32例噬血细胞综合征实验室检查及临床分析

目的分析继发性噬血细胞综合征(HPS)患者的实验室指标及临床特点,以提高对该病的认识。方法对2005年6月~2015年6月在广西肿瘤防治研究所治疗的32例继发性HPS患者的病因、临床表现、实验室特征、治疗及预后等进行分析。结果 32例患者中,血液系统肿瘤17例,单纯感染13例,自身免疫性疾病2例。临床上以发热(100%)、肝脾(87.5%)及淋巴结(43.8%)肿大为主要表现。实验室检查以血细胞减少(100%)、铁蛋白升高(96.9%)、自然杀伤细胞活性降低或缺失(96.9%)为主,纤维蛋白原降低(68.8%),甘油三酯升高(56.3%),81.3%的患者骨髓涂片检查可见噬血细胞现象。结论继发性HPS可由多种病因引起,临床表现多样,对可疑患者及时进行相关实验室检查有助于早期明确诊断;合并多脏器衰竭、DIC的患者预后不良。     

Epstein-Barr病毒相关噬血细胞综合征的研究进展

Epstein-Barr病毒相关噬血细胞综合征(EBV-HLH)的病情进展快,病死率高。高细胞因子血症是其发病的重要机制,临床表现多样。诊断需符合HLH诊断标准并存在EBV感染的证据。治疗首选依托泊苷、糖皮质激素、环孢素A联合应用。如化疗效果不佳,可行造血干细胞移植。现就该病的最新研究进展进行综述。     

噬血细胞综合征临床特征与影响预后因素分析

目的:探讨噬血细胞综合症的临床特征及影响预后因素,为临床治疗提供依据。方法:选择2006年1月~2011年12月我院收治的噬血细胞综合症患儿26例,回顾分析所有患儿的临床表现、实验室检查、病因及转归等资料。结果:死亡10例,好转或痊愈16例。原发与继发性HPS转归情况比较,X2=7.825,P<0.05,差异有统计学意义。两组年龄(t=2.807)、肝肿大(t=2.817)、脾肿大(t=2.651)、白细胞计数(t=2.580)、血小板(t=2.814)、纤维蛋白原(t=2.984)、乳酸脱氢酶(LDH)(t=2.667)和丙氨酸转氨酶(ALT)(t=2.514)比较,差异有统计学意义,P<0.05。结论:儿童噬血细胞综合征病因复杂,临床特征多样,应结合患儿个体特征进行临床治疗。     

37例儿童噬血细胞综合征的临床分析

目的:分析儿童噬血细胞综合征(hemophagocytic syndrome,HPS)的病因、临床表现、实验室检查结果、治疗和预后特点。方法:回顾性分析我院收治的37例HPS患儿的临床资料。结果:37例HPS患儿(男24例、女13例),年龄2月~9岁,5例(13.5%)有明显家族史,获得性HPS32例(86.5%),包括EB病毒感染16例、巨细胞病毒感染7例,其他原因9例;所有患儿均表现为发热,肝脾肿大,外周血白细胞、血红蛋白、血小板、白蛋白、纤维蛋白原减低,TG、ALT、AST、LDH、铁蛋白升高;5例遗传性HPS患儿死亡4例,放弃治疗1例,剩余32例患儿中好转20例(62.5%),包括痊愈17例,完全缓解后继续治疗中3例,未好转12例(37.5%),其中死亡7例,病情危重放弃治疗3例,复发2例。12例未好转病例中,9例为EBV感染,1例为肾母细胞瘤,1例为幼年类风湿性关节炎合并CMV感染,1例原因不明。遗传性HPS的好转率较继发性HPS明显降低,差异有统计学意义(X2=5.30,P0.05),继发性HPS中EBV感染者的好转率较非EBV感染者低,差异有统计学意义(X2=4.80,P0.05)。结论:及时诊断儿童HPS并明确其病因,对该病的治疗及预后具有重要意义。     

儿童噬血细胞综合征38例临床分析

摘要目的：探讨噬血细胞综合征患儿的临床特征、治疗及预后。方法：对38例噬血细胞综合征患儿的Il盏床症状、体征、试验检查结果、治疗及预后,进行回顾性总结与分析。结果：38例患儿主要表现为发热、肝脾淋巴结肿大、外周血细胞减少、铁蛋白升高、凝血功能异常;经针对性治疗后,治愈3例,好转20例,疗效不佳自动出院9例,死亡6例。结论：噬血细胞综合征病因复杂,临床表现多种多样,病情凶险,预后较差,旱期诊断及治疗对预后十分关键。     

噬血细胞综合征1例报告

噬血细胞综合征（hemophagocytic syndrome,HPS）是一种由于某些感染性疾病如伤寒、布氏杆菌病、感染性心内膜炎、病毒性肝炎、败血症、结核病等引起的反应性组织细胞增生症,不成熟的组织细胞吞噬血液细胞,因此得名。该病于1979年由Risdall等首先报道。近年来有关报道逐渐增多,现将我院收治的1例报告如下。     

烙铁头蛇毒纤维蛋白原溶酶研究——Ⅱ.对纤维蛋白原及凝血酶的作用

烙铁头(T.mucrosquamatus)蛇毒纤维蛋白原溶酶TMVFg能水解三肽底物Bz-Phe-Val-Arg-PNA,但对凝血酶的良好底物Cbz-Gly-Pro-Arg-PNA却活性甚低。TMVFS显著延长血浆凝血酶时间。血浆复钙时间及纤维蛋白原溶液凝血酶时间。同时,TMVFg体外也能延长全血凝固时间,表明具有抗凝作用。纤维蛋白原-纤维蛋白转换实验表明:TMVFg水解纤维蛋白原产生的纤维蛋白原断片(FDP)除具有抗凝血酶,抑制纤维蛋白聚合活性外,还能促进纤维蛋白的聚合。 进一步用FPLC分离TMVFg水解人纤维蛋白原混合液,得两个FDP断片功能峰,FDP组分Ⅰ和FDP组分Ⅱ。其中FDP组分Ⅰ能抑制纤维蛋白凝块形成;FDP组分Ⅱ能促进纤维蛋白凝块形成,抑制TMVA(烙铁头蛇毒血小板活化素,它可不通过ADP、花生四烯酸途径而诱导血小板聚集),但对ADP诱导的家兔血小板聚集无影响。TMVFg对凝血酶水解三肽底物Cbz-Gly-Pro-Arg-PNA及凝固纤维蛋白原的活性也有一定抑制作用。 实验证明,TMVFg抗凝的主要作用机理是其水解纤维蛋白原产生的断片对纤维蛋白原凝固的抑制作用、FDP断片抗凝血酶作用及TMVFg本身对凝血酶活性的抑制所引起的,但在二者之间,前者是主要的。 从研究结果发现:TMVFg水解纤维蛋白原所产生的断片有一类能加速凝血酶凝固纤维蛋白原的过程,这就发现了FDP断片的     

纤维蛋白（原）在动脉粥样硬化发病中的作用及机制

在高胆固醇饮食诱发的家兔动脉粥样硬化模型上,发现ＡＳ早期即出现高纤维蛋白原血症,血浆Ｆｇ和胆固醇水平呈正相关。组织免疫化学检查发现,Ｆｇ密布于血管内皮下和平滑肌细胞间质。     

水貂血浆纤维蛋白原的测定

水貂(Mustela vison)是一种珍贵的毛皮动物。到目前为止,对于水貂的研究大多集中在生殖和生活习性方面,而对水貂血液的研究却很少。国外有人研究水貂的血红蛋白组成(Labbe等,1979)、水貂的血浆电泳(Tandon,1980)和水貂血浆中脂蛋白和胆固醇的含量(Nikjtin,1983)等。但是,对于水貂血浆纤维蛋白原的研究至今未有报道。 本项研究采用了饱和氯化钠溶液法,通过分光光度计,测定了哈尔滨市城高子良种     

梅花鹿和马鹿血浆纤维蛋白原的测定

梅花鹿(Cervus nippon)和马鹿(Cervus elaphus)为我国主要产茸鹿种。锯茸时出血很多,但很快止血并形成血痂,不发生因失血致死现象。产生这种现象的机制,除血液中其它凝血因子外,与鹿血浆纤维蛋白原的含量有关。目前尚未见到国内外关于梅花鹿和马鹿血浆纤维蛋白原含量的报道。 本研究用盐析和分光光度法,根据Lambert-Beer定律     

Viral load in Epstein-Barr virus-associated hemophagocytic syndrome

The viral load in peripheral blood from patients with Epstein-Barr virus (EBV)-associated hemophagocytic syndrome was measured by real-time quantitative PCR and compared with that in infectious mononucleosis. Patients with EBV-associated hemophagocytic syndrome generally had larger viral burdens, although it was difficult to differentiate EBV-associated hemophagocytic syndrome from infectious mononucleosis simply by viral load. The difference in viral load seemed to be clearer in peripheral blood mononuclear cells than in plasma.     

Expression of the fibrinogen genes in rat megakaryocytes

A variety of evidence suggests that megakaryocytes synthesize fibrinogen and comparative immunochemical and structural studies indicate that fibrinogen produced in or associated with megakaryocytes may be different than fibrinogen produced in the liver. Two studies have reported that the gamma' chain, which is produced from the gamma chain gene by alternative splicing, is absent from fibrinogen produced in the megakaryocyte. Since there is only a single gene for each of the three fibrinogen chains the reported structural differences suggest different mechanisms for production of hepatic and megakaryocytic fibrinogen. We have begun an investigation of the varying mechanisms for expression of the fibrinogen genes by examining the structure of fibrinogen mRNA's in the two tissues. Fibrinogen mRNA's of identical length are found in both liver and megakaryocytes. Furthermore, despite the reported absence of the gamma' chain in platelet-associated fibrinogen, we have used a probe specific for the alternative spliced region of the gamma' mRNA to clearly demonstrate this chain in megakaryocyte mRNA. These studies indicate that the gamma' mRNA is either not translated in platelets or that the gamma' chain is unable to associated with the alpha and beta chains to form a mature molecule.     

The significance of ferritin in cancer: Anti-oxidation,inflammation and tumorigenesis

The iron storage protein ferritin has been continuously studied for over 70 years and its function as the primary iron storage protein in cells is well established. Although the intracellular functions of ferritin are for the most part well-characterized, the significance of serum (extracellular) ferritin in human biology is poorly understood. Recently, several lines of evidence have demonstrated that ferritin is a multi-functional protein with possible roles in proliferation, angiogenesis, immunosuppression, and iron delivery. In the context of cancer, ferritin is detected at higher levels in the sera of many cancer patients, and the higher levels correlate with aggressive disease and poor clinical outcome. Furthermore, ferritin is highly expressed in tumor-associated macrophages which have been recently recognized as having critical roles in tumor progression and therapy resistance. These characteristics suggest ferritin could be an attractive target for cancer therapy because its down-regulation could disrupt the supportive tumor microenvironment, kill cancer cells, and increase sensitivity to chemotherapy. In this review, we provide an overview of the current knowledge on the function and regulation of ferritin. Moreover, we examine the literature on ferritin's contributions to tumor progression and therapy resistance, in addition to its therapeutic potential.     

The presence of heat-labile factors interfering with binding analysis of fibrinogen with ferritin in horse plasma

Background

Horse fibrinogen has been identified as a plasma specific ferritin-binding protein. There are two ways in the binding of ferritin-binding protein with ferritin: one is direct binding and the other is indirect binding which is heme-mediated. The aim of this study was to analyze the binding between horse fibrinogen and ferritin.

Findings

Although fibrinogen in horse plasma did not show the binding to ferritin coated on the plate wells, after following heat-treatment (60°C, 30 min) of horse plasma, plasma fibrinogen as well as purified horse fibrinogen bound to plates coated with horse spleen ferritin, but not with its apoferritin which lost heme as well as iron after the treatment of reducing reagent. Binding of purified or plasma fibrinogen to ferritin was inhibited by hemin and Sn-protoporphyrin IX (Sn-PPIX), but not by PPIX or Zn-PPIX.

Conclusions

Heat-treatment of horse plasma enabled plasma fibrinogen to bind to plate well coated with holo-ferritin. From the binding analysis of fibrinogen and ferritin, it is suggested that horse fibrinogen recognized iron or tin in complexed with the heme- or the hemin-ring, and also suggest that some fibrinogens circulate in the form of a complex with ferritin and/or heat-labile factors which inhibit the binding of fibrinogen with ferritin.

     

Expression of heteropolymeric ferritin improves iron storage in Saccharomyces cerevisiae

Saccharomyces cerevisiae was engineered to express different amount of heavy (H)- and light (L)-chain subunits of human ferritin by using a low-copy integrative vector (YIp) and a high-copy episomal vector (YEp). In addition to pep4::HIS3 allele, the expression host strain was bred to have the selection markers leu2(-) and ura3(-) for YIplac128 and YEp352, respectively. The heterologous expression of phytase was used to determine the expression capability of the host strain. Expression in the new host strain (2805-a7) was as high as that in the parental strain (2805), which expresses high levels of several foreign genes. Following transformation, Northern and Western blot analyses demonstrated the expression of H- and L-chain genes. The recombinant yeast was more iron tolerant, in that transformed cells formed colonies on plates containing more than 25 mM ferric citrate, whereas none of the recipient strain cells did. Prussian blue staining indicated that the expressed isoferritins were assembled in vivo into a complex that bound iron. The expressed subunits showed a clear preference for the formation of heteropolymers over homopolymers. The molar ratio of H to L chains was estimated to be 1:6.8. The gel-purified heteropolymer took up iron faster than the L homopolymer, and it took up more iron than the H homopolymer did. The iron concentrations in transformants expressing the heteropolymer, L homopolymer, and H homopolymer were 1,004, 760, and 500 micro g per g (dry weight) of recombinant yeast cells, respectively. The results indicate that heterologously expressed H and L subunits coassemble into a heteropolymer in vivo and that the iron-carrying capacity of yeast is further enhanced by the expression of heteropolymeric isoferritin.