小儿特发性血小板减少性紫癜的有关临床特点

目的探讨小儿特发性血小板减少性紫癜(ITP)的临床特点。方法对我院收治的255例ITP患儿的临床资料进行分析。结果1、男:女=1.43,中位年龄31个月,2岁以下占47.06%;急性型占91.37%,慢性型占8.63%。2、47.84%有前驱感染病史,31.76%在发病前1~4周有预防接种史。3、病原学检查阳性率73.81%,其中HPVB1945.24%。4、预防接种疫苗中乙肝疫苗34.57%,百白破疫苗24.69%,麻疹疫苗8.64%。5、临床表现94.12%以轻、中度皮肤粘膜出血为主,重度出血仅占5.88%。6、就诊时血小板数量:平均22.47×109/L,≤20×109/L占56.47%。7、骨髓常规涂片巨核细胞总数增多的占77.06%,分类中成熟无血小板产生的巨核细胞数>原始幼稚巨核细胞数>成熟有血小板产生的巨核细胞数>裸核巨核细胞数。8、给予以肾上腺皮质激素为主的治疗,97.42%血小板在2周内达正常,复发率4.29%。9、疫苗相关ITP的中位年龄6月,就诊时平均血小板数量22.3×109/L,95.06%患儿为轻中度出血;骨髓巨核细胞数增多者占75.68%;病原学检查阳性率为85.71%,其中HPVB19占64.29%;93.83%患儿治疗后平均4.90天血小板恢复正常水平,复发率3.7%。结论1、小儿ITP患者大多数为急性型,预后良好。2、病毒感染与小儿ITP关系密切,HPVB19在小儿ITP发病中有重要意义。3、疫苗相关的ITP发生率高于以往报道,除发病年龄小外临床特点与其他ITP相似,相关疫苗中以乙肝、百白破疫苗多见,应引起注意。4、HPVB19阳性患儿临床特点与一般ITP大致相同。5、以肾上腺皮质激素为主的治疗方案治疗小儿ITP疗效显著;大剂量丙种球蛋白和大剂量肾上腺皮质激素对有严重出血或血小板极低的患儿止血效果明显,可以避免血小板输注和相关死亡的发生。     

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患儿合并柯萨奇B组病毒感染的临床特点及分析

目的　探讨特发性血小板减少性紫癜 (ITP)患儿合并柯萨奇病毒B组 (CVB)感染的临床特点。方法　对 44例ITP患儿做骨髓细胞学分类 ,同时进行CVB、巨细胞病毒 (CMV)、EB病毒、乙肝病毒 (HBV)检查及血常规动态观察。结果　ITP患儿合并病毒感染中CVB明显多于EB、CMV、HBV等 ,有CVB感染阳性与CVB感染阴性的ITP患儿比较 ,骨髓巨核细胞总数明显增高 (P <0 0 1) ,外周血血小板计数和平均血小板体积明显降低(均P <0 0 1)。结论　CVB感染对ITP患儿血小板系统可能具有抑制作用     

Duration and morbidity of chronic immune thrombocytopenic purpura in children: five-year follow-up of a Nordic cohort

Aim: To describe the clinical course, morbidity and platelet recovery in an unselected Nordic cohort of children with chronic Immune Thrombocytopenic Purpura (ITP). Methods: Prospective 5‐year follow‐up of 96 children with ITP lasting more than 6 months, with reporting of hospital admissions, severity of bleeding episodes and stabilization of platelet counts above 20, 50 and 150 × 10⁹/L. Results: The estimated 5‐year recovery rate was 52%; exclusion of 12 splenectomized children did not change the estimate. Events eliciting admission to hospital occurred in 39 (41%). Major haemorrhages occurred in eight children (8%), including a nonfatal intracranial haemorrhage in one child (1%). The overall admission rate was 0.4/year of thrombocytopenia, decreasing during follow‐up as thrombocytopenia converted to milder degrees. Early recovery within 2 years of diagnosis occurred in 35%, was associated with low morbidity and was more likely in young children with abrupt onset of symptoms. Conclusion: In a Nordic cohort of children with chronic ITP, one half had recovered 5 years after diagnosis, more than half never required hospitalization and <10% experienced serious bleeding episodes, always with a platelet count <20 × 10⁹/L. Aggressive management can be restricted to the minority of children with continuing severe thrombocytopenia and frequent, clinically significant bleeding events.     

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。     

幽门螺杆菌感染与儿童特发性血小板减少性紫癜

特发性血小板减少性紫癜(ITP)是以自身抗体介导的血小板破坏为特征的一组疾病,近年来许多研究显示部分成年人ITP的发生与幽门螺杆菌(Hp)感染密切相关,清除Hp后ITP患者的血小板数量有明显提高.研究认为Hp相关的ITP与抗原交叉反应有关,但其确切机制目前尚不明确;儿童ITP是否需要清除Hp治疗,国内外也存在争议.     

HLA frequencies and haplotypes in children with idiopathic thrombocytopenic purpura (ITP)

26 patients with an acute reversible ITP and 6 with chronic ITP were tissue typed, together with their healthy first-degree relatives. The HLA frequencies of the different groups were compared with those of a normal control population. The only significant difference between the groups was an increase in the frequency of Aw32 in acute ITP patients. HLA-Aw32 was present in 26.9% of patients, but in only 0.8% of the controls (corrected P=0.000027). The possible importance of associations between antigens of the HLA-A locus with certain diseases are discussed. Family analyses and haplotype determinations proved to be unproductive because no familial clustering of ITP was found.     

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。     

Anti-D immunoglobulin in the treatment of idiopathic thrombocytopenic purpura

Anti-D was evaluated in 8 RhD positive patients (6 males, 2 females) aged 2–21 years (mean 10 years) with idiopathic Thrombocytopenic Purpura (ITP). Five patients with chronic ITP and 3 patients with acute ITP were administered Anti-D in the dosage of 50 ug/kg intramuscularly (IM) for 3 consecutive days. One patient of chronic ITP received two courses of Anti-D. Patients were followed up for 7 to 16 months (mean 9 months). All three cases of acute ITP had a complete response and are in remission between 3 to 12 months of follow up. Two of five cases of chronic ITP had a partial response. Rise in platelet count was observed within 72–124 hours, and duration of response varied between 10 to 15 days. None of these patients had any significant side effects of anti-D immunoglobulin therapy. Intramuscular administration of anti-D is safe, effective and low cost alternative to IVlgG in the treatment of acute ITP.     

High doses of gamma globulin and methylprednisolone therapy for idiopathic thrombocytopenic purpura in children

In four children with chronic idiopathic thrombocytopenic purpura (ITP), high doses of gamma globulin or methylprednisolone were effective in increasing platelet counts transiently, yet there was no quantitative relationship between the platelet responses to these two therapies. This finding suggests a qualitative difference in the mechanisms of increasing platelets by the two drugs. High doses of gamma globulin and methylprednisolone showed a synergistic effect on the initial and maximal platelet responses, suggesting an intensification of the effect of gamma globulin by methylprednisolone. This combination therapy should be clinically useful, particularly in the control of acute haemorrhage or in preparations for emergency surgery.Abbreviation ITP idiopathic thrombocytopenic purpura Offprint requests to: Department of Pediatrics, Faculty of Medicine, Kyushu University, 60, 3-1-1, Maidashi,Higashiku, Fukuoka, 812, Japan     

Evaluation of Japanese practice guidelines for newly diagnosed childhood idiopathic thrombocytopenic purpura

Background: Practice guidelines for childhood idiopathic thrombocytopenic purpura (ITP) were proposed in 1998 and 2004 in Japan. It is important to evaluate the feasibility and validity of the guidelines because the guidelines are based not on evidence but on opinion. Methods: Records of 30 consecutive hospitalized patients aged 0–15 years (median, 3 years; 17 boys, 13 girls) were retrospectively reviewed. The feasibility and validity of the 2004 guidelines were evaluated by calculating an implementation rate, a response rate to first treatments, and a final clinical outcome. Results: Deviation from the guidelines was found in two patients. Management of the other 28 patients followed the guidelines. The implementation rate was 93.3%. Twenty‐one of the 28 eligible patients (75%) responded to the first treatment, whereas seven patients (25%) required additional treatment. In 23 patients (76.7%) platelet counts returned to normal within 6 months. Seven patients (23.3%) developed chronic conditions. All patients had no complications and achieved a platelet count >150 000/µL within 6 years. Conclusion: The 2004 Japanese guidelines are feasible and valid for the management of newly diagnosed childhood ITP patients.     

儿童慢性特发性血小板减少性紫癜的临床转归

目的总结儿童慢性特发性血小板减少性紫癜(cITP)的临床经过及主要临床特点,以期对儿童cITP的应对策略有所帮助。方法回顾性分析58例儿童慢性ITP的自发缓解情况与主要临床特点。结果(1)58例cITP患儿中,33例(56.9%)获得自发缓解,中位缓解时间1年;预计累计自发缓解率在诊断后6~12个月、2、3和4年内分别为40%,47%,47%,55%,无死于出血者。(2)儿童cITP的起病年龄、性别、前驱感染史、病初PLT、抗核抗体及病初对药物治疗的反应均与疾病转归无关(P≥0.05)。(3)急、慢性ITP患儿的最低血小板计数分布基本相同(P=0.156),轻、中度出血的分布没有差别(P=0.329),但cITP重度出血多于急性ITP,分别为13.8%和1.9%。结论儿童cITP中的部分病例也呈现自限性经过,起病时的临床表现及早期药物治疗对疾病转归无明显影响。     

Corticosteroids versus intravenous immune globulin for the treatment of acute immune thrombocytopenic purpura in children: a systematic review and meta-analysis of randomized controlled trials

OBJECTIVE: To compare the effectiveness of corticosteroids with intravenous immune globulin (IVIG) for the initial treatment of children with acute immune thrombocytopenic purpura (ITP). STUDY DESIGN: A systematic review and meta-analysis of randomized controlled trials comparing corticosteroids with IVIG. Studies were identified from eight electronic databases, meeting abstracts, expert consultation, and hand-searched reference lists. Two authors independently reviewed potentially eligible studies and extracted data. The number of patients with a platelet count >20,000/mm3, 48 hours after treatment initiation, was the primary outcome. Relative risks (RR) and risk differences were pooled using a random effects model, and numbers needed to treat (NNT) were calculated. RESULTS: A total of 1248 abstracts were reviewed, 55 articles were retrieved, and 10 studies were included. The RR (steroids vs IVIG) of achieving a platelet count >20,000/mm3 at 48 hours was 0.74 (95% CI: 0.65, 0.85), and the NNT was 4.55 (95% CI: 3.23, 7.69). CONCLUSION: Children treated with corticosteroids for acute ITP are 26% less likely to have a platelet count >20,000/mm3 after 48 hours of therapy, when compared with children treated with IVIG. Given the importance of low platelets in the pathogenesis of intracranial hemorrhage (ICH), this difference may hold important clinical implications.     

个体化剂量丙种球蛋白联合地塞米松治疗儿童重症特发性血小板减少性紫癜78例疗效观察

目的观察个体化剂量静脉注射用丙种球蛋白(IVIG)联合地塞米松治疗儿童特发性血小板减少性紫癜(ITP)的疗效。方法重症ITP患儿入院后均给地塞米松及IVIG[400 mg/(kg.d)],3 d后测外周血血小板计数,≥100×109/L停用IVIG,继用地塞米松;若<100×109/L,再继续用原剂量IVIG 2 d。结果治疗3 d后,78例患儿中,37例外周血血小板计数升至100×109/L以上,余41例继续治疗后,31例升至100×109/L以上。结论个体化剂量IVIG联合地塞米松是治疗儿童重症ITP的有效办法,可节省一定医疗费用。     

Intended management of children with acute idiopathic thrombocytopenic purpura: a national survey

OBJECTIVE: In Australia acute idiopathic thrombocytopenic purpura (ITP) is mainly treated by paediatricians (either general paediatricians or paediatric haematologists/oncologists). A survey was conducted to gauge the current practice of treating children with acute ITP in Australia. METHODS: All practising Australian paediatricians registered by the Royal Australasian College of Physicians were surveyed regarding their intended management of children with acute ITP. The questionnaire, adapted from a study of paediatric haematologists/oncologists in North America, presented four clinical scenarios of children with acute ITP with a platelet count of 3000 x 10(9)/L, with and without mucosal bleeding (wet and dry purpura, respectively). Questionnaires were returned by mail or filled in online at a dedicated webpage. RESULTS: Five hundred and sixty-three of 1097 (51%) paediatricians responded to the survey. Data from 140 who had treated at least one child with ITP in the previous 12 months were analysed. Respondents indicated that children with acute ITP are usually or always hospitalised (58-92%) and that 48% would be given active treatment, even with dry purpura. Various regimens of i.v. immunoglobulin or corticosteroids are used when treatment is administered. In comparing Australian and North American management of acute ITP there were many similarities, although Australian paediatricians were less likely to arrange a bone marrow aspirate if corticosteroids were prescribed. CONCLUSIONS: There is great variation in the intended management of children with acute ITP in Australia. Previously published management recommendations regarding investigation and treatment have had little impact on intended practice. Prospective studies are required to evaluate hypotheses so as to produce evidence-based recommendations for treatment of patients with acute ITP.     

TPO、TSP-1和TGF-β_1在特发性血小板减少性紫癜患儿骨髓表达的改变及其意义

目的探讨特发性血小板减少性紫癜(ITP)患儿骨髓血小板生成素(TPO)、血小板反应素1(TSP1)和转化生长因子β1,(TGFβ1)水平变化的意义。方法收集22例ITP患儿及17例相对正常儿童骨髓;ELISA法检测骨髓上清液TPO、TSP1和TGFβ1的含量。结果ITP患儿骨髓TSP1和TGFβ1水平均明显高于对照组(P<0.05);TPO水平稍高于对照组,但差异无显著性(P>0.05)。结论检测ITP患儿的TPO、TSP1和TGFβ1表达水平对研究ITP的发病机制有重要价值。     

特发性血小板减少性紫癜患者血小板抗体及淋巴细胞亚群的研究

目的探讨血小板相关抗体(PAIgG)和T淋巴细胞亚群的变化,在特发性血小板减少性紫癜(ITP)免疫发病机制中的作用、临床意义。方法采用间接免疫荧光法测定30例ITP患者及20例正常对照组的PAIgG,20例ITP患儿治疗后复查PAIgG。同时采用流式细胞仪直接免疫荧光法检测外周T血淋巴细胞亚群。结果ITP组PAIgG阳性率为80%,正常对照组为20%(P<0.001),ITP组PAIgG明显高于正常对照组(P<0.001),20例ITP患儿治疗后复查PAIgG,其数值明显下降,差异有显著性(P<0.001)。T淋巴细胞亚群中,ITP组CD3、CD4、CD4/CD8显著低于正常对照组(P<0.01),CD8则显著高于正常对照组(P<0.01)。结论抗血小板相关抗体对提高ITP的诊断、疗效及预后的判断有一定的实用价值,T淋巴细胞亚群的变化能较好的反映ITP的病理机制。     

急性特发性血小板减少性紫癜患儿免疫功能变化及其临床意义

目的检测特发性血小板减少性紫癜(ITP)患儿的免疫功能并探讨ITP的发病机制。方法抗血小板抗体(PAIgG)测定采用放射免疫法,IgA、IgG、IgM测定采用WL-快速免疫消浊比浊法,T淋巴细胞亚群测定采用APAAP法,血清IL-2、sIL-2R和IL-6测定采用ELISA法。结果 ITP患儿的IL-2、IL-6,IgA、IgM、CD8及IL-2R表达均明显升高(P<0.01),CD4+、CD4+/CD8+细胞均明显减少(P<0.01)。结论细胞免疫及体液免疫异常共同参与ITP致病机制,调节淋巴细胞亚群平衡有助于寻找ITP治疗的新途径。     

儿童急性ITP Th1/Th2细胞功能状态初步研究

目的探讨急性儿童特发性血小板减少性紫癜(ITP)Th1/Th2细胞功能状态及其在ITP免疫发病机制中的作用。方法采用流式细胞术胞内标记法检测外周血CD4+T细胞IL-4和IFN-γ阳性表达率;逆转录-聚合酶链反应(RT-PCR)及荧光定量PCR检测外周血CD4+T细胞中IFN-γ、IL-4、IL-5、IL-13、T-bet、GATA-3、SOCS-1、SOCS-3和TIM-3等Th相关的细胞因子及转录因子mRNA表达。结果①急性ITP患儿Th1细胞阳性率明显低于正常同年龄对照组(3.36%±1.25%vs12.71%±2.29%,P<0.01),Th2细胞比例明显升高(2.63%±1.35%vs0.46%±0.17%,P<0.01),Th1/Th2比值显著降低(1.45±0.57vs34.13±5.76,P<0.01);②急性ITP患儿GATA-3、SOCS-3mRNA表达明显增高,T-bet及SOCS-5表达与同年龄对照组无显著差异(P>0.05),TIM-3表达明显增高(P<0.01);③急性ITP患儿CD4+T细胞高表达Th2类细胞因子IL-4、IL-5、IL-13(P<0.01),Th1类细胞因子IFN-γ与正常对照比较无显著性差异(P>0.05)。结论急性ITP患儿Th2细胞过度活化,可能与ITP免疫功能紊乱有关。