among the 5 irradiated groups when equipment variables, total doses, field arrangements, fractionation, and protraction were analyzed. These results should be interpreted in the light of the group 4 children who had the highest CNS relapse rates (e.g., 33 % for low risk patients vs. 4–16% for their counterparts in the other 5 groups), but nonetheless had a generally similar bone marrow and survival experience. Exceptions to the foregoing are the better five-year survival rates of 64 and 73 % respectively for group 3 and group 6 high-risk boys, contrasted with 25–42 % for their counterparts in the other 4 groups.     

1003例急性白血病骨髓中嗜酸粒细胞的观察

１００３例急白骨髓中Ｅ数量、阶段及形态的观察结果表明各类急白均有Ｅ的异常改变，以Ｍ４ＥＯ尤为显著，Ｍ２ａ、Ｍ２ｂ及Ｍ５ｂ的部分病例亦有较明显的数量及形态异常，Ｍ３和ＡＬＬ的变化较轻微。检出的Ｅ以幼稚阶段为主，成熟阶段较少，且幼稚Ｅ的形态改变比成熟Ｅ明显，呈核左移及核浆发育不平衡现象。Ｍ４ＥＯ均有明显的Ｅ形态异常改变，Ｅ及幼稚Ｅ均＞０．０５；认为Ｍ４ＥＯ的诊断标准以骨髓非红系中Ｅ＞０．０５为宜，同时必须强调形态的异常改变，尤其是幼稚Ｅ的异常改变。     

Clinical trial risk in leukemia: Biomarkers and trial design

This study analyzed the risk of clinical trial failure for leukemia drug development between January 1999 and January 2020. The specific leukemia subtypes of interest were acute lymphocytic leukemia (ALL), chronic lymphocytic leukemia (CLL), acute myeloid leukemia (AML), and chronic myeloid leukemia (CML). Drug development was investigated using data obtained from https://www.clinicaltrials.gov and other publicly available databases. Drug compounds were excluded if they began phase I testing for the indication of interest before January 1999, if they were not industry sponsored, or if they treated secondary complications of the disease. Further analysis was conducted on biomarker usage, drug mechanisms of action, and line of treatment. Drugs were identified following our inclusion criteria for ALL (72), CLL (106), AML (159), and CML (47). The likelihood (cumulative pass rate), a drug would pass all phases of clinical testing and obtain Food and Drug Administration approval, was 18% (ALL), 10% (CLL), 7% (AML), and 12% (CML). Biomarker targeted therapies improved the success rates by three‐ and sevenfold, for ALL and AML, respectively. Enzyme inhibitors doubled the cumulative success rate for AML. First‐line therapy and kinase inhibitors both independently doubled the cumulative success rate for CLL. Oncologists enrolling patients in clinical trials can increase success rates by up to sevenfold by prioritizing participation in trials involving biomarker usage, while consideration of factors such as drug mechanism of action and line of therapy can further double the clinical trial success rate.     

Differentiation of human leukemia cells and its usefulness for clinical diagnosis

Various chemical inducers have effects on the induction of terminal differentiation of human myelogenous leukemia cell lines. We studied morphological and functional changes of human leukemia cells freshly obtained from patients using 12-O-tetradecanoyl phorbol-13-acetate (TPA), retinoic acid (RA) or dimethyl sulphoxide (DMSO). The myeloid leukemia cells cultured with TPA became adherent to plastic culture dishes, and then developed macrophage-like morphology with long filamentous pseudopods within 48 h incubation. They showed marked enhancement of the ability to phagocytose latex particles. But these acquired properties did not always parallel each other, suggesting that the mechanism of functional maturation of leukemic cells induced by chemical agents was not identical with that of morphological changes. On the other hand, the lymphoid leukemia cells did not show morphological and functional changes when cultured with the above inducers. It is suggested that exposure of leukemic cells to TPA for relatively short times (12–24 h) may be useful for determining whether they are of myeloid or lymphoid origin. These characteristic changes were also observed in leukemic cells from the myeloid or lymphoid crisis of chronic myelogenous leukemia.     

c-kit在急性白血病中的表达及其临床意义

目的 :研究c kit受体 (c kitR ,CD1 1 7)在急性白血病 (AL)中的表达。重点了解c kitR表达对急性非淋巴细胞白血病 (ANLL)的诊断价值及其与ANLL的临床和生物学特征的关系。方法 :采用流式细胞术 (FCM)分别检测并比较 2 4例急性淋巴细胞白血病 (ALL)和 47例ANLL初诊患者骨髓单个核细胞 (MNC)跨膜c kitR表达的阳性率及阳性水平 ,并设立 1 0例正常人骨髓标本作为阴性对照组。结果 :ALL患者的c kitR表达的阳性率及阳性水平与正常人相比无显著性差异 (P >0 .0 5)。ANLL患者的c kitR表达率及阳性水平则均显著高于正常人和ALL患者(P均 <0 .0 1 )。c kitR阳性率以M1 、M2 最高 ,M4最低。ANLL患者c kitR表达与外周血白细胞计数、肝脾肿大无关(P >0 .0 5) ,但与染色体核型异常及LDH水平增高有关 (P均 <0 .0 5)。结论 :ANLL患者的c kitR阳性表达率及阳性水平均显著高于正常人和ALL患者 ,提示c kitR可作为AL患者MIC分型诊断的髓系免疫表型依据 ,可用以协助ANLL的诊断以及ANLL与ALL的鉴别诊断     

B-cell Chronic Lymphocytic Leukemia in Chronic Immune Thrombocytopenic Purpura: a Case Report

A patient suffering from chronic immune thrombocytopenia developing B-cell chronic lymphocytic leukemia seventeen years later, is reported. The possible mechanisms of association between the two disorders are discussed.     

白血病干细胞的天然耐药性分析

复发或耐药难治的急性白血病不能治愈的原因是患者体内存在一群具有自我更新能力的白血病干细胞（LSC）。虽然这些细胞数量极少,但可自我更新,具有很强的增殖潜能,在白血病发生和复发过程中起着关键性作用。全文对LSC起源、静歇性、抗凋亡性与易产生耐药性特点进行分析,提示有针对性的杀伤LSC,不但可有效克服白血病的天然耐药性,也是解决难治性白血病低缓解率、高复发率和低生存期的有效途径。     

Absolute lymphocyte count is a novel prognostic indicator in ALL and AML: implications for risk stratification and future studies

BACKGROUND: Leukemia is the leading cause of disease-related death in children, despite significant improvement in survival and modern risk stratification. The prognostic significance of absolute lymphocyte counts (ALC) was evaluated in young patients with acute myeloblastic leukemia (AML) and acute lymphoblastic leukemia (ALL). METHODS: In all, 171 consecutive de novo cases of AML and ALL, age 350 cells/microL carries an excellent prognosis, with a 5-year overall survival (OS) of 85% (HR 0.2, P= .012). Similarly in ALL, an ALC-15 <350 cells/microL predicts poor survival, with a 6-year RFS of 43% (HR 4.5, P= .002), whereas an ALC-15 >350 cells/microL predicts excellent outcome, with a 6-year OS of 87% (HR 0.2, P= .018). Importantly, ALC remains a strong predictor in multivariate analysis with known prognostic factors. CONCLUSIONS: ALC is a simple, statistically powerful measurement for patients with de novo AML and ALL. The results, when combined with previous studies, demonstrate that ALC is a powerful new prognostic factor for a range of malignancies. These findings suggest a need for further exploration of postchemotherapy immune status and immune-modulating cancer therapies.