表现异质的大颗粒淋巴细胞白血病6例临床分析

目的提高对大颗粒淋巴细胞白血病（LGLL）的认识及鉴别诊断。方法对6例临床表现显著异质的LGLL的临床特征及实验室检查并结合文献进行临床分析。结果2例惰性T—LGLL和1例慢性NK—LGLL,临床呈慢性病程,主要表现为贫血和脾肿大,对治疗反应好;1例侵袭性T—LGLL及2例侵袭性NK—LGLL,临床以全身症状明显并有肝、脾肿大,预后极差。4种LGLL分别有各自的免疫表型特点。结论LGLL的诊断需结合临床、细胞学检测、免疫分型综合分析并加与鉴别诊断。     

免疫分型对白血病诊断的临床意义

目的研究急慢性白血病免疫分型特点，更好地指导临床诊断及治疗。方法采用形态学、免疫学检测方法，对471例急慢性白血病进行回顾性分析总结。结果418例急性白血病(AL)中24例形态学分型与免疫分型诊断不符合，其中11例通过免疫分型纠正，诊断准确性提高2．6％。53例慢性白血病(CL)中慢粒免疫分型与形态学分型基本符合，慢淋多为B淋系表达。结论免疫分型为白血病的诊断及治疗提供了更全面的信息资料。     

应用形态学、核型、免疫学和分子生物学方法联合检测急性髓细胞性白血病M_(2b)的研究

对20例急性髓细胞性白血病（AML）M2b亚型进行了形态学、免疫学、细胞遗传学和分子生物学方法（MICM）的联合检测。结果显示，形态学分型确诊率为90％（18／20），免疫学分析示80％（16／20）有髓系抗原表达，具有其典型特征者为40％（8／20）。染色体异常t（8；21）检出率为95％（19／20），采用RTPCR技术检测全部病例均显示AWL1－ETO融合基因阳性，提示在MICM联合诊断中，融合基因检测对AML－M2b的诊断，治疗及微小残留病监测有重要意义。     

免疫学和细胞遗传学诊断FAB标准难以分型的急性白血病

为探讨免疫分型及细胞遗传学在急性白血病（AL）中的诊断价值，对30例FAB标准难以分类的AL患者进行了检查。结果显示FAB难以分型的AL可以分成三组：（1）M2／M3难以鉴别，主要根据t（8；21）、t（15；17）等染色体异常诊断；（2）过氧化物酶（POX）阴性的AL，主要根据免疫分型诊断。主要包括M0、M7、部分M1、M5等急性髓细胞白血病及急性淋巴细胞白血病；（3）杂合型急性白血病，免疫分型是最重要的诊断手段。结果提示免疫分型及染色体分析是形态学诊断的重要补充，应成为AL的常规诊断手段。     

新疆314例恶性淋巴瘤的临床及病理资料分析

目的研究恶性淋巴瘤的临床、病理特点。方法根据WHO（2008）分类标准对314例恶性淋巴瘤患者进行临床及病理资料分析。结果非霍奇金淋巴瘤（NHL）271例。43例HL中以混合细胞性经典霍奇金淋巴瘤为主。NHL中,B细胞淋巴瘤多于T细胞淋巴瘤。结外淋巴瘤少于结外淋巴瘤.NHL中发病率较高的淋巴瘤类型依次为：弥漫大B细胞淋巴瘤,慢性淋巴细胞性白血病/小淋巴细胞性淋巴瘤。不能分类的B细胞淋巴瘤,黏膜相关淋巴组织细胞淋巴瘤,NK/T细胞淋巴瘤,外周T细胞淋巴瘤。少数民族和汉族均以弥漫大B细胞淋巴瘤为主。结论应用WHO（2008）分类对恶性淋巴瘤进行分析研究,具有重要的临床意义。     

胞浆抗原检测在急性双表型白血病中的诊断价值

目的 探讨胞浆抗原检测在急性双表型白血病(BAL)中的临床诊断、鉴别诊断价值。方法 采用流式细胞术(FCM)对500例初治急性白血病(AL)患，统一进行常规一线抗体的检测，其中对一线抗体诊断不清的80例加做二线胞浆抗原染色。结果 80例加做胞浆抗原检测后，依据EGIL(1998年)积分系统，符合BAL有10例(10／500例，2．0％)：T系／B系双表型3例(cCD3、cCD79a双阳性)、T系／髓系双表型3例(cCD3、MPO双阳性)、B系／髓系双表型4例(cCD79a、MPO双阳性)；符合急性未分化型白血病1例(0．2％)；符合AL伴系表达69例。结论 对诊断不清的AL病例，加做胞浆抗原检测，对进一步提高白血病的诊断水平，特别是对未分化型、双表型以及双表型的分类诊断、双表型与伴系表达白血病的鉴别诊断具有重要价值。     

以皮肤病变为首发症状的侵袭性NK细胞白血病1例报道并文献复习

侵袭性NK细胞白血病（aggressive NK cell leukemia,ANKL）为大颗粒淋巴细胞白血病（large granular lymphocyte leukemia,LGLL）中一型,为CD3-NK系LGL增生所致。1990年由Imamura,N．等首次提出,2001年WHO造血组织和淋巴组织肿瘤分类中将其列入成熟（外周）T／NK细胞肿瘤,成为一个独立的病种。ANKL多见于亚洲人群,主要见于少年和青壮年,有证据表明其发病与EB病毒相关。患者常表现为发热、肝脾肿大、     

流式细胞仪检测细胞因子的方法学研究及临床应用

目的：录求一种新的检测细胞因子的方法，探讨小儿T淋巴细胞性急性白血病（T－ALL）细胞内的干扰因子IL－10和IL－12的表达及其临床意义。方法：应用流式细胞仪（FCM）测定七例T－ALL患儿白血病细胞和正常T细胞中的IL－10和IL－12表达。结果：IL－2在T－ALL患儿非白血病T细胞中部分有表达，而正常对照T细胞和T－ALL患儿白血病细胞均无表达。IL－10在T－ALL患儿正常T细胞、白血病细胞和正常对照T细胞中均未检测到表达。结论：应用FCM对白血病患儿细胞内细胞因子的检测可为临床诊治提供客观的实验数据。急性淋巴细胞性白血病（ALL）患儿体内可能存在TH1／TH2样细胞因子产生的失平衡状态，这可能是ALL发病中的重要机制之一。     

CD56在非霍奇金淋巴瘤中的表达和意义

目的探讨CD56在非霍奇金淋巴瘤中NK/T细胞淋巴瘤的表达和意义。方法应用组织形态学及免疫组织化学分析对29例我院既往诊断的恶性淋巴瘤组织进行HE、PSA染色,同时作CD56的免疫表型分析。结果10例弥漫性大B淋巴瘤和6例外周T细胞淋巴瘤肿瘤细胞CD56表达均阴性,12例NK/T细胞淋巴瘤CD56表达阳性,1例NK/T细胞淋巴瘤可疑。结论CD56可作为非霍奇金淋巴瘤中NK/T细胞淋巴瘤分类鉴别的重要指标。     

应用形态学,核型,免疫学和分子生物学方法联合检测急性髓细胞性白血 …

对２０例急性髓细胞性白血病（ＡＬＭ）Ｍ２ｂ亚型进行了形态学，免疫学，细胞遗传学和分子生物学方法（ＭＩＣＭ）的联合检测，结果显示，形态学分型确诊率为９０％（１８／２０），免疫学分析示８０％（１６／２０）有髓系抗原表达，具有其典型征者４０％（８／２０），染色体异常ｔ（８；２１）检出率为９５％（１９／２０），采用ＲＴ－ＰＣＲ技术检测全部病例均显示ＡＭＬ１－ＥＴＯ融合基因阳性，提示ＭＩＣＭ联合诊断中，融合     

Rituximab in B-cell disorders other than non-Hodgkin's lymphoma

Rituximab is a human/mouse chimeric monoclonal antibody that binds to the CD20 antigen and is expressed at all stages of B-cell development. Rituximab has demonstrated efficacy as monotherapy and in combination with chemotherapy in the treatment of both indolent and aggressive non-Hodgkin's lymphoma (NHL). Rituximab treatment results in rapid depletion of B-cells and this has led to the consideration of other B-cell disorders as candidates for rituximab therapy. Recent studies have demonstrated the efficacy of rituximab in a variety of such disorders, including chronic lymphocytic leukemia (CLL), post-transplant lymphoproliferative disorder (PTLD), Waldenstr?m's macroglobulinemia (WM), multiple myeloma (MM), idiopathic thrombocytopenic purpura (ITP), hairy-cell leukemia (HCL) and cold agglutinin disease (CAD). In patients with CLL, increasing the dose and/or frequency of rituximab treatment has given improved response rates compared with the standard dose schedule used in NHL, and combination immunochemotherapy has yielded an overall response rate of 92% (with a 60% complete response rate). Clinical trials have also demonstrated evidence of efficacy for rituximab in PTLD, WM and relapsed or refractory ITP. Efficacy of rituximab in CAD and relapsed or refractory HCL has also been demonstrated in small studies and case reports. Available data thus indicate that rituximab can be an effective therapy in a wide range of CD20+ lymphoid disorders.     

Enzastaurin hydrochloride for lymphoma: reassessing the results of clinical trials in light of recent advances in the biology of B-cell malignancies

Introduction: The B-cell receptor (BCR) is critical for the development and persistence of B-cell non-Hodgkin lymphoma (B-NHL). Protein kinase C-beta (PKC-?) has been identified as one of the key signaling hubs downstream of the BCR and constitutes a valuable target in B-NHL. As a potent PKC-? inhibitor, enzastaurin is currently being tested in Phase II/III trials. Areas covered: This review summarizes the latest results and ongoing clinical trials with enzastaurin in light of basic scientific advances in the understanding of various lymphoid cancers, including diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma (MCL), follicular lymphoma (FL), chronic lymphocytic leukemia (CLL) and Waldenstr?m's macroglobulinemia (WM). Expert opinion: While its continued clinical development is uncertain, enzastaurin should be regarded as a stepping stone for the development of future therapies; indeed, the recent research has provided valuable insight into the possible molecular mechanisms that explain its limited clinical activity especially in the treatment of DLBCL and MCL. It should be noted that there is still some interest in enzastaurin, in combination, for the treatment of WM.     

Duvelisib for the treatment of chronic lymphocytic leukemia

ABSTRACT

Introduction

Duvelisib, a first in class, oral, dual PI3 k-delta/gamma inhibitor recently received FDA approval for previously treated CLL (chronic lymphocytic leukemia)/SLL (small lymphocytic lymphoma) and follicular lymphoma. Data coming from the phase III ‘DUO’ trial, in fact, showed a superior progression-free survival (PFS) in CLL patients treated with duvelisib compared to ofatumumab     

Enzastaurin hydrochloride for lymphoma: reassessing the results of clinical trials in light of recent advances in the biology of B-cell malignancies

Introduction: The B-cell receptor (BCR) is critical for the development and persistence of B-cell non-Hodgkin lymphoma (B-NHL). Protein kinase C-beta (PKC-β) has been identified as one of the key signaling hubs downstream of the BCR and constitutes a valuable target in B-NHL. As a potent PKC-β inhibitor, enzastaurin is currently being tested in Phase II/III trials.

Areas covered: This review summarizes the latest results and ongoing clinical trials with enzastaurin in light of basic scientific advances in the understanding of various lymphoid cancers, including diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma (MCL), follicular lymphoma (FL), chronic lymphocytic leukemia (CLL) and Waldenström's macroglobulinemia (WM).

Expert opinion: While its continued clinical development is uncertain, enzastaurin should be regarded as a stepping stone for the development of future therapies; indeed, the recent research has provided valuable insight into the possible molecular mechanisms that explain its limited clinical activity especially in the treatment of DLBCL and MCL. It should be noted that there is still some interest in enzastaurin, in combination, for the treatment of WM.     

105例儿童白血病的免疫分型与形态结果分析

目的 研究儿童白血病骨髓细胞形态学及免疫分型之间的关系.方法 对本院2016年新收治的105例儿童白血病进行回顾性分析,总结其流行病学特点及白细胞抗原的表达情况.结果 (1)ALL、AML患儿男女比例分别为2.13∶1,1.44∶1;中位发病年龄分别为3岁,2岁.(2) 105例儿童白血病FAB合型:初诊急性髓系白血病(AML) 22例,急性淋巴细胞白血病(ALL) 72例,未分类急性白血病2例,慢性粒细胞白血病(CML)4例,幼年型粒-单核细胞白血病(JMML)2例,神经母细胞瘤(NB)骨髓浸润2例,淋巴瘤骨髓转移1例;72例急慢性白血病免疫分型:急性髓系白血病(AML)16例,急性B淋巴细胞白血病(B-ALL) 47例,急性T淋巴细胞白血病(T-ALL)4例,慢性粒细胞白血病(CML)2例,幼年型粒-单核细胞白血病(JMML)1例,淋巴瘤骨髓转移1例,未见明显异常1例.(3)免疫分型与形态学分型完全符合率为91.67％(66/72),部分符合率为2.78％(2/72),完全不符合率为5.55％(4/72).(4)CD19、CD34、CD10、HLA-DR在儿童急性B淋巴细胞白血病中表达高,分别为100.00％(47/47)、80.85％(38/47)、89.36％(42/47)、100.00％(47/47);CD20在儿童急性B淋巴细胞白血病中表达均为12.77％(6/47);而CD13和CD33在儿童急性B淋巴细胞白血病中的表达率均为21.28％(10/47).结论 免疫分型对儿童白血病的诊断有重要作用,但并不能完全取代形态学分析,同时弥补了骨髓常规检查的不足.     

某医院1981～2005年接诊白血病患者的发病资料分析

目的研究医院收治白血病特点。方法回顾性分析该院1981~2005年诊断的急性白血病患者325例、慢性白血病患者79例的资料。结果①急性白血病男性患者较女性患者多见(男∶女=1.43∶1),其中急性髓性白血病(AML)患者较急性淋巴细胞白血病(ALL)患者多见(AML∶ALL=1.8∶1);慢性白血病中慢性髓性白血病(CML)较慢性淋巴细胞白血病(CLL)多见(CML∶CLL=3.65∶1);②AML患者以青中年为主(78.5%),ALL患者以青中年(71.6%)和儿童为主(21.6%);CML患者以青中年为主(88.7%),CLL患者主要是60岁以上的老年人居多;③该院急性白血病(AL)以农村患者为主,慢性白血病以城市患者为主;儿童和老年白血病以城市患者为主;④该院儿童白血病发病与季节无明显相关;⑤化学性毒物、射线和免疫抑制剂的使用可能是白血病患者的主要危险因素。结论该结果与文献中关于白血病的某些研究成果相符,但也有一些与之不同,笔者认为有待于增加病例数,做进一步观察和分析。     

急性淋巴细胞白血病24例形态学与免疫学分型分析

目的 探讨急性淋巴细胞白血病（ALL）形态学与免疫学分型的关系,以提高白血病的诊断水平.方法 针对24例ALL患者行骨髓细胞形态学、骨髓细胞化学染色及免疫分型检测,比较2者在ALL分型上的异同.结果 24例ALL患者中,FAB分型L1 15例、L2 7例、L3 2例;WHO免疫分型T-ALL 7例、B-ALL 17例.结论 免疫学分型是诊断ALL的重要手段,是对形态学分型的补充和修正,它对ALL患者的诊断、治疗和判断预后具有重要的指导意义.     

Therapeutic effects of a combination treatment with cefmetazole and netilmicin against infections complicated with hematological disorders

The efficacy and safety of a combination regimen using cefmetazole (CMZ) and netilmicin (NTL) were evaluated in the treatment of infections complicated with hematological disorders. Primary diseases in 31 patients included in the evaluation were acute myelocytic leukemia (3 cases), acute lymphocytic leukemia (2 cases), malignant lymphoma (14 cases), chronic myelocytic leukemia (2 cases), chronic myelocytic leukemia blast crisis (4 cases), myelodysplastic syndrome (2 cases), aplastic anemia (3 cases), and malignant histiocytosis (1 case). Complicated infections included 29 cases of suspected septicemia, 1 case of septicemia and 1 case of pneumonia. Clinical responses were excellent in 6 (19.4%), good in 12 (38.7%), fair in 1 (3.2%) and poor in 12 (38.7%). The total clinical efficacy rate was 58.1%. No significant effect of initial neutrophil counts was observed on response rates. Patients who showed increasing neutrophil counts during therapy had higher response rates than those in whom the neutrophil count decreased or remained unchanged at levels less than 500/mm3 in after neutrophil counts. No side effects were observed in any of the 31 patients. In conclusion, this combination therapy of CMZ and NTL thus appears to be useful and safe in therapies for infections complicated with hematological disorders.     

应用免疫组化技术进行白血病免疫分型的初步研究

本文用 APAAP 免疫组化方法对20例急性白血病进行免疫分型初步研究,结果表明17例(85%)急性白血病有特征性或相关性标志,其中非 T-ALL10例(Ⅰ、Ⅱ、Ⅲ型为3、2和5例),T-ALL 2、AML 1、AMMoL 及双表型各2例。不能确定表型1例(5%)及无标志者2例(10%),认为免疫组化有利于鉴别形态特殊的白血病。临床(瑞氏染色)诊断、细胞化学及免疫组化检测三项结果一致者9/16(56.25%),临床诊断与免疫组化两项一致者3例,临床诊断与细胞化学结果一致,而免疫表型不能确定及无标志者2例,三项结果均不一致者2例。10例住院患者的临床表现及预后与免疫表型无相关性。