广东省人群中人嗜T细胞病毒Ⅰ型感染的血清流行病学调查及其与人类疾病的关系

收集广东省各类人群血清标本２２２４份，用间接免疫荧光法检测人类嗜Ｔ细胞病毒Ⅰ型（ＨＴＬＶ－１）抗体，结果：ＨＴＬＶ－１抗体阳性３５份，总阳性率为１．５７％；其中健康人群血清１８１０份，抗体阳性２３份，阳性率为１２７％；献血员血清２４８份，抗体阳性１份，阳性率０４０％；白血病患者血清１０９份，阳性８份，阳性率７３４％，与健康人群比较，有非常显著性差异（Ｐ＜０００５）；神经系统疾病患者血清５７份，阳性３份，阳性率５２６％。     

Functional analysis of PTPN11/SHP-2 mutants identified in Noonan syndrome and childhood leukemia

Noonan syndrome (NS) is characterized by short stature, characteristic facial features, and heart defects. Recently, missense mutations of PTPN11, the gene encoding protein tyrosine phosphatase (PTP) SHP-2, were identified in patients with NS. Further, somatic mutations in PTPN11 were detected in childhood leukemia. Recent studies showed that the phosphatase activities of five mutations identified in NS and juvenile myelomonocytic leukemia (JMML) were increased. However, the functional properties of the other mutations remain unidentified. In this study, in order to clarify the differences between the mutations identified in NS and leukemia, we examined the phosphatase activity of 14 mutants of SHP-2. We identified nine mutations, including a novel F71I mutation, in 16 of 41 NS patients and two mutations, including a novel G503V mutation, in three of 29 patients with leukemia. Immune complex phosphatase assays of individual mutants transfected in COS7 cells showed that ten mutants identified in NS and four mutants in leukemia showed 1.4-fold to 12.7-fold increased activation compared with wild-type SHP-2. These results suggest that the pathogenesis of NS and leukemia is associated with enhanced phosphatase activity of mutant SHP-2. A comparison of the phosphatase activity in each mutant and a review of previously reported cases showed that high phosphatase activity observed in mutations at codons 61, 71, 72, and 76 was significantly associated with leukemogenesis.     

Deficient IFNα production in hairy cell leukemia

Summary Since the application of low doses of IFN-alpha is necessary to maintain remissions in Hairy Cell Leukemia (HCL) it is of interest whether peripheral blood mononuclear cells (MNC) of HCL patients can be induced in vitro to produce IFN-alpha. 9 patients suffering from advanced HCL were included in the study. The diagnoses were confirmed by characteristic findings in peripheral blood and bone marrow biopsies. For IFN treatment we initially used natural IFN-alpha (Bioferon) and switched later to recombinant IFN-alpha₂ (Boehringer). MNC of 5 patients before IFN therapy and of 6 patients during IFN therapy (2–47 weeks) were induced by phythemagglutinin (PHA), Corynebacterium parvum (C.p.), and sendai virus (SV). PHA is known to induce IFN-gamma. Both, C.p. and SV induced IFN-alpha but no IFN-gamma in MNC of healthy controls and of IFN treated breast cancer patients. In HCL patients normal antiviral activities could be induced by PHA. Zero or only low antiviral activities could be induced in MNC from 9 patients tested on 22 occasions. It is concluded that MNC from patients with advanced HCL can be induced to produce IFN-gamma but no IFN-alpha. Since IFN-alpha but not IFN-gamma is produced by monocytes it is likely that reduced numbers of monocytes which were found in our HCL patients before and during IFN treatment account for the described deficiency of IFN-alpha production.     

测定白血病脑脊液中SIL—2R，IL—6表达的临床意义

为了探讨急性淋巴细胞性白血病 (ALL)患者脑脊液中可溶性白介素 - 2受体 (SIL - 2R)、白介素 6(IL - 6)的表达及意义 ,采用双抗夹心ELISA法测定 3 0例ALL患者脑脊液 (CSF)中SIL - 2R ,IL - 6水平 ,并与 10名正常者进行对照 .结果 :ALL合并中枢神经系统白血病 (CNS -L)组较CNS -L已缓解及未合并CNS -L组二者水平显著升高 (P <0 0 1) ;CNS -L已缓解组二者水平接近对照组 (P >0 0 5 ) ;骨髓缓解及好转组二者水平低于治疗无效组 .结果表明 :监测二者水平变化有助于CNS -L早期诊断、估计预后、判断疗效 .     

湖南省江华瑶族自治县14年白血病流行病学调查

目的：了解白血病在瑶族人群中的发病情况及危险因素。方法：调查1984－1997年全县白血病新发病例,计算其发病率并进行比较分析。结果：本县白血病年发病率为1．61／10万,明显低于全国白血病年均发病率,瑶族人群低于汉族人群。白血病的发病有时一空集积现象。结论：瑶族人群白血病率不高。     

白细胞清除术联合化疗治疗高白细胞急性白血病疗效观察

目的：比较白细胞清除术联合化疗治疗高白细胞急性白血病与单纯化疗治疗高白细胞急性白血病的疗效。方法：采用前瞻性同期对照研究方法。结果：11例高白细胞急性白血病经白细胞清除术后化疗,CR率达54．5％,早期死亡率9％。单用化疗例,仅1例完全缓解,早期死亡3例（42．8％）。结论：白细胞清除术联合化疗治疗高白细胞急性白血病是目前较先进的一种方法。     

应用基因表达谱芯片研究硫化砷作用后K562细胞基因表达的变化

目的：利用基因表达谱芯片检测K562细胞在硫化砷作用前后基因表达的差异性进行比较研究。方法：研究对象为人慢性粒细胞白血病细胞株K562,用cy3和cy5两种不同的荧光染料通过逆转录反应将K562经硫化砷作用前后的mRNA分别标记成两种探针,并与载有一组靶的基因表达谱芯片进行杂交,通过计算机扫描分析得到这些基因在经硫化处理前后的表达差异,结果：筛选出包括与DNA结合,转录和转录因子,蛋白翻译,细胞周期等相关表达差异的基因共11条,其中7条表达上调,4条表达下调,结论：周期素E2,周期素G2可能参与硫化砷诱导K562细胞凋亡发生机制。     

111例急性T淋巴细胞白血病免疫表型分析

选用一组抗人白细胞表面分化抗原单克隆抗体和基因探针,依靠间接免疫荧光技术、Ap-AAP桥联酶标免疫组化染色和分子杂交手段.对111例急性T淋巴细胞白血病(T-ALL)进行了免疫分型研究。本研究所观察到的T-ALL细胞表型绝大多数反映着正常胸腺细胞分化发育阶段或某些少见类型细胞的表型特征,亦有少数病例则无正常淋巴细胞相对应的表型,表明T-ALL细胞的高度异质性。对T-ALL表型的研究为今后深入探讨胸腺内淋巴细胞的分化成熟以及T-ALL发生学提供了良好的研究模式。     

小儿急性白血病骨胳X线表现(附10例分析)

结合10例临床资料分析,认为小儿急性白血病浸润骨骼的主要X线表现为骨质稀疏、干骺端横行透明线,溶骨性破坏,骨膜反应及病理骨折等。上述表现可出现于临床症状之前,对小儿急性白血病的诊断起着重要作用,特别是2岁以上患儿长骨干骺端白血病线是本病诊断的重要依据。     

非亲缘异基因骨髓移植治疗急性和慢性白血病

目的评价非亲缘异基因骨髓移植（URD-BMT）治疗急性和慢性白血病的临床疗效以及并发症的防治方法。方法由台湾慈济骨髓捐赠中心提供HLA相合非亲缘供髓,对11例急性白血病和慢性白血病患者进行骨髓移植术,其中急性淋巴细胞性白血病2例（CR1）、急性髓细胞性白血病2例（ANLL-CR1）、慢性髓细胞性白血病7例。10例患者预处理方案为移植前7至4d马利兰(BU)4mg·kg-1·d-1,移植前3至2d环磷酰胺（CTX）60mg·kg-1·d-1。1例慢粒急淋变患者预处理方案全身放疗(TBI)7.5GY,移植前4至3d环磷酰胺60mg·kg-1·d-1。供髓输注有核细胞（2.6～4.8）×108/kg,CD34+细胞（3.82～12.8）×106/kg,粒单-集落形成单位(CFU-GM)（1.71～3.68）×105/kg。预防急性移植物抗宿主病（aGVHD）方案霉酚酸酯（MMF）+环孢菌素A（CsA）+短程氨甲喋呤（MTX）。预防肝静脉阻塞病（VOD）方案低剂量肝素持续静滴加前列腺素E1脂质微球（Lipo-PGE1）。以更昔洛韦预防巨细胞病毒感染（CMV）方案。非亲缘供髓与受者间HLA-A、B、DR基因型完全相合8例,1个亚型不相合2例,2个亚型不相合1例。结果全部患者移植后均获得造血重建,中性粒细胞>0.5×109/kg中位时间18d,血小板>20×109/L中位时间27d。10例患者经DNA短串联重复序列多态性分析证明为供者骨髓植活。发生急性GVHDⅣ度1例,aGVHDⅡ度2例,重度VOD和Ⅲ度房室传导阻滞1例,出血性膀胱炎3例,死于间质性肺炎1例,1例患者于移植后7个月复发,予以供者淋巴细胞输注,目前尚处于治疗中。无病生存10例。结论非亲缘异基因骨髓移植术是急性和慢性白血病有效治疗方法。本组患者对aGVHD、VOD和CMV采用的预防方案是有效和安全的。