γ-干扰素和白细胞介素-4在特发性血小板减少性紫癜发病机制中的作用

目的　探讨γ 干扰素 (IFN γ)和白细胞介素 4(IL 4)在特发性血小板减少性紫癜发病中作用。方法　用ELISA方法 ,检测IL 4,IFN γ ,PAIgG在特发性血小板减少性紫癜 (ITP)治疗前后的变化情况。结果　ITP患者治疗前IL 4明显高于对照组 ,治疗达到缓解后 ,IL 4明显下降 ,P <0 .0 5 ,而未缓解组 ,IL 4未见明显变化。治疗前的IFN γ与对照组相比。虽有增加 ,但差异无显著性 (P >0 .0 5 )。结论　IL 4,IFN γ对ITP患者Th1/Th2应答及临床相关性起着一个重要调节作用。     

特发性血小板减少性紫癜输入血小板制剂的意义

特发性血小板减少性紫癜(idiopathic thromobocytopenicpurpura,ITP)是临床上常见的出血性疾病,常需要输注血小板制剂。临床资料一般资料选择2003年4月~2005年8月我院血液科住院的71例(男33,女48)ITP患者,年龄11~77岁,中位年龄31.3岁,分为对照组和血小板输注治疗组。对照组36例     

血美安对特发性血小板减少性紫癜小鼠免疫细胞因子的调节作用

目的通过建立特发性血小板减少性紫癜(ITP)小鼠动物模型,观察血美安对ITP小鼠外周血细胞因子的影响,探讨血美安的免疫调节机制。方法 40只Balb/c小鼠随机分为阴性对照组(A组)、模型组(B组)、阳性对照组(C组)、血美安(D组)组。除A组外,其余各组隔日注射豚鼠抗小鼠血小板血清(GP-APS)建立ITP小鼠模型。注射GP-APS 6 d后,A组和B组灌服0.9%氯化钠溶液,C组醋酸泼尼松按5 mg/kg灌胃,血美安胶囊按含药量为49.1 g/L灌胃。观察小鼠一般情况,连续给药14 d后处死小鼠;分离血清检测血小板、干扰素-γ(IFN-γ)和白细胞介素(IL)-4水平。结果与A组比较,IFN-γ水平显著升高(P0.05);血小板和IL-4水平显著下降(P0.05),IFN-γ/IL-4比值升高。与B组比较,D组IFN-γ水显著下降(P0.05);血小板和IL-4水平显著升高(P0.05),IFN-γ/IL-4比值降低具有显著差异(P0.05)。结论血美安可通过影响Th1/Th2细胞因子水平而对ITP免疫功能起到调节作用。     

特发性血小板减少性紫癜患者治疗前后Th1／Th2水平变化及其临床意义

目的：研究特发性血小板减少性紫癜（ITP）患者药物治疗前后辅助性T淋巴细胞（Th）1/Th2细胞因子水平的变化情况及其临床意义。方法：应用流式细胞仪检测60例ITP患者药物治疗前后干扰素γ（IFN-y）、白细胞介素（IL）-2、IL-4、IL-10水平,分析Th1/Th2细胞因子水平的变化情况,并结合疗效作相关性分析。以60名健康体检者为对照。结果：药物治疗有效的ITP患者治疗后Th1细胞因子IL-2、IFN-1水平较治疗前及对照组均显著升高（P〈0．01）。而Th2细胞因子IL-4及IL-10水平在3组间均无显著性差异（P＞0．051．Th1/Th2比例升高。ITP患者Th1细胞因子IL-2、IFN．叮水平在治疗显效组表达均明显高于良效及进步组和无效组（P〈0．05）,良效及进步组与治疗无效组比较亦有显著性差异（P〈0．05）;经相关性分析,患者的疗效与IFN-γ、IL-2均呈正相关。结论：ITP患者药物治疗后Th1细胞因子IL-2、IFN-γ表达升高,且Th1与ITP患者疗效密切相关。     

特发性血小板减少性紫癜患者外周血中树突状细胞共刺激分子表达研究

特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病,其特征是未成熟的血小板在单核巨噬系统被吞噬细胞破坏引起血小板减少.本文旨在讨论树突状细胞(DC)共刺激分子CD80、CD86在ITP发病机制中的作用.     

妊娠合并特发性血小板减少性紫癜

特发性血小板减少性紫癜（ITP）是一种以外周血小板数目持续减少为特征的自身免疫性疾病。在成人中好发于40岁以下育龄女性,且不影响生育功能,所以妊娠合并ITP是临床医生经常遇到的问题,其机率报道不一,约为1～2／1000名妊娠妇女,     

慢性特发性血小板减少性紫癜患者PAIgG、ACAIgG的测定及意义

应用ELISA法对 41例慢性特发性血小板减少性紫癜 (ITP)患者和 2 5例健康对照者进行血浆和 (或 )血清血小板相关抗体 (PAIgG)、抗心磷脂抗体 (ACAIgG)测定 ,并常规血小板计数。发现ITP患者治疗有效组治疗后血小板计数明显升高、PAIgG含量显著降低 ,治疗前较治疗后及对照组均有显著性差异 (P <0 .0 1 ) ,且血小板计数与PAIgG之间呈显著性负相关 (r =- 0 .738,P <0 .0 0 1 )。而治疗无效组血小板计数、PAIgG均无明显变化。ACAIgG治疗前后无明显变化 ,且未发现与血小板及PAIgG相关。PAIgG、ACAIgG含量增高提示ITP患者针对血小板磷脂及其他糖类蛋白等不同抗原产生了自身免疫反应 ,为免疫抑制剂的应用提供了理论依据     

干扰素治疗老年特发性血小板减少性紫癜9例报告

特发性血小板减少性紫癜 (ｉｄｉｏｐａｔｈｉｃｔｈｒｏｍｂｏｃｙｔｏｐｅｎｉｃｐｕｒｐｕｒａ ,ＩＴＰ)是临床常见的一种出血性疾病。老年人ＩＴＰ常反复发作 ,泼尼松治疗的完全缓解率仅 10 %～ 15 %[1] ,其他替代疗法如长春新碱、硫唑嘌呤、环磷酰胺、达那唑等副作用大 ,应用于老年患者病死率高[2 ] 。 1996年 2月至 2 0 0 0年 10月 ,我院用α 干扰素治疗老年ＩＴＰ患者 9例 ,取得一定疗效 ,现报道如下。1　 对象和方法1 1　病例　全部病例均为住院患者 ,符合 1986年 12月首届中华血液学学会全国血栓与止血会议拟定的ＩＴＰ诊断标准[3 …     

特发性血小板减少性紫癜的治疗

特发性血小板减少性紫癜(idiopathic thrombo—cytopenic purpura,ITP)是一种由自体免疫介导的血小板破坏,导致血小板数量减少,该病是临床上最常见的一种血小板减少性疾病。成人ITP多见于18～40岁的女性患者,国外资料显示,男：女比例为1．7：1～1．2：1,诊断时的平均年龄为56～60岁。     

网织血小板检测在特发性血小板减少性紫癜中的临床价值

特发性血小板减少性紫癜(ITP)是一种因为产生抗自身血小板抗体促使血小板减少而使机体器官受损的特异性自身免疫性疾病。血小板减少引起的出血是ITP较常见的表现之一。血小板减少的主要原因大致为两类：血小板生成减少和血小板破坏加速。网织血小板(RP)是新释放入血液循环的血小板，是富含RNA类似于网织红细胞(Ret)的最年轻的血小板。RP的数目反映了人体血小板的更新速度、血小板增生情况。2002年11月～2003年4月我们检测了50例健康人、48例ITP患者、35例急性白血病、25例再生障碍性贫血外周血的RP，旨在探讨RP在特发性血小板减少性紫癜诊断和治疗中的临床价值，以便更好为临床上诊治ITP提供一种新的手段。     

Pulsed intravenous high-dose dexamethasone in adults with chronic idiopathic thrombocytopenic purpura

The role of pulsed high-dose dexamethasone (DXM) in the treatment of patients with chronic idiopathic thrombocytopenic purpura (ITP) is still uncertain. Following an early report in which it was described as an effective and well-tolerated treatment with a sustained platelet response in 100% of cases, a number of subsequent studies have failed to confirm such favorable results. As all these studies were conducted on small numbers of patients, we investigated further the effectiveness and side effects of this therapeutic modality in a larger cohort. Thirty-two patients with chronic ITP were scheduled to receive six monthly courses of intravenous DXM at the dose of 40 mg/day for 4 consecutive days. All patients had ITP that had been resistant to between two and five different therapeutic regimens, including 9 patients who had already failed splenectomy. All patients had to be seen 2 weeks after each cycle to asses their response as well as secondary effects. Three patients failed to respond and clinically required other therapy. Thirteen patients (41%) had a partial (platelet count between 50 and 100 x 10(9)/liter) or complete (platelet count >100 x 10(9)/liter) response to treatment, responses being mostly transient. Responses were observed early during the course of treatment, usually right after the first cycle of DXM. There were no late responses. Side effects were mild and did not require discontinuation of treatment. No clinical or laboratory parameter was found to predict treatment outcome. We conclude that high-dose DXM has a limited effect in patients with chronic ITP. Novel approaches and controlled multicenter trials may help identify new therapeutic strategies for this disease.     

Dapsone for chronic idiopathic thrombocytopenic purpura in children and adults--a report on 90 patients

Data on 90 patients (55 adults and 35 children) with chronic idiopathic thrombocytopenic purpura (ITP) and a platelet count of <50 x 10(9)/L treated with dapsone at a dose of 1-2 mg/kg/d are presented. A response was observed in 57 (63.3%) patients. The average time for response was 3.5 months (range 1-9) and the average duration of treatment with dapsone was 10.4 months (range 4-14). Overall response rates of 65.7% and 61.8% were observed in children and adults respectively. Side effects requiring discontinuation of therapy were observed in three (2%) patients. These results demonstrate that dapsone is an effective, inexpensive and well-tolerated treatment for chronic ITP, in both children and adults and could be considered for patients who fail steroid therapy.     

Effect of Helicobacter pylori eradication on platelet recovery in Japanese patients with chronic idiopathic thrombocytopenic purpura and secondary autoimmune thrombocytopenic purpura

The prevalence of Helicobacter pylori infection and the effect of its eradication on platelet count in 48 Japanese patients with autoimmune thrombocytopenic purpura (AITP), including 40 chronic idiopathic thrombocytopenic purpura (ITP) and eight secondary AITP, were investigated. H. pylori infection was found in 25 ITP patients (62.5%) and in two secondary AITP (25%). H.pylori eradication was obtained in 19 of 19 infected ITP patients (100%), who were not in remission (platelets < 100 x 109/l) at the time of infection assessment. During follow-up (median 14.8 months), 12 of 19 H. pylori-eradicated patients (63.2%) showed a significant increase in platelet count accompanied by a significant decrease of platelet-associated immunoglobulin G (IgG). This response was maintained in all responding patients throughout the follow-up period. However, two infected patients with secondary AITP did not show platelet increase after eradication. The assessment of H. pylori infection and its eradication should be attempted in ITP as this approach could be an effective strategy, at least for some of these patients.     

Dapsone for refractory chronic idiopathic thrombocytopenic purpura

Summary. Fifteen patients with refractory chronic idiopathic thrombocytopenic purpura (ITP) were treated with dapsone (lOOmg/d) for 1-31 months. The overall response rate to dapsone was 40%. Five patients responded in 1 month and one patient in 2 months. No pretreatment characteristics -sex, age, platelet count or duration of ITP - were correlated with response to dapsone. Treatment was well tolerated. The most frequent adverse effect was dose-related haemolytic anaemia. In our experience, dapsone provides an inexpensive and well-tolerated alternative for patients with ITP who had inadequate reponses to conventional therapy.     

Serum leptin levels in patients with idiopathic thrombocytopenic purpura

OBJECTIVES: The purpose of this study was to evaluate serum leptin levels in idiopathic thrombocytopenic purpura (ITP), in order to determine the influence of leptin on the pathogenesis of ITP. SUBJECTS AND METHODS: Forty-six untreated patients with chronic ITP were compared with 40 healthy people of similar age, sex and body mass index (BMI). Serum leptin levels (ng/mL) were measured by enzyme-linked immunosorbent assay (ELISA). RESULTS: We found that the mean serum leptin levels in patients with ITP (22.11 +/- 15.84 ng/mL) were significantly (P < 0.001) higher than that in healthy control volunteers (5.44 +/- 4.84 ng/mL). Furthermore, serum leptin levels in patients with ITP were inversely related (r = -0.86, P < 0.001) to the platelet counts and positively related to the platelet-associated IgG (PAIgG) levels (r = 0.7, P < 0.001). The levels of PAIgG and platelet counts were significantly different between leptin-positive (level greater than mean +/- 2 SD control value) and leptin-negative patients. CONCLUSION: These findings suggest that leptin might play an important role in the pathogenesis of ITP.     

Acute idiopathic thrombocytopenic purpura in children

Acute idiopathic thrombocytopenic purpura (ITP) characteristically follows a viral illness in preschool children. The exact role of viruses in the pathogenesis of this disorder remains uncertain, but the finding of markedly elevated levels of platelet-associated IgG serves to distinguish it from the chronic form of the disease and permits speculation on the mechanisms of platelet destruction. Although the spleen is important in both antibody production and platelet destruction, bone marrow synthesis of IgG has also been shown to be increased. The clinical course may be alarming, but mortality is low and prognosis excellent. Controversy has surrounded the role of steroids in the management of acute childhood ITP in retrospective studies. Controlled studies, however, indicate that thrombocytopenia is reversed sooner in treated patients. New assays for platelet-associated IgG offer new insights into this disorder and will allow delineation of acute and chronic disease at the time of diagnosis.     

High-dose intravenous IgG for chronic idiopathic thrombocytopenic purpura in adults

Summary Sixteen adult patients of mean age 48 years with chronic ITP were studied for platelet response to high-dose (0.4 g/kg body weight per day for five consecutive days) intravenous polyvalent intact IgG in the absence of any concurrent treatment. The platelet count returned to normal values in nine patients, a partial response (rise in the platelet count between 50 and 150 ×10⁹/l) was observed in three cases. One patient refractory to any other treatment went into a sustained remission. In the other responsive patients the response was only transient. Among seven splenectomised patients only three responded to IgG infusions versus nine in the non-splenectomised group. The length of ITP history appeared as a more critical factor for the response to IgG than previous splenectomy.     

抗CD20抗体治疗慢性特发性血小板减少性紫癜机制的研究

目的:通过抗CD20抗体(Rituximab,商品名:美罗华)与慢性特发性血小板减少性紫癜(cITP)骨髓体外培养,了解cITP患者B细胞的活化与凋亡的状况。方法:选择cITP患者30 例,对照组缺铁性贫血患者10例,进行骨髓体外培养,于培养前、培养3 d、培养6 d、培养9 d,检测B细胞相关分子(CD19、CD20、CD23)和透射电镜观测淋巴细胞凋亡状态。结果:cITP患者骨髓CD20、CD23分子表达显著高于对照组CD20、CD23分子表达(P<0.01)。加抗CD20抗体和加半量抗CD20抗体培养前后B细胞相关分子(CD19、CD20、CD23)检测结果差异有统计学意义(P<0.01)。加抗CD20抗体组透射电镜观测有淋巴细胞凋亡。结论:抗CD20 抗体能靶向性地与表达CD20抗原的B细胞结合,通过抗体抗原反应,诱导B细胞加速凋亡。     

CD28/CTLA-4:B7在特发性血小板减少性紫癜患者外周血淋巴细胞中的表达及意义

目的:探讨特发性血小板减少性紫癜(ITP)患者外周血淋巴细胞CD28、CTLA-4(CD152)、B7-1(CD80)及B7-2(CD86)的表达及意义。方法:采用免疫荧光标记和流式细胞术检测41例ITP患者和40例健康对照者外周血CD3+CD28+细胞、CD3+CD152+细胞、CD80+CD19+细胞和CD86+CD19+细胞分别占淋巴细胞的比例及血小板表面相关抗体水平,进行2组对比、分析。结果:与正常对照组相比,急性ITP患者外周血CD3+CD28+细胞和CD3+CD152+细胞差异无统计学意义(P0.05),CD80+CD19+细胞增多(P0.05),CD86+CD19+细胞显著增多(P0.01),慢性ITP患者CD86+CD19+细胞增多(P0.05);急性ITP患者外周血CD86+CD19+细胞较慢性ITP患者增多(P0.05);与正常对照组相比,急性ITP患者PAIg's、PAIgG和PAIgM水平显著增高,慢性ITP患者PAIgG水平增高;CD80、CD86表达与PAIgG水平之间存在显著的相关性(均P0.01)。结论:ITP患者外周血B淋巴细胞上CD86和CD80表达均异常,可能与其发病相关。     

Interleukin 18 and interleukin 18 binding protein in patients with idiopathic thrombocytopenic purpura

To evaluate the balance of interleukin IL18 and its endogenous antagonist IL18 binding protein ( IL18BP ) in patients with idiopathic thrombocytopenic purpura (ITP), plasma IL18 , IL18BP , interferon gamma ( IFNG ) and IL4 levels, as well as platelet counts were measured in patients with active ITP ( n  = 23), ITP in remission ( n  = 21) and in healthy subjects ( n  = 24) by enzyme linked immunosorbent assay (ELISA). Using real-time quantitative polymerase chain reaction, the mRNA expression of IL18 , IL18BP , IFNG , IL4 , T-box ( TBX21 ) and GATA-binding protein 3( GATA3 ) were studied in all subjects. The results showed that IL18 and IFNG protein and mRNA levels were significantly increased in patients with active ITP than in control subjects, but that IL18BP were not significantly elevated in ITP patients, which resulted in an elevated ratio of IL18/IL18BP in patients with active disease. During remission stages, the levels of these cytokines were comparable to those of healthy controls. The elevated levels of IL18/IL18BP in plasma during active stages of disease suggest a possible role in the pathogenesis and course of ITP.