病毒感染与慢性特发性血小板减少性紫癜

目的为探讨病毒感染与慢性特发性血小板减少性紫癜(CITP)的关系。方法选择32例CITP患者(年龄8个月~13岁,男13,女19)及30例健康查体正常对照儿童(年龄1岁~14岁,男14,女16)。采用间接ELISA法测定两组血清巨细胞病毒(CMV)、肝炎病毒[包括乙型肝炎病毒(HBV)和丙型肝炎病毒(HCV)]和人类疱疹病毒(HHV)相关抗体。结果慢性ITP患儿各种病毒相关抗体阳性率高于对照组,其中巨细胞病毒抗体阳性率两组间比较差别具有显著性意义(P<0·05)。结论①慢性反复性病毒感染可能是导致ITP反复发作的重要原因之一,②临床上对ITP患者特别是慢性ITP患者应该常规进行病毒学实验室检查,有助于判断预后,指导治疗。     

儿童特发性血小板减少性紫癜研究进展

儿童特发性血小板减少性紫癜(ITP)虽是一种常见疾病,但诊断和治疗仍有很多未能解决的问题。儿童发病有其特点起病急、自限性居多;男童发病率偏高;以严重血小板减少为主,而少见威胁生命的严重出血;临床过程大多平静,多数6个月内恢复,未恢复的患儿仍有一定自行恢复可能,因此被定义为仅有血小板减少、而其他方面健康的儿童身上发生的疾病状态。在发病机制方面已由过去简单认为血小板特异抗体吸附引起单核-巨噬系统吞噬造成血小板减少衍变到对机体免疫尤其是细胞免疫异常的认识,个体的遗传易感性也成为关注的焦点。被称为“免疫捕捉法”的血小板抗体检测方法逐步替代特异性差的血小板抗体检测法,血小板功能和幽门螺杆菌的监测及意义尚需探讨。对血小板(20~30)×109/L且无明显出血的患儿,观察和等待是最佳方法,而需要治疗时丙种球蛋白和糖皮质激素是一线治疗方法。目前新的治疗方法有大剂量地塞米松、抗CD20、血小板生成素(TPO)类似物等。由于远期效果不肯定、手术并发症及感染暴发,儿科医师对于脾切除越来越持谨慎态度。ITP患儿的社会心理情况也引起了人们重视。相信随着科学发展,儿童ITP的相关问题会逐步得到解决。     

特发性血小板减少性紫癜的诊断和治疗

目的特发性血小板减少性紫癜(ITP)是一种因免疫异常导致的疾病。它的诊断目前主要依靠病史、临床表现、实验室检查。但目前对其诊断、治疗仍然存在争议。本文对近年来有关ITP的诊断、临床分级、鉴别诊断、治疗情况作一介绍。实用儿科临床杂志,2006,21(3):185-188     

特发性血小板减少性紫癜诊断治疗进展

特发性血小板减少性紫癜是小儿最常见的血小板减少性疾病,其发病机制尚不完全清楚,诊断上需与其他伴随血小板减少疾病相鉴别,大部分患者预后较好,但少数慢性难治性病例可反复发作,处理较为棘手。现将该病近年来诊断和治疗方面的一些新进展作一介绍,供临床参考。     

进一步提高小儿特发性血小板减少性紫癜的研究水平

特发性血小板减少性紫癜（ＩＴＰ）是常见的小儿获得性出血性疾病,其年发病率为小儿群体的４／１０万～８／１０万［１］。小儿病例多为急性型。急性ＩＴＰ病例中７％～２８％可转为慢性型［１,２］。一、关于诊断、治疗问题１９８７年全国小儿血液病学术会议拟定了“特...     

儿童特发性血小板减少性紫癜血小板功能研究

目的研究儿童特发性血小板减少性紫癜(ITP)血小板体内活化状态及体外活化功能,探讨血小板在ITP发病机制中的作用。方法流式细胞术(FCM)检测体内和体外5’-二磷酸腺苷(ADP)刺激后血小板表面活化分子GPIIb/IIIa和CD62P分子的表达,同时检测CD62P阳性血小板该分子的平均荧光强度(MCF)。结果ITP组与对照组体内活化血小板百分比有显著性差异(P=0.0001),ITP组显著低于对照组;两组CD62P分子的MCF无显著性差异。体外刺激血小板活化后,两组活化血小板百分比有显著性差异(P=0.0077),ITP组低于对照组;两组CD62P分子的MCF有显著性差异(P=0.0059),ITP组低于对照组。结论ITP患儿体内血小板处于低活化状态,提示其出血原因不仅是血小板数量减少,同时存在血小板功能减低,低活化的原因很可能是血小板自身功能障碍。     

特发性血小板减少性紫癜血小板参数变化及其临床意义

目的探讨特发性血小板减少性紫癜(ITP)时血小板参数的变化及其临床意义。方法对确诊为急性型特发性血小板减少性紫癜患儿67例分别测定其治疗前后的血小板数(PLT)、平均血小板体积(MPV)及血小板分布宽度(PDW)及大血小板比率(P-LCR),同时测定50例健康儿童以上血小板参数作为对照组。治疗前与对照组及与治疗后分别进行比较。并对血小板与血小板参数进行相关性分析。结果(1)ITP患儿治疗前PLT明显低于对照组,而PDW、MPV、P-LCR明显高于对照组,差异均具有极显著性(P<0·001),治疗后PLT上升,PDW、MPV、P-LCR下降,与治疗前比较差异具有极显著性(P<0·001)。(2)ITP轻、中、重度患儿随病情加重PLT进行性下降,而PDW、MPV、P-LCR进行性增大,但极重度患儿MPV反而变小。(3)PLT与MPV呈负相关(P<0·001)。MPV与PDW、PDW、MPV与P-LCR、PDW与P-LCR呈正相关(P<0·05)。结论血小板参数的动态观察有助于ITIP的鉴别诊断、病情判断及疗效观察。     

特发性血小板减少性紫癜125例临床疗效分析

本文总结了常用的五种治疗ＩＴＰ方案,探讨适合我国国情及小儿ＩＴＰ特点的治疗方法,Ⅰ组口服氨肽,素。Ⅱ组在Ⅰ组在的基础上加氢化考的松静点后改强的松口服。Ⅲ组在Ⅰ、Ⅱ组的基础上加用ＩＶＩｇ。Ⅳ组则在Ⅰ组基础上单用ⅣＩｇ。Ⅴ组在Ⅰ、Ⅱ组基础上加长春新碱静注。结果：五组治疗总有效率分别为１００％、８１．７％、７５％、６０％、５０％。统计学处理后Ⅰ组疗效优于Ⅳ、Ⅴ组,而Ⅰ组与Ⅱ组、Ⅲ组之间疗效无显著差异。结     

特发性血小板减少性紫癜患者血浆内皮素初探

探讨特发性血小板减少性紫癜患者血浆内皮素的变化。方法：对80例特发性血小板减少性紫癜患者血浆内皮素进行放免分析测定。结果：特发性血小板减少性紫癜患者血浆内皮素明显升高，不同程度的出血组间及急性期与恢复期存在着显著差异（P＜0．01）。结论：血浆内皮素在特发性血小板减少性紫癜患儿中明显增高，同出血程度的轻度呈正相关性，并随着疾病的恢复而降低。     

Simplified Immunoglobulin Treatment of Idiopathic Thrombocytopenic Purpura

ABSTRACT. The recommended dose of intravenous IgG for idiopathic thrombocytopenic purpura has been 0.4 g/kg on 5 consecutive days. A simplified approach, giving a single infusion of 0.8–1.0 g/kg over 8 hours, has been tried in a series of 11 children with newly diagnosed disease. In 8 cases the infusion produced a prompt platelet response culminating at 128–502×10⁹/1 after 3–13 days, and 4 of these cases required no further treatment while 2 needed a booster infusion due to an early relapse and 2 followed a chronic course. In 3 cases platelet responses were poor in spite of supplementary doses to a total of 1.4–2.0 g/kg: 2 infants failed to achieve normal platelet counts and 1 case with fulminant bleeding manifestations proved completely resistant. Significant side effects were not observed. These results indicate that IgG-therapy practically may be initiated with a single infusion, the resulting platelet response indicating the need for further infusions.     

Oral Megadose Methylprednisolone Versus Intravenous Immunoglobulin for Acute Childhood Idiopathic Thrombocytopenic Purpura

Twenty children with acute idiopathic thrombocytopenic purpura (ITP) were randomized to receive either oral megadose methylprednisolone (MDMP) or intravenous immunoglobulin G (IV IgG). Normal platelet counts (150 × 10⁹/l) were obtained in 6 patients of each group in 3 days and in 8 and 9 patients treated with oral MDMP and with IV IgG within 1 week, respectively. It is concluded that oral MDMP could easily be used for the early elevation of platelet counts, which is important for ITP treatment.     

Treatment of Acute Idiopathic Thrombocytopenic Purpura (AITP): Cooperative Italian Study Group Results

A cooperative Italian study group on acute idiopathic thrombocytopenic purpura (AITP) has been designed to evaluate efficacy and safety of no treatment at the onset of the disease and sequential treatment with immunoglobulin and high dose steroid. One hundred thirty-eight patients with AITP entered in the trial. Eleven patients were treated before the end of the waiting period because of bleeding. One hundred twenty-seven (92%) received no treatment for the first 10 days of the disease, 65 patients (51.18%) recovered spontaneously, 62 patients were treated with immunoglobulin, and 52 (83.8%) of them responded positively but only 36 (58.06%) permanently. There was no statistical difference between the results obtained with 400 mg/kgfor 5 days versus 200 mg/kg. Twenty-four patients were treated with high doses of steroids, 20 (83.3%) with positive response, and 10 (41.66%) were permanently cured. Four (3.14%) of the patients enrolled in the protocol still had active disease at the end of treatment, and 10 relapsed within 4 months after the end of the treatment.     

Treatment of Acute Idiopathic Thrombocytopenic Purpura (AITP): Cooperative Italian Study Group Results

A cooperative Italian study group on acute idiopathic thrombocytopenic purpura (AITP) has been designed to evaluate efficacy and safety of no treatment at the onset of the disease and sequential treatment with immunoglobulin and high dose steroid. One hundred thirty-eight patients with AITP entered in the trial. Eleven patients were treated before the end of the waiting period because of bleeding. One hundred twenty-seven (92%) received no treatment for the first 10 days of the disease, 65 patients (51.18%) recovered spontaneously, 62 patients were treated with immunoglobulin, and 52 (83.8%) of them responded positively but only 36 (58.06%) permanently. There was no statistical difference between the results obtained with 400 mg/kgfor 5 days versus 200 mg/kg. Twenty-four patients were treated with high doses of steroids, 20 (83.3%) with positive response, and 10 (41.66%) were permanently cured. Four (3.14%) of the patients enrolled in the protocol still had active disease at the end of treatment, and 10 relapsed within 4 months after the end of the treatment.     

抗-D免疫球蛋白治疗儿童特发性血小板减少性紫癜研究进展

抗-D免疫球蛋白的临床应用是特发性血小板减少性紫癜(ITP)的治疗方法。研究表明抗-D免疫球蛋白治疗儿童ITP有效、安全、经济、使用方便,可以替代IVIG作为一线治疗药物。2011年,美国血液病协会在以证据为基础的免疫性血小板减少症的实践指南中建议Rh阳性、脾未切除的患者使用抗-D免疫球蛋白作为一线治疗药物。现就抗-D免疫球蛋白的作用机制、治疗和不良反应进行综述。     

网织血小板测定对特发性血小板减少性紫癜诊疗的价值

目的　探讨外周血网织血小板 (RP)变化对特发性血小板减少性紫癜 (ITP)的诊断价值及疗效判定。方法　利用流式细胞仪测定ITP(ITP组 )、再生障碍性贫血及白血病 (非ITP组 )及对照组外周血RP百分率 ,并计算其绝对值。结果　ITP组RP百分率明显高于非ITP组及对照组 (P <0 .0 1 ) ,绝对值明显低于对照组 (P <0 .0 1 ) ;非ITP组RP百分率与对照组比较无明显差异 (P >0 .0 5) ,但RP绝对值明显低于对照组 (P <0 .0 1 ) ;ITP组治疗有效者RP百分率明显降低 ,与治疗前比较差异显著 (P <0 .0 1 )。结论　RP百分率及绝对值可反映骨髓血小板生成情况 ,对血小板减少症发病机制判断具有重要意义 ,可作为血小板减少症鉴别诊断的重要指标 ,是确诊ITP诊断依据和疗效判定的监控指标。     

婴幼儿特发性血小板减少性紫癜401例临床特点

目的 分析婴幼儿特发性血小板减少性紫癜(ITP)的临床特点,并比较婴儿与幼儿ITP的疗效.方法 收集401例婴幼儿ITP,均给予激素冲击治疗和静脉滴注免疫球蛋白治疗,疗效判定依据血小板计数的高低和出血症状的改善分为完全缓解、有效、无效.401例婴幼儿ITP按年龄分为婴儿组(≤1岁)和幼儿组(>1～3岁),按病程分为急性(病程≤6个月)和慢性(病程>6个月),对其临床资料进行回顾性分析,应用SPSS 12.0软件进行统计分析.结果 1.婴儿组与幼儿组均为男童比例高,但2组间性别比较差异无统计学意义(χ2=0.682,P>0.05).2.婴儿组入院时血小板中位计数低于幼儿组,差异有统计学意义(Z=2.668,P<0.05).3.婴儿组骨髓巨核细胞增高比例低于幼儿组,差异有统计学意义(χ2=16.322,P<0.001);婴儿组产板巨核细胞中位数低于幼儿组,差异有统计学意义(Z=2.065,P<0.05).4.婴儿组经治疗后血小板计数达到或超过100×109 L-1的时间短于幼儿组,差异有统计学意义(Z=3.542,P<0.001).5.急性患儿入院时血小板中位计数低于慢性患儿,差异有统计学意义(Z=2.100,P<0.05).6.输注血小板患儿的住院时间与未输注血小板患儿相比,差异无统计学意义(Z=1.385,P>0.05).结论 婴幼儿ITP中,男童比例高,大部分无明显诱因,以皮肤黏膜出血为主;婴儿入院时血小板中位计数较低,血小板上升至正常的时间较短,对治疗反应较幼儿好,幼儿急性ITP可以变为慢性;输注血小板并不能缩短ITP患儿的住院时间.     

ITP患儿外周血T细胞T-bet/GATA-3基因的检测及意义

目的研究急慢性ITP患儿Th1、Th2类细胞因子和转录因子T-bet和GATA-3的相关性,探讨其在ITP发病机制。方法选择30例ITP患儿,运用T细胞分离富聚柱得纯化的T细胞,分别用ELISA法和RT-PCR技术检测ITP患儿血清中的IFN-γ、IL-4水平和外周血T细胞转录因子T-bet和GATA-3的表达状态,设30例健康儿童为对照组。结果慢性ITP患儿血清中IL-4水平较正常人降低,IFN-γ的水平升高,GATA-3 mRNA表达水平下降,T-be tmRNA表达水平升高;而急性ITP患儿外周血中IL-4水平较正常人升高,血清中IFN-γ水平及T-bet/GATA-3比值无显著性差异。结论慢性ITP患儿外周血Th1/Th2比例失衡,T-bet/GATA-3的表达失调,可能引起慢性ITP的免疫紊乱:而急性ITP患儿的发病机制可能与T-bet/GATA-3的表达无关。     

血小板生成素和巨核细胞对特发性血小板减少性紫癜患儿预后的影响

目的探讨血小板生成素(TPO)和巨核细胞计数对特发性血小板减少性紫癜(ITP)患儿预后的意义。方法采用夹心酶联免疫吸附法测定122例ITP患儿血清TPO水平,根据巨核细胞数分为A组(巨核细胞数≤35/6 cm2)与B组(巨核细胞数>35/6 cm2),两组进行TPO水平比较,并观察各组疗效。结果A和B组TPO水平分别为(368.33±39.47)ng/L,(141.48±11.28)ng/L。A组明显高于B组,有显著性差异(t=7.44 P<0.01)。A组治疗有效率为23.8%,明显低于B组84.2%,其差异有显著性(χ2=6.54 P<0.05)。A组有效5例TPO为(109.60±15.59)ng/L,无效16例TPO为(449.18±29.59)ng/L,其差异有显著意义(t=-6.23 P<0.01)。结论ITP患儿血清TPO水平存在较大差异,TPO水平明显增高且无巨核细胞数增高者疗效差,可能提示预后不良。