免疫抑制治疗无效的特发性血小板减少性紫癜患者血小板相关免疫球蛋白的亚型分析

目的分析糖皮质激素、环孢菌素A治疗无效的特发性血小板减少性紫癜(ITP)患者血小板相关免疫球蛋白(PAIg)的亚型。方法采用酶联免疫法(ELISA)对19992004年绵阳市中心医院收治的17例激素治疗无效、4例激素 环孢菌素A治疗无效以及30例激素治疗有效ITP患者的PAIg亚型进行检测。结果17例激素治疗无效的患者PAIg各亚型抗体较之激素治疗有效ITP患者差异有显著性(P<0·01),激素治疗无效组PAIg各亚型水平均明显增高;激素 环孢菌素A治疗无效组PAIgM较之激素治疗有效ITP患者有所增高(P<0·05)。结论激素治疗无效的ITP患者PAIg各亚型均显著增高,提示PAIg在介导ITP患者异常免疫反应过程中起重要作用,PAIg各亚型水平的增高可能有助于预测ITP患者对激素治疗的敏感性。     

评价血小板相关抗体定量测定法对特发性血小板减少性紫癜的诊断价值

目的 :评价血小板相关抗体 (PAIg)定量测定法对诊断特发性血小板减少性紫癜 (ITP)的价值。 方法 :应用酶联免疫竞争抑制试验 (ELISA)定量测定血小板相关抗体 ,计算试验的灵敏度、特异度、准确度、阳性预测值 ,绘制受试者ROC曲线进行诊断价值的评价。结果 :根据常用判断PAIg阳性的临界值 ,血小板相关抗体试验诊断ITP的灵敏度较高 (95 % ) ,但特异度低 (48.8% ) ,准确度低 (5 7.5 % ) ,阳性预测值低 (30 .2 % )。用ROC曲线寻找诊断ITP的最佳临界值 ,用新的临界值 (PAIgG >16 0ng/10 7血小板或PAIgA >2 5ng/10 7或PAIgM >35ng/10 7)时 ,可提高诊断试验的特异度 ,从 4 8.8%提高到 6 2 .8% ,可确诊更多的患者 ;但准确度和阳性预测值仍低 ,分别为 6 7.9%和 36 .0 %。结论 :PAIg试验诊断ITP的特异度、准确度、阳性预测值较低 ,帮助诊断ITP的临床价值有限 ,即使用新的临界值提高了试验的特异度 ,但诊断ITP的价值仍不大。     

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     

流式细胞术研究特发性血小板减少性紫癜患者血小板相关抗体

目的：探讨特发性血小板减少性紫癜（ITP）患者血小板表面相关抗体（PAIg）在诊断及预后的价值。方法：应用流式细胞术（FCM）检测84例ITP患者及20例正常人PAIgG、PAIgM、PAIgA。结果：初发ITP和复发ITP患者组与正常对照组比较，PAIgG、PAIgA差异有统计学意义（P〈0．01），PAIgM差异无统计学意义（P〉0．05）。PAIgM与PAIgA之间有显著相关性，r=0．451（P〈0．01）。结论：①PAIg增高可作为诊断初发ITP的重要指标之一；②FCM检测ITP患者血小板表面PAIg敏感性好、特异性高，适用于临床，对ITP的诊断及预后评价有较好的实用价值；③初发型ITP患者以PAIgG和PAIgA增高为主，预后较好；复发型以PAIgM增高为主，预后较差，易复发。     

单剂大剂量免疫球蛋白治疗复发性特发性血小板减少性紫癜

特发性血小板减少性紫癜 (ＩＴＰ)的发生与体内产生血小板相关抗体有关 ,临床经典治疗采用肾上腺糖皮质激素 ,但激素无效或复发的病例则须选择其它免疫抑制剂及脾切除术等治疗 .自Ｉｍｂａｃｈ[1] 首次使用静注免疫球蛋白 (ＩＶＩＧ)治疗ＩＴＰ以来 ,已有许多研究报告肯定了ＩＶＩＧ对ＩＴＰ的有效治疗作用 ,通常的用法为 0 .4 ｇ/ｋｇ ,每日 1次 ,连用 5ｄ。本文报告单剂大剂量静滴免疫球蛋白 (ｌｇ/ｋｇ)治疗复发性特发性血小板减少性紫癜的结果。病例资料患者男性 ,4 6岁。 7年前在外院诊断ＩＴＰ后口服泼尼松治疗 ,获完全缓解 ,大约半年…     

特发性血小板减少性紫癜的体液免疫机制

现代体液免疫学观点认为 ,特发性血小板减少性紫癜(ITP)的发生主要由自身抗体介导。自身抗体致敏的血小板通过巨噬细胞表面的Fcγ受体在脾脏等部位加速清除[1] 。笔者就近年来ITP体液免疫机制的重要研究进展加以综述。1 血小板相关抗体 :1975年 ,Dixon等[2 ] 首次用定量方法检测了血小板表面的免疫球蛋白 ,称其为血小板相关IgG(PAIgG)。此后国内外各种免疫检测方法均证明 :大多数ITP患者的PAIgG水平升高 ,PAIgG与血小板计数、血小板寿命间呈负相关 ,与血小板大小呈正相关。大量资料同时显示PAIgG升高对ITP并非特异 ,还可见于继…     

IgG-VCR联合治疗慢性特发性血小板减少性紫癜

特发性血小板减少性紫癜（ITP）临床多见，其病程迁延，容易复发，是一难治性疾病。我们采用静脉输注免疫球蛋白（IgG）和长春新碱（VCR）联合治疗ITP30例，收到良好效果，报告如下：1资料与方法1．1一般情况30例均为住院患者，男17例，女13例，年龄15～56岁，平均32岁。病程1～8年，平均3年。30例均符合第五届全国血栓与止血会议有关ITP的诊断标准。骨髓检查巨核细胞数正常或增高，其中多见颗粒型，成熟型少见。入院时血小板数（6．0～54）×109／L，平均28×109／L。其中7例血小板数（6．0～8．0）×109／L，属于重型。临床表现：…     

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,     

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。     

Sites of Platelet Destruction in Idiopathic Thrombocytopenic Purpura

Platelet life span and sites of platelet sequestration were studied on 20 occasions in 15 patients with idiopathic thrombocytopenic purpura (ITP) using ⁵¹Cr-labelled cells and external scintillation scanning. In 16 cases when platelet concentrations were greater than 5000 per cu.mm., labelled cells were destroyed predominantly in the spleen. Only in four patients with platelet levels less than 2500 per cu.mm. was significant hepatic sequestration observed. In the latter group, half-disappearance times of labelled cells were extremely short, ranging from 2 to 9 minutes. The data suggest that in ITP, as in other clinical and experimental situations where circulating cells are sequestered following their reaction with antibodies, lightly sensitized cells are removed from the circulation at a relatively slow rate by the spleen, while more heavily sensitized cells are liable to rapid destruction in the liver. Thus, in ITP, the site in which labelled cells are sequestered appears related to the severity of the disease at the time of study, rather than to any qualitative difference in the nature of the disease among individual patients.     

High Dose Intravenous IgG Reduces Platelet Associated Immunoglobulins and Complement in Idiopathic Thrombocytopenic Purpura

4 children with idiopathic thrombocytopenic purpura were treated with high dose i.v. IgG. Platelet associated immunoproteins were determined with an Elisa technique. Platelet counts increased in all patients, while simultaneously platelet associated IgG, IgA, IgM, C3 and C4 decreased. Serum antiplatelet antibody increased during treatment suggesting that administration of high doses of IgG interferes with the binding of antiplatelet antibody.     

Platelet Survival and Platelet Production in Idiopathic Thrombocytopenic Purpura (ITP)

S ummary . Platelet mean life span (MLS) and platelet production were measured in 3–5 patients with idiopathic thrombocytopenic purpura (ITP) and in 21 healthy subjects.
The mean platelet production in ITP was 2.3 times normal: the highest values were 3–5 times normal. There was a highly significant negative correlation between platelet production and peripheral platelet count. With platelet counts above 50000μl, platelet turnover was within the upper part of the normal range, but with lower platelet counts, turnover progressively increased. It is concluded that the bone marrow in ITP increases platelet production in response to a low platelet count and that this response does not differ from that hitherto known to occur in man and animals rendered thrombocytopenic by thrombopheresis.
The disappearance curve of ⁵¹Cr labelled platelets in ITP consists of two components, a rapid initial one covering the first 15 min after infusion and then a slower one. The pattern was the same whether autologous or homologous platelets were used for labelling. It is suggested that the initial part of the curve does not represent in vivo survival but is due to slight damage to the platelets during the labelling procedure. These slightly damaged cells can resume normal viability when infused into a normal recipient but are rendered less viable when further damaged by platelet antibodies in patients with ITP. This explains the low recovery of infused labelled platelets in ITP recipients, despite the fact that the size of their splenic platelet pool is normal.     

小儿特发性血小板减少性紫裥抗血小板抗体亚类的研究

采用鼠抗人IgG亚类单克隆抗体酶联免疫吸附法对急、慢性ITP患儿进行血小板表面相关IgG(PAIgG)亚类及血清IgG亚类测定。结果表明:ITP患儿抗血小板抗体有IgG亚类限制性,主要以PAIgG_1和PAIgG_3增高为主。其原因可能与某些感染及免疫调节异常有关。血清IgG亚类缺陷可能是ITP病因之一。PAIgG亚类在诊断和判断疗效中起重要作用。     

ITP患者血小板活化依赖性自身抗原的研究

本文采用改良单克隆抗体特异性抗原捕获酶免疫法(MACE)使用5种抗原血小板单克隆抗体对59例ITP患者进行了相关自身抗原研究,结果28例ITP患者血浆呈现抗血小板自身抗体阳性(47.46％),其中单独抗GPI_b/2例、抗GPⅡ_b/Ⅲ_α5例、抗α颗粒膜蛋白-1401例,抗凝血酶敏感蛋白6例,另14例出现2种以上自身抗体,以抗GPⅡ_b/Ⅲ_α为多见。找们建议将以上抗体针对的抗原称为血小板活化依赖性自身抗体。虽然我们未发现这类自身抗原与其它类型自身抗原间的临床表现及治疗结果存在明显差异,但这类自身抗原应引起重视,有必要对此作进一步观察和深入研究。     

Adult Idiopathic Thrombocytopenic Purpura

The therapeutic options for the indolent non-Hodgkin's lymphomas have evolved significantly over the years. However, at present, allogeneic haematopoietic stem cell transplantation (HSCT), despite its significant transplant associated complications, is still the only option which may be delivered with curative potential. The debut of reduced-intensity conditioning regimens has extended the applicability of allogeneic transplants to groups of patients ineligible for conventional myeloablative allografts. However, there are still substantial hurdles to overcome, namely that of relapse, graft-versus-host disease and infection. Whilst follow-up is still relatively limited for a disease group with a median survival of up to 10 years, early results are extremely encouraging and further studies are warranted.     

Idiopathic Thrombocytopenic Purpura in Children; Detection of Platelet Autoantibodies by Immunofluorescence

There are 3 varieties of idiopathic thrombocytopenic purpura (ITP) in children: the acute, the chronic and the recurrent forms. We have tested the platelets from 80 children with these forms of ITP by an immunofluorescence test. Platelet-bound immunoglobulins were detected in 19/22 acute, 41/48 chronic and 5/10 recurrent cases of ITP, i.e. in 65 of a total of 80 cases. A significance differences between acute and chronic ITP was found in the observation that only IgM was bound to the platelets of 7/16 patients with acute ITP. In all cases of chronic ITP with a positive direct immunofluorescence test, IgG was present on the platelet membrane. It is assumed because some patients with chronic ITP do not have elavated platelet-bound immunoglobulins, and show refractoriness to immunosuppressive therapy, that in these patients the disorder is not of a humoral autoimmune nature.     

Microthrombocytosis and Platelet Fragmentation Associated with Idiopathic/Autoimmune Thrombocytopenic Purpura

Platelet volume distribution curves were obtained in 20 control subjects and in 21 patients with idiopathic/autoimmune thrombocytopenic purpura. A striking increase in microthrombocytes as well as megathrombocytes was noted in 86% of patients on one or more occasions, particularly in the prsence of severe thrombocytopenia. The entire spectrum of platelet volume distribution curves noted in patients could be reproduced experimentally in rabbits following intravenous injection of anti-platelet antibody. Differential centrifugation studies with control subjects revealed that microthrombocytes were light platelets and megathrombocytes were heavy platelets. Electron microscopy in patients with thrombocytopenia revealed that microthrombocytes were composed of intact small platelets as well as platelet fragments. It is concluded that severe peripheral destruction of platelets is associated with an increase in microthrombocytes as well as megathrombocytes.