特发性血小板减少性紫癜患者抗心磷脂抗体的检测

目的了解抗心磷脂抗体(ACA)与特发性血小板减少性癜(ITP)的关系。方法采用 ELISA 法检测 ACA-IgG,ACA-IgM。结果 30例 ITP 患者 ACA-IgG 阳性率为70%(21例),其中高滴度(index value≥2.2)50%(15 例)；IgM 阳性率为56.7%(17例),高滴度20%(6例)。20例健康对照组 ACA-IgG 阳性率5%(1例),IgM 阳性率5% (1例)。结论 ITP 患者 ACA-IgG 和 ACA-IgM,尤其是高滴度 ACA-IgG 对 ITP 的诊断有一定临床意义。     

墓头回治疗特发性血小板减少性紫癜的疗效与机制

目的 研究中药墓头回治疗特发性血小板减少性紫癜(ITP)的临床疗效.方法 观察墓头回治疗后ITP患者的症状改善情况,常规计数血小板数目的 变化;用流式细胞仪检测治疗前、后抗血小板抗体G(PAIgG)的变化.结果 墓头回治疗后ITP患者的治愈率为84.62%,治疗后PAIgG表达降低,血小板数目增加.结论 墓头回对ITP患者具有一定的临床治疗作用.     

不同方案治疗小儿特发性血小板减少性紫癜的疗效观察

目的　比较不同方案治疗小儿特发性血小板减少性紫癜 (ITP)的疗效。方法　将ITP患者分为四组 :Ⅰ组 (丙球 )、Ⅱ组 (丙球 +地塞米松 )、Ⅲ组 (地塞米松 )、Ⅳ组 (强的松 )。结果　Ⅰ～Ⅱ组止血时间、血小板上升至正常时间较Ⅳ组明显缩短 (P <0 .0 1) ,Ⅰ、Ⅱ组血小板上升峰值、总有效率明显高于Ⅳ组 (P <0 .0 1)。Ⅲ组血小板上升至正常所需时间明显长于Ⅰ、Ⅱ组 ,其上升峰值亦显著低于Ⅰ、Ⅱ组 ,但其止血时间、有效率与Ⅰ、Ⅱ组无显著差异 ,且明显优于Ⅳ组。结论　对于小儿特发性ITP尤其是血小板过低 (<2 0× 10 9/L)易致危及生命的出血 ,首选静滴丙球与地塞米松联合治疗。而对于血小板较低但全身症状不严重且经济困难的患者 ,大剂量地塞米松冲击疗法不失为一种有效、经济的治疗方法。     

Multiple in silico tools predict phenotypic manifestations in congenital thrombotic thrombocytopenic purpura

Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare, recessively inherited genetic disorder with varying clinical presentation that is caused by ADAMTS13 mutations. Several studies have found limited associations between ADAMTS13 mutations and cTTP phenotype. The use of in silico tools that examine multiple mutation characteristics may better predict phenotype. We analysed 118 ADAMTS13 mutations found in 144 cTTP patients reported in the literature and examined associations of several mutation characteristics, including N‐terminal proximity, the evolutionary conservation of the affected amino acid position, as well as amino acid charge/phosphorylation and genetic codon usage to disease phenotype. Structure‐altering mutations were examined for their impact on ADAMTS13 function based on existing ADAMTS13 crystallographic data (AA 77‐685). Our in silico data indicate that: (i) The position of the mutation in the N‐ or C‐terminus, (ii) evolutionary conservation and (iii) codon usage of the affected mutation position are associated with disease parameters, such as age of onset, organ damage and fresh frozen plasma prophylaxis. In conclusion, the usage of multiple in silico tools presents a promising strategy in refining predictions for the diverse presentation of cTTP. Enhancing our utilization of in silico tools to find genotype‐phenotype associations will create better‐tailored approaches for individual patient treatment.     

30例血栓性血小板减少性紫癜临床分析

目的 探讨血栓性血小板减少性紫癜（thrombotic thrombocytopenic purpura,TTP）患者的临床特征、治疗方案及预后,以提高TTP诊疗水平。方法 回顾性分析2012年4月至2018年12月住院的30例TTP患者的临床表现、实验室检查、治疗及预后。结果 30例TTP患者中男性14例,女性16例,平均年龄（51.33±14.88）岁,三联征17例（57%）,五联征11例（37%）;加重2例,复发2例,难治1例,死亡16例（53%）;24例（80%）患者行血浆置换,血浆置换组与非血浆置换组之间病死率差异有统计学意义（P<0.05）;血浆置换次数≤ 4次与血浆置换次数>4次者病死率差异无统计学意义（P>0.05）;血浆置换前后血红蛋白、血小板、乳酸脱氢酶值,差异有统计学意义（P<0.05）。结论 TTP诊断需要依据临床表现及实验室检查,血浆置换可显著降低TTP患者病死率,对于明确诊断后的患者应尽早应用。     

Splenectomy versus medical treatment for idiopathic thrombocytopenic purpura

BACKGROUND: Treatment strategies for idiopathic thrombocytopenic purpura (ITP) are still uncertain and its management is primarily empirical. The aim of this study was to investigate the role of splenectomy in the therapy of ITP and to evaluate whether medical or surgical treatment is superior. METHODS: Ninety-two patients with ITP were included in the study. All of these patients had medical therapy and 38 of them underwent splenectomy subsequently. Follow-up was completed in 91 patients after a median of 64 months. RESULTS: Side effects of medical therapy were noticed in 32 patients (35%), whereas after surgery only 2 patients (5%) had minor complications. A complete or partial remission was achieved in 35 patients (92%) after splenectomy, whereas this was achieved in only 27 patients (30%) after medical therapy. On multivariate analysis splenectomy and age were the only significant independent factors for complete and partial remission. CONCLUSIONS: Splenectomy is highly effective and safe in the treatment of ITP and is superior over medical therapy. These results should stimulate the discussion about splenectomy for ITP, possibly establishing evidence-based guidelines for surgical treatment in hematology.     

糖皮质激素诱导特发性血小板减少性紫癜患儿外周血淋巴细胞凋亡的体外研究

为探讨糖皮质激素对儿童急性特发性血小板减少性紫癜(AITP)外周血淋巴细胞凋亡的诱导作用,对6例初诊儿童AITP外周血淋巴细胞进行糖皮质激素、糖皮质激素受体拮抗剂(RU486)等体外干预培养,用流式细胞术检测细胞凋亡,以凋亡率为定量指标,用DNA电泳对细胞凋亡进行定性分析.结果显示糖皮质激素组的淋巴细胞凋亡率明显高于对照组和经RU486预处理组,具有显著性差异;糖皮质激素组细胞的DNA电泳呈现清晰的"梯状"(1adder)条带,而对照组和RU486预处理组均未出现,提示糖皮质激素可诱导淋巴细胞凋亡,这一作用可被其受体拮抗剂阻断,说明糖皮质激素诱导AITP外周血淋巴细胞凋亡可能是通过糖皮质激素受体途径来实现的.     

血管性假性血友病因子裂解酶活性检测方法的建立及其临床应用

目的 建立一种检测血管性假性血友病因子裂解酶(vWF-cp)活性的方法,并探讨其临床应用.方法 用Sepharose CL-4B柱层析法从正常人新鲜冰冻血浆中分离出血管性假性血友病因子(vWF)作为酶反应底物.然后联合应用十二烷基硫酸钠-聚丙烯酰胺凝胶电泳、免疫印记与发光绘图法(SWL法),分别检测60名健康成人、28名脑梗塞和7名血栓性血小板减少性紫癜(TTP)患者的血浆vWF-cp活性,并比较各组的检测结果.结果 该法准确检测了60名健康成人和35名患者的血浆vWF-cp活性,批内和批间变异为4.81%和8.63%.60名健康成人vWF-cp活性为(86.53±17.49)%;脑梗塞vWF-cp活性为(77.15±16.72)%,其活性低于健康成人(P＜0.05);7例TTP,血浆置换前vWF-cp活性为(9.06±7.17)%,其活性显著低于健康成人(P＜0.01);血浆置换24 h后vWF-cp活性为(47.00±6.27)%,其活性有所升高,但仍低于健康成人(P＜0.01);恢复期vWF-cp活性为(83.18±8.83)%,其活性接近健康成人(P＞0.05).结论 SWL法可准确检测健康成人和疾病患者的血浆vWF-cp活性,其敏感性、特异性和重复性较好,可应用于临床检验.与健康成人相比,脑梗塞患者vWF-cp活性降低;TTP患者vWF-cp活性显著降低,血浆置换能有效提高TTP患者vWF-cp活性水平.     

特发性血小板减少性紫癜的研究进展

特发性血小板减少性紫癜属祖国医学的"血证"、"发斑"等范畴,本文从中医角度出发,结合近10年相关文献资料,就其病因病机,辨证分型,治法,治疗进行了归纳,并就中西医结合治疗本病现状及主要用法进行了探讨。从而提出中医药治疗本病的特色和中西医结合治疗本病的优势,并针对不足之处作出了剖析和展望。