急性白血病纤溶系统检测的临床意义

目的 探讨急性白血病（ＡＬ）患者纤溶系统的异常。方法 发色底物法和ＥＬＩＳＡ法测定９３例患者血浆一系列纤溶指标。结果 患者血浆组织纤溶酶原激活物活性、二聚体（Ｄ－Ｄ）水平显著升高;纤溶酶原活性（ＰＬＧ）、ａ２抗纤溶酶活性（ａ２－ＰＩ）、纤溶酶原派激活抑制物活性（ＰＡＩ）水平降低;缓解后均恢复正常。结论 ＡＬ患者存在纤溶系统的激活,部分指标与ＡＬ分型、出血程度和预后有关。     

急性早幼粒细胞白血病纤溶异常研究

急性早幼粒细胞白血病纤溶异常研究胡炯沈志祥仇倩瑶王学锋王鸿利出血是急性早幼粒细胞白血病（ＡＰＬ）的常见临床症状。通常认为ＡＰＬ的出凝血异常主要是并发ＤＩＣ，但近年的研究提示，纤溶系统异常是ＡＰＬ另一主要特征。我们通过了对纤溶指标的系统检测和动态观察了...     

急性早幼粒细胞白血病纤溶异常与治疗进展

急性早幼粒细胞白血病 (ＡＰＬ)出血的原因甚为复杂 ,其中过度纤溶是引起出血的重要原因之一 ,自本世纪七十年代至今 ,已对其机制及相应治疗进行了深入的研究 ,本文就这些方面的研究综述如下。1　ＡＰＬ纤溶研究概况1 1　ＡＰＬ患者纤溶系统组成成分的研究概况1 1 1　纤溶酶与纤溶酶原　有作者发现在ＡＰＬ患者血浆中纤溶酶原水平是降低的[1 ,2 ] ,而胡炯等[3] 则检测 1 5例初治、 8例复治和 1 4例持续缓解ＡＰＬ患者纤溶酶水平分别为1 1 4 3 %± 2 5 7%、 1 1 5 8%± 2 1 9%、 1 0 0 6%± 2 3 0 %与正常值 (75 %～ 1 4 0 % )相比无明…     

急性早幼粒细胞白血病出凝血及纤溶指标观察

急性早幼粒细胞白血病也就是急性髓系白血病的M_3型,出血是其常见的临床症状,最易并发DIC。观察M_3出凝血及纤溶指标的变化,可为临床用药提供依据。     

117例急性白血病患者纤溶抑制物研究

目的 探讨急性白血病(AL)患者凝血酶激活的纤溶抑制物(TAFI)、纤溶酶原激活剂抑制物(PAI)、α2抗纤溶酶(α2:-PI)等纤溶抑制物的变化及其临床意义.方法 采用ELISA法测定117例AL患者和50名正常对照的PAI-1抗原(PAI-1:Ag)含量、TAFI抗原含量;采用发色底物法测定PAI活性、α2-PI活性、TAFI活性.结果 ①AL患者的α2-PI活性水平明显低于对照组,其中急性淋巴细胞白血病患者[(96.8±21.2)%]较对照组[(129.1±13.1)%]下降更明显;②急性髓系白血病患者PAI-1:Ag含量[(37.8±9.2)μg/L]高于对照组[(33.8±4.9)μg/L];③急性早幼粒细胞白血病患者PAI-1:Ag含量[(37.8±9.0)μg/L]高于对照组,TAFI活性水平[(13.3±4.8)mg/L]低于对照组[(16.9±2.6)mg/L],急性单核细胞白血病患者PAI-1:Ag含量[(39.9±11.6)μg/L]高于对照组;④复发/难治组的PAI-1:Ag含量[(39.6±11.6)μg/L]高于对照组;⑤明显出血组TAFI活性水平[(13.2±5.3)mg/L]低于对照组及无出血组[(17.0±4.6)mg/L];⑥TAFI活性与出血程度呈显著负相关,r=-0.276(P《0.05).结论 α2-PI及TAFI活性降低是AL出血的原因之一,且TAFI活性与卅血程度呈显著负相关.     

急性心肌梗死时纤溶系统的改变与溶栓治疗

急性心肌梗死 (ＡＭＩ)已被确认 90 %是由冠状动脉内血栓形成而引起的 ,及时的溶栓治疗可以缩小梗死面积 ,改善预后。ＡＭＩ时改变血浆凝血及纤溶系统活性 ,观察溶栓与抗凝对预后的影响已成为国内外学者关注的重点。因此 ,我们对 3 6例急性心肌梗死时溶栓治疗期间纤溶系统活性的变化进行了研究。1　材料与方法1.1　材料　选择发病 2 4小时内符合 1979年诊断制订的ＡＭＩ标准[1] 的急性心肌梗死患者 3 6例 ,男 2 4例 ,女 12例 ,年龄 45～60岁 ,平均年龄 ( 4 5 .2± 5 .7)岁。随机分为尿激酶 (ＵＫ )组 2 0例和去纤酶 (ＤＦ)组 16例。选择同期…     

急性心肌梗死患者溶栓治疗前后凝血与纤溶系统的变化

目的探讨急性心肌梗死(AMI)患者溶栓治疗前后不同时间段凝血与纤溶系统的变化情况.方法对41例经溶栓治疗的AMI患者分别于治疗前及治疗后4、8、12、48 h和3、7 d共7次抽取静脉血,分别检测凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)、纤维蛋白原(Fg)、D-二聚体(DD)、纤溶酶原(PLG)、α2-纤溶酶抑制物(α2-PI)、组织型纤溶酶原激活物(t-PA)和纤溶酶原激活物抑制剂-1(PAI-1)等指标的活性或含量.结果所有患者经溶栓治疗后,均导致PT、APTT的明显延长,t-PA活性、DD含量的明显增高,PLG、α2-PI、PAI-1活性和Fg含量的明显降低(与溶栓前比较,P均<0.01).但这种变化为时较短,至溶栓后12 h,各项指标已出现不同程度的恢复,t-PA与PAI-1已回复至溶栓前水平.结论凝血与纤溶活性的变化与溶栓疗效关系密切,应用时监测PLG、α2-PI、t-PA、PAI-1、Fg和DD等指标,对判断溶栓疗效有重要价值.     

白血病患者纤溶活性的测定

目的：为了解白血病患者血浆纤维蛋白原（ＦＩＢ）、纤溶酶原（ＰＬＧ）和Ｄ－二聚体（Ｄ－ｄｉｍｅｒ）的活性变化。方法以ＳＴＡ Ｃｏｍｐａｃｔ全自动血凝仪及ＡＣＬ－２００型自动血凝仪测定２０例正常健康人、１２例急性原始粒细胞白血病（Ｍ１、Ｍ２）、１０例急性早幼粒细胞白血病（Ｍ３）、８例急性淋巴细胞白血病（ＡＬＬ）、６例慢性粒细胞白血病（ＣＭＬ）患者血浆的纤维蛋白原含量（ＦＩＢ）、纤溶酶原活性（ＰＬＧ）及     

Chromosome abnormalities and prognosis in childhood acute leukemia]

The chromosomal classification system of childhood acute leukemia according to ploidy or structural chromosomal changes of leukemia cells is clinically useful for the prediction of the treatment outcome. While patients with hyperdiploid (greater than 50 chromosomes) ALL enjoy the best prognosis, those with hypodiploid or pseudodiploid ALL are expected to have unfavorable clinical outcome. Among various translocations or deletions in ALL, t(4;11), t(9;22) and t(8;14) predict the worst clinical outcome. AML patients with t(8;21) or inv (16) have better 50% survival than those with other chromosome changes or normal diploidy. Chromosome analysis of leukemia cells should be included in the diagnostic workup of childhood acute leukemia.     

Chromosomal abnormalities in myelodysplastic syndromes and acute myeloid leukemia

Clonal chromosome abnormalities are found in more than half the patients with hematologic malignancies. Karyotype is an independent prognostic factor in these patients. Cytogenetic findings correlate significantly with morphologic, immunologic, and clinical features as well as response to treatment, remission duration, and survival. The number of different cytogenetic abnormalities is enormous; however, many cytogenetic findings frequently occur in a given disease (e.g., abnormalities of 5 or 7 in 75% to 90% of patients with therapy-related AML). Some abnormalities are found only in myeloid malignancies, for example, the t(8;21)(q22;q22) and rearrangements of chromosome 16q22, both of which have a good prognosis. Other abnormalities usually are found in both myeloid and lymphoid malignancies, for example, the t(4;11)(q21;q23) and t(9;22)(q34;q11), both of which have a poor prognosis. The Human Gene Mapping Conferences have compiled much cytogenetic data and produced several interesting correlations in myeloid malignancies: rearrangements of 3q21-26 with myeloid proliferations associated with environmental exposure (similar to abnormalities of 5q, 7q, 12p, and 17q), aberrations of 12p, 11q13 and 11q23 with both myeloid and lymphoid disorders, and the lack of myeloid involvement and abnormalities of chromosomes 14 and 18. In conclusion, cytogenetic analysis of neoplastic cells at diagnosis for patients with MDS, AML, and SAML is required for appropriate diagnosis and treatment. The use of chromosome abnormalities to separate patients into high- and low-risk groups eventually may allow us to be more effective in selecting curative therapy.     

Clinical and biological importance of cytogenetic abnormalities in childhood and adult acute lymphoblastic leukemia

Among the approximately 7,000 cytogenetically abnormal childhood and adult B- and T-lineage acute lymphoblastic leukemias (ALL) published to date, numerous recurring chromosomal aberrations and abnormality patterns have been identified, and it has been clearly shown that the cytogenetic features often correlate closely with specific morphologic, immunophenotypic, and clinical parameters. Thus, karyotypic investigations are now routinely performed for diagnostic and prognostic purposes in ALL, with the chromosomal abnormalities/cytogenetic patterns playing a major role for proper risk assessment and choice of treatment. At the same time, the cytogenetic analyses have resulted in the identification of more than 70 different genes, located at the breakpoints of ALL-associated structural chromosomal abnormalities, that are causally implicated in the leukemogenic process. Hence, the genetic studies have also improved our understanding of the mechanisms of leukemogenesis. However, the almost staggering amount of cytogenetic information presently available has made it increasingly difficult to obtain a general overview of the clinical and biological importance of karyotypic patterns in ALL. Here, we summarize and review the cytogenetic features of childhood and adult ALL, with emphasis on their molecular genetic consequences and their clinical impact.     

Clinical and biological importance of cytogenetic abnormalities in childhood and adult acute lymphoblastic leukemia

Among the approximately 7,000 cytogenetically abnormal childhood and adult B- and T-lineage acute lymphoblastic leukemias (ALL) published to date, numerous recurring chromosomal aberrations and abnormality patterns have been identified, and it has been clearly shown that the cytogenetic features often correlate closely with specific morphologic, immunophenotypic, and clinical parameters. Thus, karyotypic investigations are now routinely performed for diagnostic and prognostic purposes in ALL, with the chromosomal abnormalities/cytogenetic patterns playing a major role for proper risk assessment and choice of treatment. At the same time, the cytogenetic analyses have resulted in the identification of more than 70 different genes, located at the breakpoints of ALL-associated structural chromosomal abnormalities, that are causally implicated in the leukemogenic process. Hence, the genetic studies have also improved our understanding of the mechanisms of leukemogenesis. However, the almost staggering amount of cytogenetic information presently available has made it increasingly difficult to obtain a general overview of the clinical and biological importance of karyotypic patterns in ALL. Here, we summarize and review the cytogenetic features of childhood and adult ALL, with emphasis on their molecular genetic consequences and their clinical impact.     

15例伴11p15异常急性髓系白血病患者的临床特征分析

目的 分析伴11p15异常的急性髓系白血病(AML)患者细胞遗传学和临床特征,并探讨其对预后的影响.方法 回顾性分析1994至2010年于浙江大学医学院附属第一医院住院及门诊伴11p15异常AML患者的临床及实验室资料,并进行预后分析.结果 在1725例初发AML患者中检出15例11p15异常,检出率为0.87％,其中t(7;11)异常6例,t(1;11)和t(11;12)异常各2例,t(2;11)、t(11;11)、t(11;14)、del( 11)及inv(11)异常各1例.15例11p15异常患者中,M2 10例,M53例,M1和M4各1例.6例t(7;11)异常患者均为M2型,其中5例患者白血病细胞可见Auer小体.15例患者中12例进行了化疗,7例获得缓解,但中位持续完全缓解时间仅为8(4 ～12)个月;其中13例患者均已死亡,中位生存期为11(2～19)个月.结论 11p15异常为AML中少见的再现性染色体异常,以t(7;11)最常见,并有独特的临床及实验室特征;伴11p15异常的AML患者治疗效果差,预后不良.     

11q23异常儿童急性白血病的临床及实验室分析

目的　研究伴 11q2 3异常儿童急性白血病 (AL)的形态学、免疫学、细胞遗传学与其临床特征和预后的关系。方法　对 32 0例AL中 18例伴有 11q2 3异常的患儿进行回顾性分析 ,包括细胞形态学观察、流式细胞仪细胞免疫表型检测、R带核型分析和临床预后。以同期核型正常的 2 0例AL患儿作为对照。结果　 18例伴 11q2 3异常AL患儿中 14例为急性淋巴细胞白血病 (ALL) ,4例为急性髓系白血病 ,其中M5b3例 ,M2a1例。进行免疫表型分析的 16例患儿中 ,13例有淋系抗原表达 ,3例除表达髓系抗原外均显示CD34 阳性。异常核型有 6种 :t(4 ;11) (q2 1;q2 3) 6例 ;t(10 ;11) (p13;q2 3) 3例 ;t(11;19) (q2 3;p13) 1例 ;t(11;17) (q2 3;q11) 1例 ;t(8;11) (q2 3;q2 3) 1例 ;del(11) (q2 3) 6例。本组AL完全缓解 (CR)率为 72 .2 % ,与对照组的 80 .0 %相比 ,差异无显著性 (P >0 .0 5 ) ,而死亡率为 6 1.1% ,与对照组的 2 5 .0 %相比 ,差异有显著性 (P <0 .0 5 )。结论　 11q2 3异常主要见于儿童ALL和急性单核细胞白血病 ,具有较为独特的临床、血液学和预后特点。     

Fibrinolytic abnormalities in acute respiratory distress syndrome (ARDS) and versatility of thrombolytic drugs to treat COVID‐19

The global pandemic of coronavirus disease 2019 (COVID‐19) is associated with the development of acute respiratory distress syndrome (ARDS), which requires ventilation in critically ill patients. The pathophysiology of ARDS results from acute inflammation within the alveolar space and prevention of normal gas exchange. The increase in proinflammatory cytokines within the lung leads to recruitment of leukocytes, further propagating the local inflammatory response. A consistent finding in ARDS is the deposition of fibrin in the air spaces and lung parenchyma. COVID‐19 patients show elevated D‐dimers and fibrinogen. Fibrin deposits are found in the lungs of patients due to the dysregulation of the coagulation and fibrinolytic systems. Tissue factor (TF) is exposed on damaged alveolar endothelial cells and on the surface of leukocytes promoting fibrin deposition, while significantly elevated levels of plasminogen activator inhibitor 1 (PAI‐1) from lung epithelium and endothelial cells create a hypofibrinolytic state. Prophylaxis treatment of COVID‐19 patients with low molecular weight heparin (LMWH) is important to limit coagulopathy. However, to degrade pre‐existing fibrin in the lung it is essential to promote local fibrinolysis. In this review, we discuss the repurposing of fibrinolytic drugs, namely tissue‐type plasminogen activator (tPA), to treat COVID‐19 associated ARDS. tPA is an approved intravenous thrombolytic treatment, and the nebulizer form has been shown to be effective in plastic bronchitis and is currently in Phase II clinical trial. Nebulizer plasminogen activators may provide a targeted approach in COVID‐19 patients to degrade fibrin and improving oxygenation in critically ill patients.     

Chromosomal abnormalities in acute leukemias

Conventional cytogenetic techniques are the standard for the diagnosis and follow-up of patients with AML and ALL. Some characteristic translocations associated with various groups of AML diagnoses, such as t(8;21), t(15;17), and inv(16) for M2, M3, and M4eo, respectively, have been recognized for years. The most common cytogenetic abnormality found in childhood ALL and hyperdiploid adult ALL is t(9;22). Future directions include increased use of FISH and molecular diagnostic techniques. The clinical cytogenetics laboratory plays a major role in the diagnosis and management of AML and ALL.     

Electrocardiographic abnormalities in acute pancreatitis.

It has been reported that electrocardiographic abnormalities may be associated with acute pancreatitis. However, the data are lacking or sketchy. The aim of this study was to assess the frequency and type of electrocardiographic abnormalities present in patients with acute pancreatitis. Fifty-six consecutive patients with acute pancreatitis and without previous history of heart disease were studied. Eleven patients had arterial hypertension. Forty-one patients had mild pancreatitis and 15 had the severe form of the disease. On admission, all patients underwent a standard 12-leads electrocardiogram and a serum electrolyte determination. Nineteen healthy subjects were also studied as controls. Twenty-seven patients (48.2%) (10 with severe pancreatitis and 17 with mild pancreatitis) had a normal electrocardiogram. In the remaining 29 patients (51.8%), one patient with severe pancreatitis had atrial extrasystoles and eight had bradycardia (less than 60 beats/minute) (two with severe pancreatitis and six with mild pancreatitis); 14 patients had changes of the T-wave and/or the ST-segment (two with severe pancreatitis and 12 with mild pancreatitis); seven patients showed disturbances of the intraventricular conduction (one with severe pancreatitis and six with mild pancreatitis): four had left anterior hemiblock, two had complete left bundle branch block and one had left anterior hemiblock and incomplete right bundle branch block; one patient with mild pancreatitis had atrioventricular block (first degree). No differences in heart rate, RR interval, PR interval and QT interval were found when patients with acute pancreatitis were compared with healthy subjects, nor when patients with severe pancreatitis were compared with those having the mild form of the disease. Seventeen of the 29 patients with electrocardiographic abnormalities (52.6%) also had serum electrolyte alterations. More than 50% of the patients with acute pancreatitis had electrocardiographic abnormalities and electrolyte alterations were also present in about one-half of these.