大剂量静脉丙种球蛋白治疗特发性血小板减少性紫癜

静脉丙种球蛋白(IVIG)是治疗特发性血小板减少性紫癜(ITP)的新方法,但白于药源和经济原因,我国临床应用仍不普遍.本文介绍5例IVIG治疗ITP的情况.     

两种剂量丙种球蛋白治疗重症特发性血小板减少性紫癜临床观察

重症特发性血小板减少性紫癜（ITP）患者,由于血小板数极低,有可能发生颅内及重要脏器出血而危及生命,需要大剂量丙种球蛋白（丙球）静脉点滴。但由于应用大剂量丙球费用昂贵,许多患者不能承受。我们应用亚标准剂量丙球治疗重症ITP,并与标准剂量治疗重症ITP进行对照观察。现将结果报道如下。     

静脉输注载长春新碱和足叶乙甙血小板治疗特发性血小板减少性紫癜

特发性血小板减少性紫癜 (ＩＴＰ)是临床上常见的获得性出血性疾病。本病首选糖皮质激素治疗 ,约 70 %～ 90 %的患者有较好的临床效果 ,但持续完全有效者仅约 2 5 %。Ｋａｐａｔｋｉｎ等[1] 将对糖皮质激素治疗无效或需口服较大剂量的泼尼松 (>10～ 15ｍｇ)才能维持血小板达安全水平 (>3 0×10 9/Ｌ)的成人病例视为难治性ＩＴＰ。对难治性ＩＴＰ的治疗是一个棘手的难题。由此 ,我们应用足叶乙甙和长春新碱偶联血小板治疗对糖皮质激素无效或耐药的ＩＴＰ病例 ,旨在探讨难治性ＩＴＰ治疗的有效方法。一、资料和方法1.对象 :2 0例难治性ＩＴＰ…     

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。     

脾切除治疗成人特发性血小板减少性紫癜

特发性血小板减少性紫癜 ( ITP)是一种自身免疫性疾病 ,多采用泼尼松或免疫抑制剂等药物治疗 ,但部分患者常因上述治疗无效 ,伴有较严重出血症状。1 980年以来 ,我院对 54例经药物治疗无效的成人 ITP患者行脾切除治疗。现报告如下。1 　 资料与方法1 .1 　 临床资料54例患者中 ,男 2 0例 ,女 34例 ;男 :女 =1 :1 .7;年龄 1 4～ 57岁 ,中位数 31 .5岁。急性型 5例 ,慢性型 49例。病程 <6个月者 1 1例 ( 2 0 .4% ) ,6～1 2个月者 1 7例 ( 31 .5% ) ,>1～ 1 3年者 2 6例( 4 8.2 % )。既往用药物治疗无效者 (包括暂时有效、但停药或减药后血…     

122例特发性血小板减少性紫癜血小板输注疗效观察

目的:探讨特发性血小板减少性紫癜(ITP)患者临床血小板输注的指征.方法:比较122例ITP患者输注血小板和未输注血小板的临床转归;比较血小板输注前及输注24 h后血小板计数.参照PAIg,分析抗体与血小板输注疗效的相关性.结果:67例未输注血小板的患者中,有2例PLT＜10×109/L的患者发生严重出血危及生命,55例输注血小板的惠者,有17例(31%)PLT较输注前降低,其中7例输注后PLT＜10×109/L,未发生严重出血;ITP患者血小板输注无效率86%,PAIg升高患者血小板输注无效率升高,有统计学差异.结论:如无明显的出血症状,ITP血小板输注指征建议PLT＜10×109/L,应输注少白细胞同型单采血小板制荆.     

特发性血小板减少性紫癜无效输注原因分析

目的:探讨特发性血小板减少性紫癜(ITP)患者血小板无效输注的原因.方法:90例确诊的ITP患者中对输注血小板的48例计算血小板计数增高指数(CCI),评价输注的效果并分析与感染、脾脏肿大、血小板抗体、骨髓巨核细胞数和免疫指标等因素的关系.结果:18例应用血小板和激素治疗的患者,44.4%有效输注,30例应用血小板、激素和丙种球蛋白患者,53.3%有效输注.24例无效输注患者中在感染组与非感染组、脾脏肿大组与正常组、血小板相关抗体升高组与正常组,有效输注率有显著性差异.骨髓巨核细胞数升高组与正常组、调节性T细胞低表达组与正常组,有效输注率差异无统计学意义.结论:ITP患者输注血小板50%存在无效输注,其原因可能与感染、脾脏肿大、血小板相关抗体有关.因此控制感染和抑制免疫应作为首选治疗,而血小板的输注应严格把握指征.     

特发性血小板减少性紫癜的治疗现状

特发性血小板减少性紫癜（ITP）是一组由多种原因引起的免疫机制障碍导致血小板破坏增加的临床综合征,是体液免疫与细胞免疫共同参与的自身免疫性疾病,与感染、免疫因素、肝脾及遗传等因素有关;免疫因素为发病的主要原因,多数学者认为,其发病与体液免疫有关,机体对血小板相关抗原发生免疫反应,产生血小板抗体,抗体致敏的血小板由单核巨噬系统迅速清除导致持续性血小板下降而发病。近年来研究发现,     

丙种球蛋白治疗免疫性血小板减少性紫瘢9例

我们静脉应用大剂量丙种球蛋白(IVIG)治疗9例免疫性血小板减少性紫瘢(ITP)患者,结果报告如下.1 资料与方法1.1 病例选择 9例ITP患者符合首届中华血液学学会全国血栓与止血学术会议制定的诊断标准.男1例,女8例.中位年龄34.7(16～58)岁.初治2例,复治7例.复治病人都曾接受过正规激素治疗,加用免疫抑制剂治疗2例,脾切除术后复发1例.病程中位时间165天(1.5～16年).临床上都有明显的出血症状,多为皮肤;粘膜出血点及瘀斑,有口腔粘膜血泡4例,月经过多3例,血尿1例.治疗前血小板计数11.8±3.22×10~9/L(8～18×10~9/L)、P_AIgG135.9±74.4 ng/10~7plt(56～32Ong/10~7 plt).1.2 治疗方法 5例患者单用HD-IVIG,余4例     

Clinical effect of intravenous immunoglobulin on chronic idiopathic thrombocytopenic purpura

Summary High dose immunoglobulin infusions showed a marked effect on platelet counts in eight out of nine chronic ITP patients and in one SLE patient. In the comparison of different IgG-preparations, the pepsin treated IgG F (ab′)₂ showed no platelet elevation while the sulfonated did. The elevated platelet count could not be maintained after discontinuation of IgG infusions, but in six out of ten patients the platelet level remained above the pretreatment values. This new treatment seems to be safe and effective in adulthood ITP.     

Recurrence of thrombocytopenia in patients splenectomized for idiopathic thrombocytopenic purpura

Summary The effects of splenectomy in the treatment of chronic idiopathic thrombocytopenic purpura (ITP) were analysed in 177 subjects (118 adults and 59 children) operated upon between two and 25 years earlier.In 145 patients, thrombocytopenia regressed immediately or within 2 months after the operation. Regression of thrombocytopenia later during follow up was observed in a further 14 cases.Recurrence of thrombocytopenia after good immediate result of splenectomy developed in 32 patients. In 13 cases the recurrence was transient but in the remaining 19 patients it was long-lasting. In these cases presence of accessory spleens was suggested by the absence of Howell-Jolly bodies in the peripheral blood smear and demonstrated by splenic scan in 16 cases, and confirmed in 13 out of 15 splenectomized patients. Resplenectomy caused in 12 patients permanent disappearance of thrombocytopenia.     

Myelofibrosis and idiopathic thrombocytopenic purpura

 A case of idiopathic myelofibrosis (IMF) with concomitant autoimmune thrombocytopenic purpura (AITP) is reported. The literature on platelet antibodies in IMF is reviewed. Received: 25 March 1997 / Accepted: 11 July 1997     

Red cell sequestration during high dose intravenous immunoglobulin in idiopathic thrombocytopenic purpura

Summary The cellular interactions involved in the platelet response after immunoglobulin infusion in acute and chronic idiopathic thrombocytopenic purpura (ITP) are unknown. There have been a number of theories including the competitive inhibition of platelet-binding to macrophages by the preferential sequestration of immunoglobulin coated red cells. We report a study to examine this hypothesis. Adult acute and chronic patients were given infusions of immunoglobulin at a rate of 0.4 g/kg body weight, daily for 5 days. Serum haptoglobin, lactate dehydrogenase and the absolute reticulocyte counts were monitored and no significant change in any value was seen during the period of study. A red cell survival was performed on four of the patients and no increase in the rate of red cell clearance occurred during the infusion period. We conclude from this that in these patients no significant degree of haemolysis was provoked by the infusion although this does not preclude this as a mechanism of action in some individuals.     

High-dose dexamethasone as a first- and second-line treatment of idiopathic thrombocytopenic purpura in adults

The current first-line choice of treatment of idiopathic thrombocytopenic purpura (ITP) in adults, prednisone, is effective but has many side effects. Furthermore, reduction of the dose leads to a relapse of ITP in a majority of cases. Courses of high-dose dexamethasone (HD) aim to avoid these problems. We treated 36 patients with newly diagnosed or recurrent ITP with an 8-day course of HD, with a peak dose of 40 mg/day. The courses were repeated up to a maximum of six courses, with a 28-day interval. Acute and chronic effects of HD on platelet counts were observed, as well as side effects. HD led to an acute response (rise of platelet count to a level above 50×10⁹/l) in 83%. When HD was given as a first-line treatment, 59% of patients were still in remission after 31 months. When HD was given as a second-line treatment, 50% of patients were in remission after 5 months, declining to 25% after 54 months. Side effects were frequent but rarely dangerous. In conclusion, acute effects of HD were excellent. Long-term effects of HD as a first-line therapy of ITP were good, but its long-term effects as a second-line therapy were much poorer.     

特发性血小板减少性紫癜患者B淋巴细胞凋亡研究

目的探讨B淋巴细胞在特发性血小板减少性紫癜(ITP)发病机制中的作用。方法用流式细胞术检测ITP患者外周血B淋巴细胞表面Fas蛋白、FasL蛋白以及胞浆内抗凋亡蛋白bcl2的表达水平。结果ITP患者外周血中B淋巴细胞表面Fas和FasL蛋白表达与对照组相比无统计学意义,B淋巴细胞胞浆内bcl2的表达水平高于正常人(P<0.05)。结论B淋巴细胞凋亡受抑可能参与ITP自身免疫的发病机制。     

Platelet recovery in patients with idiopathic thrombocytopenic purpura after eradication of Helicobacter pylori

The relationship between Helicobacter pylori infection and idiopathic thrombocytopenic purpura (ITP) has been investigated in several studies. We investigated the prevalence of H. pylori infection and the clinical effects of eradication in 22 Japanese patients with chronic ITP. H. pylori infection was found in 14 (63.6%) of the patients by histologic and culture examinations of biopsy samples obtained by gastrointestinal endoscopy. H. pylori was eradicated by proton pump inhibitors and 2 kinds of antibiotics in 13 (92.9%) of the 14 patients in whom the results of treatment could be evaluated. Five (38.4%) of those 13 patients had platelet recovery (platelet count of more than 100 x 10(9)/L and an increase of more than 30 x 10(9)/L with respect to the baseline value) after eradication. The median follow-up period was 15 months. One patient who had a complete response had a partial relapse after cessation of prednisolone treatment without any evidence of H. pylori reinfection. Another patient, in whom H. pylori was not eradicated even after 2 treatment sessions, had a partial response after treatment. A screening examination for H. pylori infection may be necessary for Japanese patients with newly diagnosed ITP. Although the exact mechanism underlying platelet recovery after H. pylori eradication is not clear, the results of this study indicated that H. pylori eradication treatment is a good option for some patients with chronic ITP.     

幽门螺杆菌与特发性血小板减少性紫癜发病关系的研究进展

幽门螺杆菌(Hp)感染与特发性血小板减少性紫癜(ITP)的发病有关。约50%Hp阳性ITP患者根除Hp后血小板减少症得以完全或部分缓解。目前Hp相关ITP的发病机制尚不完全清楚,此文就其发病机制研究进展作一综述。     

糖皮质激素治疗慢性特发性血小板减少性紫癜疗效相关因素分析

目的：探讨影响糖皮质激素治疗慢性特发性血小板减少性紫癜疗效的有关因素。方法：采用Logistic回归对74例经糖皮质激素治疗的慢性特发性血小板减少性紫癜患者性别、年龄、是否并发除皮肤出血以外较严重的出血表现、起病至开始治疗时间、是否有脾脏肿大、是否并发肝炎病毒感染、治疗前血小板计数、骨髓巨核细胞数8项因素进行多因素分析。结果：起病至开始治疗时间，是否并发肝炎病毒感染，骨髓巨核细胞数是影响糖皮质激素治疗慢性特发性血小板减少性紫癜疗效的相关因素。结论：分析患者治疗前各项可能影响疗效的相关因素，对于指导慢性特发性血小板减少性紫癜治疗方案的选择，尤其是合理掌握糖皮质激素的使用时间有重要意义。     

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.