成人慢性难治性特发性血小板减少性紫癜的治疗进展

特发性血小板减少性紫癜 (ＩＴＰ)是指因免疫异常所致的获得性血小板减少症 ,为临床最常见的出血性疾病。糖皮质激素 (泼尼松 )被公认为ＩＴＰ的首选治疗药物 ,约有 1 / 3患者可获长期反应 ;而脾切除则是对泼尼松治疗失败患者的第一选择 ,其持续完全反应和部分反应率分别为 60 %和 1 2 % 〔1〕。目前对于应用泼尼松和脾切除治疗无效的ＩＴＰ患者的治疗相当困难 ,且这种情况主要发生在成人。但近年来关于难治性ＩＴＰ的治疗已取得许多进展 ,现综述如下。1　慢性难治性ＩＴＰ的诊断迄今关于慢性难治性ＩＴＰ尚无统一的诊断标准 ,综合有关资料…     

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。     

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。     

糖皮质激素治疗特发性血小板减少性紫癜并发重症肺炎和肺结核死亡1例

特发性血小板减少性紫癜 (ITP)系临床常见出血性疾病 ,主要死亡原因多为出血 ,特别是颅内出血。本例因糖皮质激素治疗引起空洞型肺结核、重症肺炎死亡 ,实属罕见 ,有必要总结和吸取教训。患者男性 ,2 6岁。胸、腹部及四肢出血点 8d ,口腔血泡 6d于 2 0 0 3年 3月 2 0日入院。有油漆接触史 ,否认结核病史。体检 :T 36 .6℃ ,BP 12 0 / 80mmHg。无贫血貌 ,口腔右颊黏膜一蚕豆大血泡 ,心率 74次 /min ,律齐 ,肺 (一 ) ,肝脾无肿大 ,胸腹部及四肢散在出血点。血常规 :Hb14 3g/L ,WBC5 .8×10 9/L、N 0 .5 9、L 0 .4 1,PLT 3× 10 9/L。…     

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,     

皮质激素治疗特发性血小板减少性紫癜的再评价

特发性血小板减少性紫癜（ITP）是一种自身免疫性的血小板减少综合征，约占出血性疾病的30％，是临床最常见的出血性疾病。流行病学资料显示该病在30～60岁年龄组中表现为女性发病率高于男性，而在其他年龄组中则无明显性别差异，     

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     

糖皮质激素受体与特发性血小板减少性紫癜患者激素抵抗的相关性

目的　探讨特发性血小板减少性紫癜(ITP)患者外周血单个核细胞(PBMC)内两种糖皮质激素受体(GR)α和β表达水平与糖皮质激素治疗效应之间的关系。方法　采用半定量RT PCR检测不同激素效应ITP患者和正常对照组GRα、βmRNA,采用免疫细胞化学方法检测GRα、β蛋白的表达。结果　(1)ITP激素敏感组、激素抵抗组及正常人体内均有GRα、βmRNA的表达,但以GRα为主, 其GRα/葡萄糖3磷酸脱氢酶(GAPDH)mRNA分别为1 15 ±0 75, 1 63±0 78, 1 27±0 51;(2)激素抵抗组GRβmRNA表达明显高于激素敏感组及正常对照组(P<0 05 ),其GRβ/GAPDHmRNA分别为1 42±0 73, 0 82±0 59, 0 80±0 68; (3)免疫细胞化学结果显示,激素抵抗组GRβ阳性细胞数明显高于激素敏感组及正常对照组(P<0 01 ),分别为28 8±9 9, 5 9±3 2, 5 5±6 8。结论　ITP患者糖皮质激素抵抗可能与GRβ的表达亢进有关。     

Danazol for the treatment of idiopathic thrombocytopenic purpura

Fourteen patients with idiopathic thrombocytopenic purpura (ITP) refractory to steroids and/or splenectomy were treated with danazol (200 mg 3 times a day) for 2 months. The following responses were achieved: excellent (platelet count greater than 100 X 10(9)/l) in 5 patients; good (greater than 50 X 10(9)/l, but less than 100 X 10(9)/l) in 2 patients, and poor in (no increase of platelet count) 7 patients. In three cases remission lasted more than 7 months. Danazol was well tolerated and in most patients better suited than steroids for long-term intake.     

Eltrombopag for the treatment of idiopathic thrombocytopenic purpura

Strategies aimed at stimulating platelet production are a rational approach to the treatment of patients with primary immune thrombocytopenia, as, for many of them, the low platelet count is a consequence of ineffective megakaryopoiesis. Recently, intense clinical trial activity in immune thrombocytopenia has been reported for second-generation thrombopoietic agents. These novel molecules bear no structural resemblance to thrombopoietin, but still bind and activate the thrombopoietin receptor. One of these agents is eltrombopag (formerly SB497115), an orally available, small organic compound. Randomized trials have shown the short-term efficacy of eltrombopag in elevating the platelet count of most adult patients with immune thrombocytopenia unresponsive to at least one standard treatment. No significant adverse events were observed, but long-term safety data are still lacking. Ongoing studies will reveal the potential of this agent in the management of immune thrombocytopenia for long-term maintenance therapy, as well as its relative benefit compared with standard-of-care treatment.     

�ط���ѪС����������Ĺ淶������

特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)是一种获得性自身免疫性疾病,是由于人体内产生抗血小板自身抗体导致网状内皮系统破坏血小板过多从而造成血小板减少[1].该病的年发病率为(50～100)/106,其中半数以上是儿童.     

Rituximab treatment for chronic refractory idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) is characterized by mucocutaneous bleeding and a low platelet count caused by increased autoantibodies against self-antigens and T-cell mediated cytotoxicity. About 10-30% patients with ITP will become refractory ITP. Most of them will become refractory to corticosteroids and splenectomy, as well as other available agents such as intravenous immunoglobulins, danazol, or chemotherapy. B cells not only are the passive producers of immunoglobulins, but also play an important immunoregulatory role in pathophysiology of ITP. Rituximab, a chimeric anti-CD20 monoclonal antibody that specifically targets the CD20 molecule on the B-cell surface, is useful in the treatment of ITP through B cells depletion. Rituximab has multiple mechanisms of inducing cytotoxicity in vivo, including antibody-dependent cell-mediated cytotoxicity (ADCC), complement-dependent cytotoxicity (CDC), direct apoptosis signaling, and possible vaccine effects. In most clinical reports, rituximab was given as an intravenous infusion at a dose of 375 mg/m(2) weekly for four doses. A total complete response (CR) of 33.2% and a total response of 52.9% were reported. Most results found that no clinical or laboratory parameters could predict treatment outcome. Though the infusion-related side effects of rituximab were common in ITP, it was well tolerated with rare severe side effects. In general, rituximab appears to be a promising immunotherapeutic agent for the treatment of refractory ITP. More controlled clinical trials are necessary to evaluate both the efficacy and long-term safety of the drug.     

特发性血小板减少性紫癜细胞免疫机制研究进展

特发性血小板减少性紫癜（ITP）是由于机体免疫系统功能紊乱引起的以血小板破坏增加、生成减少为特征的慢性获得性器官特异性的自身免疫性出血性疾病。其发病机制复杂，传统观点认为ITP主要是由于体液免疫异常导致抗体介导的血小板破坏增多，     

Rituximab in the treatment of relapsing idiopathic thrombocytopenic purpura

About 25 to 30% of patients with idiopathic thrombocytopenic purpura (ITP) are resistant to standard treatment with steroids and splenectomy. In these patients with chronic refractory ITP, there is no proven algorithm for standard care. Recently, the chimeric anti-CD20 monoclonal antibody rituximab was considered as an alternative treatment option in this patient group. We present a patient with frequently relapsing ITP after treatment with prednisone, splenectomy and high-dose dexamethasone. Since he experienced increasing side effects due to the steroids, he was treated with rituximab 375 mg/m2, once weekly for four weeks, resulting in a complete long-lasting response (follow-up seven months).     

利妥昔单抗治疗糖皮质激素无效的特发性血小板减少性紫癜疗效观察

目的 探讨利妥昔单抗治疗特发性血小板减少性紫癜(ITP)的疗效、安全性及治疗前后B细胞、血小板膜糖蛋白(GP)特异性自身抗体的变化.方法 利妥昔单抗(375 mg/m2,每周1次,连用4周)治疗12例糖皮质激素治疗无效的ITP患者,监测治疗前后的血常规、血清免疫球蛋白定量(IgG、IgM、IgA)、血小板GPⅡb/Ⅲa和(或)GP Ⅰ b/Ⅸ特异性自身抗体、CD+3、CD+4、CD+8、CD+19、CD+20细胞数.结果 4例完全有效,3例部分有效,2例微效,3例无效.随访中位时间5(0.5～12)个月,疗效均维持较好.有效患者治疗后血小板自身抗体均转阴.治疗前后血清IgG、IgM、IgA无明显变化,CD+3、CD+4、CD+8细胞数无明显变化.治疗后CD+19/CD+20细胞数(4.1±2.2)×106/L与治疗前(295.0±86.4)×106/L比较明显下降(P＜0.01).无严重不良反应.结论 利妥昔单抗治疗糖皮质激素无效的ITP患者安全、有效.