难分类急性白血病的诊断及预后

本文用细胞形态学、细胞化学、免疫分型、免疫球蛋白和T细胞受体基因重排及电镜过氧化物酶联合分析研究了40例难分类急性白血病(UAL)。结果发现,5例为ALL与AML误诊者,12例为杂合性急性白血病(HAL),11例为过氧化物酶阴性急性骨髓细胞白血病(MPO~--AML),12例为未分化型急性白血病(AUL)。HAL缓解率降,易伴有高WBC数和浸润体征,MPO~--AML易误诊,AUL为多系列细胞来源,并建议更加严格HAL和AUL的诊断标准。此外,作者还讨论了FAB分型和免疫分型等手段在白血病诊断分型上的作用并推荐联合用形态学、免疫学、分子生物学等多种手段研究分析UAL。     

急性白血病细胞形态学与免疫学分型的关系

背景与目的：近年来应用流式细胞术对白血病进行免疫学分型,可以了解细胞的来源及其分化阶段,提高急性白血病诊断分型的准确性.免疫学分型所反映的细胞分化水平与FAB细胞形态学分类有一定的相关性,但两者又不完全一致.本研究将探讨细胞形态学FAB分型与免疫学分型在急性白血病诊断中的相互关系.方法：2001年1月-2005年1月,本院初诊的302例急性白血病患者进行细胞形态学FAB分型,同时采用流式细胞术进行免疫学分型,比较两者在急性白血病诊断分型上的异同.结果：本组急性白血病FAB分型与免疫学分型具有较好的一致性,总符合率为89.7%,其中两者不相符合的白血病类型主要是急性混合细胞白血病（BAL）和急性未分化型白血病（AUL）.由免疫学分型诊断的BAL在FAB分型方面包括AML-M1、M2、ALL-L1、L2,其中L2最多见,其次是M1.结论：免疫学分型与FAB形态学分型有较高的符合率,应互相结合、互相补充,尤其对于怀疑BAL、AUL或形态学不典型的病例应尽早进行免疫学分型检测,以免误诊或漏诊.     

慢性粒细胞白血病急性变患者骨髓细胞P-gp和bcl-2蛋白表达水平的研究

我们对 2 4例慢性粒细胞白血病急性变 (慢粒急变 )患者骨髓细胞多药耐药糖蛋白Ｐ -ｇｐ和ｂｃｌ -2蛋白表达水平进行了初步研究 ,现将结果报告如下。1　材料和方法1.1　临床资料慢性粒细胞性白血病急性变患者 2 4例 ,男性 16例 ,女性 8例 ,年龄 15～ 6 1岁 ,中位年龄 39岁 ,其中急性淋巴细胞白血病变 (慢粒急淋变 ) 8例 ,急性非淋巴细胞白血病变 (慢粒急非淋变 ) 16例 (Ｍ1型 3例 ,Ｍ2 型 5例 ,Ｍ4 型 2例 ,Ｍ5型 6例 )。对照组为 37例原发性急性白血病患者 ,其中急性淋巴细胞白血病(原发性急淋 ) 15例 ,急性非淋巴细胞白血病 (原发性急非淋…     

HAE方案治疗难治性急性非淋巴细胞白血病

蒽环类药物联合阿糖胞苷治疗急性非淋巴细胞白血病 (急非淋 ,ANLL )可使将近 70 %的患者获得完全缓解 ,但仍有 30 %左右的患者未能达到缓解 ,这部分病例的治疗成为提高急非淋疗效的关键。大剂量阿糖胞苷对部分耐药病例有效 ,但其毒副作用较大 ,应用受到限制。高三尖杉酯碱与蒽环类药物无论结构或作用机理均有不同 ,为探讨高三尖杉酯碱对难治病例的疗效 ,我们自 1 992年 5月至 1 998年5月间对住院病人采用 HAE方案治疗难治性急非淋 2 6例 ,报告如下。1　资料与方法1 .1　病例选择1 .1 .1　一般资料　所有病例均符合 FAB急非淋诊断标准 ,…     

红白血病(M_6)分型诊断管见

法、美、英三国协作组(FAB)于1976年提出的急性非淋巴细胞白血病(ANLL或AML)的形态学分型诊断标准,1985年进行了修正,得到国际上广泛应用,但尚不完善。我国对M_6诊断标准与FAB基本相同,均未考虑到原红及早幼红细胞在红系中的重要性。我们复习了我院形态学诊断M_6的骨髓片,提出对M_6分型的粗浅看法,待商榷。材料和方法将1980年～1992年13年间形态学论为M_6者     

12例急性混合细胞白血病的诊断与疗效分析

目的分析急性混合细胞白血病（HAL）的临床和实验室特征以及疗效和预后。方法根据骨髓细胞形态学、化学染色和免疫表型结果诊断HAL,评价疗效及影响疗效的相关因素。结果12例HAL临床表现可有发热、淋巴结肿大、脾大、肝大、睾丸浸润等,所有患者骨髓增生明显活跃,近半数患者属高白细胞白血病。进行免疫表型检测者11例,B—Ly^＋／My^＋双表型8例、T—Lv^＋／My^＋双表型2例、B＋T—Ly^＋／My^＋型1例。诱导化疗的总CR率64％,6例以兼顾粒、淋二系的方案治疗后全部CR,有4例巩固强化治疗后持续缓解,平均无病生存已19（16～24）个月。结论HAL属特殊类型的白血病,其诊断有赖于对细胞形态学、化学染色和免疫分型的综合判断。对HAL的治疗应该给予兼顾粒、淋二系的诱导和巩固化疗方案。但长期疗效的评价还有待进一步观察。     

全反式维甲酸和三氧化二砷治疗急性早幼粒细胞白血病APL的进展

急性早幼粒细胞白血病(Acute Promyelocytic Leukemia,APL)是 FAB分型中急性非淋巴细胞白血病(Acute non－lymphocytic Leukemia,ANLL)的M3亚型,占所有 ANLL的 10%－15%,具有独特的临床表现,细胞形态学,细胞遗传学和分子生物学特征。近十年的研究表明APL是第一个联合应用全反式维甲酸(all-retinoic acid,ATRA),三氧化二砷(As2O3)和化疗取得成功的人类恶性肿瘤,开创了诱导分化     

100例急性白血病免疫表型特点

目的：研究急性白血病免疫表型的特点，方法：采用间接免疫荧光法分析100例急性白血病患者的免疫表型。结果：T－ALL13例，非T－ALL23例，AML49例，HAL10例，另外还发现了5例标记特殊的急性白血病。与FAB诊断符合率为86％。结论：急性白血病免疫分型可以提高诊断的准确性。     

免疫分型对急性白血病诊断及预后诊断的意义

目的探讨免疫分型对急性白血病的分型诊断及预后诊断的价值。方法采用碱性磷酸酶抗碱性磷酸酶复合物(APAAP)法检测53例急性白血病患者的免疫分型。结果 免疫分型诊断急性髓系白血病(AML)27例,T淋巴细胞白血病(T-ALL)8例,B淋巴细胞白血病(B-ALL)10例,混合型自血病(ABL)6例,未分化型自血病(AUL)2例。T-ALL CD7阳性最常见,B-ALL CD19阳性最常见。AML中抗原表达依次为CD33>CD13>CD14>CD15,CD34/CD34/HLA-DR表达阳性率为64.2％。AML中阳性病例完全缓解率低于阴性病例。ABL及AUL治疗效果差。结论 白血病免疫分型可提高确诊率,并为预后判断提供依据。     

68例成人白血病免疫分型特点分析

[目的]分析68例成人白血病免疫分型特点.[方法]采用单克隆双色或三色直接荧光标记,流式细胞仪检测以CD45-SSC辅助设门,分型根据抗体积分系统.并与FAB分型进行比较.[结果](1)62例成人白血病中未分化型占6.45%(4例),变异型占12.9%(8例).其中急性髓细胞白血病(AML)占69.35%(43例),B细胞型急性淋巴细胞白血病(B-ALL)占16.13%(10例),T细胞型急性淋巴细胞白血病(T-ALL)占6.45%(4例),AML/ALL为3.07:1.CD34的阳性率在62例成人急性白血病和6例慢性粒细胞性白血病急变患者中分别为58.06%和83.33%;免疫分型与FAB分型的符合率为83.9%.[结论]在流式细胞仪上采用CD45-SSC设门法多参数分析白血病免疫分型,并利用抗体积分系统诊断标准可为临床诊断提供重要依据.     

慢性髓系白血病急变期中免疫分型研究

 目的 探讨慢性髓系白血病急变期（CML-BC）的免疫表型特征及应用价值。方法 采用一组单克隆抗体和三色流式细胞术对36例成年人CML-BC骨髓标本进行免疫表型分析。结果 36例CML-BC患者中急性非淋巴细胞白血病变30例（83.33 %）,其中40 %（12／30）伴淋系表达;急性淋巴细胞白血病变急淋变3例（8.33 %）,其中66.67 %（2／3）伴髓系表达;急性混合型白血病变2例;急性未分化型白血病变1例。CML-BC以CD33阳性率最高91.67 %,其次是CD+13 86.11 %,CD+34 61.11 %,CD+7 33.33 %,CD+10 19.44 %,CD+19 16.67 %,CD+2 2.78 %,CD+20 5.56 %及CD+14 5.56 %。CD7与CD34共阳性27.78 %。结论 CML-BC免疫表型复杂,多系表达常见。免疫分型可协助判断CML的急变类型。     

Conversion of acute undifferentiated leukemia phenotypes: analysis of clonal development

The cellular origin of acute undifferentiated leukemia (AUL) is still a matter of controversy. We report on two cases in which the diagnosis of AUL was established according to restricted criteria. Blast cells of both patients showed phenotypic conversion during the course of disease. In one case, within 24 days from starting treatment, the leukemic phenotype changed from AUL to acute myelomonocytic leukemia (FAB L1, TdT+ to FAB M4, TdT-). The initial phenotype of this acute leukemia was characterized by the co-expression of both B-lymphoid and myeloid markers on the same cell. Moreover, analysis of esterase isoenzyme pattern showed the whole spectrum of isoenzymes typically seen in myelomonocytic leukemias already at diagnosis, yet blast cells additionally contained all three isoenzymes of beta-hexosaminidase typically seen in AUL. However, examination of immunoglobulin (Ig) heavy chain gene rearrangement initially and after conversion revealed an identical monoclonal configuration of Ig heavy chain sequences in both samples. The second AUL patient relapsed after allogeneic bone marrow transplantation with common ALL-antigen (CALLA) positive acute leukemia. Subsequent Southern blot analysis showed a novel rearranged Ig fragment compared to the analysis before transplantation indicating that the leukemic clones prior to and after transplantation were not identical. No chromosomal abnormalities were observed in both cases. These data support the view that AUL cells originate from a pluripotent stem cell that is capable to differentiate in the myelomonocytic lineage (patient 1), and confirm the value of Ig gene analysis as marker for cellular clonality.     

Immunohistochemical classification of acute leukemias using peripheral blood smears

Immunophenotypic classification of the acute leukemias (AcL) is of well documented value in those of lymphoid or uncertain origin and of increasing importance in those of nonlymphoid origin. Most of these studies have been performed on viable cell suspensions. To study the efficacy of a simpler immunohistochemical approach to the classification of the acute leukemias requiring only peripheral blood smears, 15 AcL (including three CGL-BC) were studied using an immunoalkaline phosphatase method and a panel of anti-lymphoid and anti-myeloid monoclonal antibodies. Routine cytochemistries were also performed (Sudan black, PAS). Using immunohistochemistry, five cases marked as common ALL (four were undifferentiated by cytochemistry, one ALL), eight cases as ANLL (all ANLL by cytochemistry) and two cases marked only with anti-HLA-DR (AUL by cytochemistry). These results show that immunophenotypic analysis of AUL, ALL and ANLL can be successfully performed even when only air dried peripheral blood smears are available.     

Diversity of acute non-lymphocytic leukemia analyzed with monoclonal antibodies reactive with myeloid differentiation antigens

The expression of myeloid differentiation antigens on acute nonlymphocytic leukemia cells (ANLL) was analyzed with four distinctive monoclonal antibodies (MoAbs); YM-1, HL-1, 20.2 and 20.3. These four MoAbs were shown to recognize different stages of differentiation of myeloid progenitor cells (CFU-GM) and/or mature myeloid cells. Consequently, leukemia cells of 68 adult patients with ANLL were able to be divided into four groups according to their expression of the antigens defined by these MoAbs: group 1 HL-1(-), YM-1(-), 20.2(-), 20.3(-) (13 cases); group 2 HL-1(+), YM-1(-), 20.2(-), 20.3(-) (16 cases); group 3 HL-1(+), YM-1(+), 20.2(-), 20.3(-) (13 cases) and group 4 HL-1(+), YM-1/20.2/20.3(+/(-)) (26 cases). This classification elucidated not only the maturation stages of ANLL but also the diversity of ANLL. When compared with the morphological classification, acute myeloblastic leukemia (AML) cases (M1 and M2 of the FAB classification) were evenly distributed among all four groups. These data suggest that the AML group was composed of heterogeneous leukemias which expressed surface phenotypes of different maturation stages ranging from the most immature stage to the mature stage. In contrast, the mature type (group 4) was composed of not only AML (M1 and M2), but also acute monocytic leukemia (M4) and acute myelomonocytic leukemia (M5). The clinical courses of these 68 patients revealed that the complete remission rate, remission duration and survival time were not significantly different among the four groups.     

Diagnostic value of CD117 in differential diagnosis of acute leukemias

C-kit receptor (CD117) and its ligand, stem cell factor, play a key role in normal hematopoiesis. It has been demonstrated that its expression extremely increases in leukemias with myeloid commitment. We analyzed findings on CD117 expression together with other myeloid related markers in 203 de novo acute leukemias, referred to Iranian immunophenotyping centers: Iranian Blood Transfusion Organization (IBTO) and Baghiatallah Hospital (BH). All cases were characterized based on the French American British cooperative group (FAB) and European Group for Immunological Classification of Leukemias (EGIL). The cases comprised of 111 acute myeloblastic leukemia (AML), 86 acute lymphoblastic leukemia (ALL), and 6 acute undifferentiated leukemia (AUL). CD117 was positive in 75 % of AML and 50 % of AUL, whereas none of the ALL cases was positive for this marker. Although CD117 was positive in 100 % of M5a cases, no M5b positive was found (p?=?0.036). The calculated specificity for myeloid involvement was 100 % for CD117 and CD33, and 98 % for CD13 and CD15 (p?<?0.001). The calculated sensitivity for myeloid involvement was 83, 76, 64, and 41 % for CD13, CD117, CD33, and CD15, respectively (p?<?0.001). We concluded that CD117 expression is a specific and rather sensitive marker for differential diagnosis between AML and ALL, and except for M5 subtypes, it fails to determine FAB subtypes; lack of expression in M5 can identify M5b. Therefore, it should be included in the routine primary panel for diagnosis of acute leukemias.     

100例急性白血病免疫表型特点

目的 :研究急性白血病免疫表型的特点。方法 :采用间接免疫荧光法分析 10 0例急性白血病患者的免疫表型。结果 :T- AL L 13例 ,非 T- AL L 2 3例 ,AML 49例 ,HAL 10例 ,另外还发现了 5例标记特殊的急性白血病。与FAB诊断符合率为 86 %。结论 :急性白血病免疫分型可以提高诊断的准确性。     

Combination chemotherapy of N(4)-behenoyl-1-beta-arabinofuranosylcytosine, aclarubicin, 6-MP, and prednisolone (BH-AC. AMP therapy) for adult acute non-lymphocytic leukemia

Sixteen of 20 patients(80%) with adult ANLL treated with B H-AC X AMP therapy attained complete remission (CR). According to the FAB classification, CR rate was 6 out of 8 (75%) for M1, 3 out of 5 (60%) for M2, 2 out of 2 (100%) for M3, and 5 out of 5 (100%) for M4. The median of remission duration in 16 patients who attained CR was 8 months and appeared to be longer in patients with M2, rather than other types, of leukemia than in those with the other types of leukemia. BH-AC X AMP therapy is highly effective for remission induction in adult ANLL and long term disease free survival could be expected by addition of appropriate maintenance therapy.