原发性血小板减少紫癜(Idiopathic thrombocytopenic purpura,ITP)的治疗

治疗原则对急性、重型患者,目的是防止致命性出血;对慢性或已缓解者,是促进病情缓解或维持缓解;联合用药能提高疗效。治疗方案推荐方案1~61、肾上腺皮质激素适用血小板计数明显减少,有较严重皮肤、粘膜出血,有颅内出血倾向者;脾切除前作减少手术出血的措施。用法:强的松40~60mg/日、严重出血者60~100mg/日,疗程2~4周,缓解后渐减量维持治疗。急性出血者可用氢化考的松200mg/iv qd或氟美松10~20mg/iv qd,连用3~5日,缓解后改口服强的松维持。

Acquired amegakaryocytic thrombocytopenia previously diagnosed as idiopathic thrombocytopenic purpura in a patient with hepatitis C virus infection

We report the first case of a patient with hepatitis C virus(HCV) infection and idiopathic thrombocytopenic purpura(ITP), who later developed acquired amegakaryocytic thrombocytopenia(AAMT), with autoantibodies to the thrombopoietin(TPO) receptor(c-Mpl). A 64-year-old woman, with chronic hepatitis C, developed severe thrombocytopenia and was diagnosed with ITP. She died of liver failure. Autopsy revealed cirrhosis and liver carcinoma. In the bone marrow, a marked reduction in the number of megakaryocytes was observed, while other cell lineages were preserved. Therefore, she was diagnosed with AAMT. Additionally, autoantibodies to c-Mpl were detected in her serum. Autoantibodies to c-Mpl are one of the causes of AAMT, acting through inhibition of TPO function, megakaryocytic maturation, and platelet formation. HCV infection induces several autoantibodies. HCV infection might also induce autoantibodies to c-Mpl, resulting in the development of AAMT. This mechanism may be one of the causes of thrombocytopenia in patients with HCV infection.

The Effect of Costimulatory Factors in the Pathogenesis of Chronic Idiopathic Thrombocytopenic Purpura

To investigate the effect of costimulatory factors in the pathogenesis of chronic idiopathic thrombocytopenic purpura(CITP), we examined the expression of CD80 on platelets and megakaryocytes in patients with CITP and the controls by FACS. By using CD80 monoclonal antibody (McAb) to inhibit interaction among cells which is mediated by costimulatory factors, we observed the effect of CD80 McAb on the growth and maturation of megakaryocytic progenitors of patients with CITP in vitro . The results showed the expression of CD80 on platelets and megakaryocytes in CITP group was significantly higher than that in controls ( P <0.01). There was a significantly positive correlation between the expression of CD80 on platelets and serum PAIgG in CITP (r=0.86, P <0.05). The mean of various clone numbers (CFU MK, BFU MK and mCFU MK) in CITP were all lower than those in controls ( P <0.05). In megakaryocytes co cultured with CD80 McAb, there was an increasing tendency of the number of CFU MK and big CFU MK (the number of megakaryocyte with GPⅢ a positive was more than 20) and mediate CFU MK (the number of megakaryocyte with GPⅢ a positive was 11-20). When the concentration of CD80 McAb was 10 μg/L, there was a significant difference in the number of megakaryocytic colony formation (CFU MK, BFU MK and mCFU MK) between the group with CD80 McAb and that without it ( P <0.05).These showed the abnormality of costimulatory factors had important effect in the pathogenesis of CITP.

Clinical Study on the Effect of Shengxueling (生血灵) on Idiopathic Thrombocytopenic Purpura

Objective:To observe the clinical effect of Shengxueling (生血灵, SXL) on idiopathic thrombocytopenic purpura (ITP), and study the possible mechanism.Methods: Eighty-six cases of ITP were randomly divided into two groups. The SXL group, 56 patients treated with SXL, a traditinal Chinese medicine and 30 patients administered with prednisone were taken as control. Each group took drugs for 3 months and was under follow-up observation.Results: In the SXL group, the total effective rate was 85.71%, similar to prednisone 83.33%( P >0.05) for 3 months, but the total effective rate of SXL (91.07%) were obviously better than that of the control group (53.33%) ( P <0.01) for 6 months and had no obvious adverse reaction. The patients bleeding was alleviated or stopped, the general condition was improved. At the same time, blood platelet count (PLT) was increased, platelet associated immunoglobulin (PAIg) and interleukin-4 (IL-4) were markedly dropped, the level of natural killers cells activity (NKa) increased, the rate of T lymphocyte subsets gradually returned to normal level. Megakaryocyte tended to maturation on bone marrow smear after treatment. All differences above were statistically significant.Conclusion: SXL is an effective and safe medicine for ITP. Its mechanism could regulate cytoimmune, inhibit platelet antibody to reduce the destruction of platelet, increase the number of platelet, promote the division and maturation of megakaryocyte, facilitate the production and release of platelet, lower the fragility of capillary, prevent and cure hemorrhagic tendency.

Association of primary biliary cirrhosis with idiopathic thrombocytopenic purpura

Although both primary biliary cirrhosis (PBC) and idiopathic thrombocytopenic purpura (ITP) are autoimmune diseases, the association of the 2 diseases is rare. Here, we report a case of ITP that developed during the follow-up of PBC in a 74-year- old man. The patient had been diagnosed with PBC 12 years previously, and had received treatment with ursodeoxycholic acid. The platelet count decreased from approximately 60 × 109/L to 8 × 109/L, and the association of decompensated liver cirrhosis (PBC) with ITP was diagnosed. Steroid and immune gamma globulin therapy were successful in increasing the platelet count. Interestingly, human leukocyte antigen genotyping detected the alleles DQB10601 and DRB10803, which are related to both PBC and ITP in Japanese patients. This case suggests common immunogenetic factors might be involved in the development of PBC and ITP.

Refractory case of ulcerative colitis with idiopathic thrombocytopenic purpura successfully treated by Janus kinase inhibitor tofacitinib:A case report

BACKGROUND Concomitant ulcerative colitis (UC) and idiopathic thrombocytopenic purpura(ITP) is a rare phenomenon. The management of UC with ITP can be challenging,since a decreased platelet count augments UC.CASE SUMMARY A 24-year-old man with UC and steroid-resistant ITP experienced UC flare.Although continuous infusion of cyclosporine was initiated, UC did not improve.The administration of tofacitinib subsequently led to the induction of remission.The patient has maintained remission of UC and ITP for over one year ontofacitinib treatment. Whole transcriptomic sequencing was performed forinflamed rectal mucosae obtained before and after the initiation of Janus kinase(JAK) inhibitor, suggesting that distinct molecular signatures seemed to beregulated by JAK inhibitors and other conventional therapies including tumornecrosis factor lockers.CONCLUSION Tofacitinib should be considered in refractory cases of UC with ITP.

Direct Infection of Colony Forming Unit-Megakaryocyte by Human Cytomegalovirus Contributes the Pathogenesis of Idiopathic Thrombocytopenic Purpura

Human cytomegalovirus （HCMV） late mRNA expression in megakaryoblast and in turn the pathogenesis of idiopathic thrombocytopenic purpura （ITP） patients with HCMV infection, and effectiveness of ganciclovir were investigated. Colony forming unit-megakaryocytes （CFU-MK） of 46 ITP patients with HCMV infection were incubated from patients＇ bone marrow mononuclear cells （MNC）. Reverse transcriptase-polymerase chain reaction （RT-PCR） was subsequently used for CFU-MK for HCMV-late mRNA detection, Ganciclovir therapy was given to both HCMV-late mRNA positive and negative groups for comparison of therapeutic effectiveness, The results in 19 of 46 CFU-MK culture cells specimens with positive HCMV-DNA by PCR or positive CMV-IgM by enzyme linked immunosorbent assay （ELISA） in the correspondent serum of peripheral blood were positive for HCMV-late mRNA, Sixteen out of 19 patients with positive HCMV-late mRNA CFU-MK had a positive response to ganciclovir. Amongst 27 patients with negative HCMV-late mRNA CFU-MK, only 4 positive responders to ganciclovir therapy were observed. Curative effectiveness of ganciclovir in HCMV-late mRNA positive group was significantly higher than that in HCMV-late mRNA negative group （P〈0.01）, It was suggested that HCMV could directly infect CFU-MK, which might be one of the mechanisms responsible for HCMV related ITE The ganci- clovir is an effective therapy in resulting in the increases in thrombocyte in the ITP patients whose HCMV- late mRNA was positive in their CFU-MK.

Therapeutic Uses of Lycium barbarum Polysaccharides on Idiopathic Thrombocytopenic Purpura Mice via Inhibition of Oxidative Stress

The aim of the present study was to investigate the effect of Lycium barbarum polysaccharides (LBP) on the treatment of ITP mice and to explore its mechanism. Forty idiopathic thrombocytopenic purpura (ITP) mice were divided randomly into a model control group and LBP groups I, II, III and IV. ITP mice in LBP groups I, II, III, and IV were administered LBP at four different doses (50, 100, 200 and 400 mg·kg-1·d-1, respectively) for 7 days by gavage. Blood samples were collected from the tail veins of the mice after treatment. Platelet counts were determined, and the total antioxidant status (TAS), total oxidant status (TOS) were measured with ELISA kits. The platelet count was (30.28 ± 13.42) × 109/L in the model control group, and the number of platelets in all LBP groups was higher than that in the model control group. The platelet count increased, and it reached (67.09 ± 10.81) × 109/L in LBP group I;the platelet counts in the other three groups increased significantly compared to LBP group I, and they did not differ significantly. TAS concentrations in the LBP groups were significantly increased compared to the model control group, whereas TOS concentrations were significantly decreased. Taken together, these results indicate that LBP is effective at increasing the number of platelet (PLT), and LBP may treat ITP mice via suppressing oxidative stress.

Lymphocytosis in Idiopathic Thrombocytopenic Purpura Patients Infected by <i>Helicobacter pylori</i>

Background and Objectives: Several recent reports have demonstrated a close linkage between idiopathic thrombocytopenic purpura (ITP) and Helicobacter pylori (H. pylori) infection in some patient’s populations. However, the pathogenetic mechanisms of H. pylori-induced thrombocytopenia remain obscure. Therefore, we investigated the prevalence of H. pylori infection pylori and performed a comparative analysis of a subset of H. pylori-infected patients (group A) with non-infected patients (group B) using the standard statistical methods. Design and Methods: From December 2001 to October 2002, we investigated the presence of gastric H. pylori infection in 30 adult ITP patients and 19 patients were treated with standard antibiotic therapy for H. pylori eradication (amoxicillin and clarithromycin plus lansoprazole combination). We used the standard statisticsto analyze the difference between group A and group B. Results: H. pylori eradication was achieved in 17/19 (89.4%) H.pylori-infected patients. An improvement of platelet count was observed in 14/19 patients (73.6%) who achieved the eradication. Five of these patients achieved CR (two patients were with the acute ITP) and nine patients reached PR. The difference between the mean platelet count ± S.D. before and after H. pylori therapy was statistically significant in patients with successful decontamination (65 ± 48 × 109/L vs. 200 ± 140 × 109/L;p = 0.018). Lymphocyte counts at the diagnosis of H. pylori infected cases were significant higher than those of non-infected cases (1.58 ± 0.13 ×

Current Concept for the Diagnosis and Treatment of Adult Idiopathic Thrombocytopenic Purpura

Idiopathic thrombocytopenic purpura (ITP) is a common hematologic disorder manifested by immunemediated thrombocytopenia. The estimated incidence ranges from 50 ～ 100 per million each year in western countries, roughly divided between adults and children.

Helicobacter pylori infection in patients with autoimmune thrombocytopenic purpura

AIM:To compare the prevalence of Helicobacter pylori (Hpylon)infection in autoimmune thrombocytopenic purpura (AITP)patients with that of nonthrombocytopenic controls, and to evaluate the efficacy of the treatment in H pylori(+) and H pylor(-)AITP patients. METHODS:The prevalence of gastric H pylori infection in 38 adult AITP patients(29 female and 9 male;median age 27 years;range 18-39 years)who consecutively admitted to our clinic was investagated. RESULTS:H pylori infection was found in 26 of 38 AITP patients(68.5%).H pylori infection was found in 15 of 23 control subjects(65.2%).The difference in H pylori infection between the 2 groups was not significant.Thrombocyte count of H pylori-positive AITP patients was significantly lower than that of H pylori-negative AITP patients(P<0.05). Thrombocyte recovery of H pylori-positive group was less than that of H pylori-negative group(P<0.05). CONCLUSION:H pylori infection should be considerecd in the treatment of AITP patients with H pylori infection.

Chronic Immune Thrombocytopenic Purpura in a Young Female with Rheumatoid Arthritis (Unusual Course)

We present a case of a 29-year-old female from Sudan, who was diagnosed with rheumatoid arthritis (RA) in 2005 and with immune thrombocytopenic purpura (ITP) in 2009. The ITP immediately followed using, for four weeks, a combination of medications that included rifampicin. The platelets count continued to be low thereafter. During the year following her diagnosis with ITP, she reported gradual improvement in her joints symptoms, which continued during her pregnancy in 2011. Following puerperium, her chronic ITP resolved completely;however, her joint disease flared up few months later. To our knowledge, there are no reported cases of chronic ITP, which were drug induced at first in a patient of RA except with gold therapy. Similarly, there are no reports on cases that recovered from chronic ITP after delivery. Finally, this case highlights the impact different coexisting autoimmune diseases may have on each other regarding course and prognosis.

艾曲泊帕乙醇胺片对特发性血小板减少性紫癜患儿CD40/CD40L的影响

目的探讨艾曲泊帕乙醇胺片对特发性血小板减少性紫癜(ITP)患儿CD40/CD40L轴的影响机制。方法选取80例ITP患儿作为研究对象,根据数字随机表法将其分为对照组(n=40)和研究组(n=40)。对照组给予环孢素治疗,研究组给予艾曲泊帕乙醇胺片治疗,两组均连续治疗3个月。对比两组患者的临床疗效、T淋巴细胞亚群水平(CD3^(+)、CD4^(+)和CD4^(+)/CD8^(+))以及不良反应。检测两组患者淋巴细胞膜表面和血小板膜表面的CD40、CD40L水平,使用ELISA检测血浆sCD40和sCD40L水平。结果研究组的总有效率(92.50%)明显高于对照组的总有效率(75.00%)(P<0.05)。两组治疗后的CD3^(+)、CD4^(+)、CD4^(+)/CD8^(+)、血浆s CD40表达水平均高于治疗前,CD8^(+)以及外周血CD19^(+)CD40^(+)、CD3^(+)CD40L^(+)细胞和血浆sCD40L表达水平低于治疗前;研究组CD3^(+)、CD4^(+)、CD4^(+)/CD8^(+)、血浆sCD40表达水平高于对照组,CD8^(+)以及外周血CD19^(+)CD40^(+)、CD3^(+)CD40L^(+)细胞和血浆sCD40L表达水平低于对照组(P<0.05)。两组治疗后的外周血血小板膜表面的CD40L^(+)细胞低于治疗前;研究组的外周血血小板膜表面的CD40^(+)高于对照组,CD40L^(+)细胞低于对照组(P<0.05)。研究组的总不良率(15.00%)低于对照组的总不良率(22.50%),但是两组比较差异无统计学意义(P>0.05)。结论艾曲泊帕乙醇胺片治疗ITP患儿具有良好的临床疗效,调节T淋巴细胞亚群水平,安全性良好。

不同脾蒂离断法在腹腔镜脾切除治疗ITP中的成本-效果比较

目的比较腹腔镜下原位二级脾蒂离断法脾切除术(laparoscopic splenectomy by secondary pedicledivision,LSSP)和腹腔镜下直线切割闭合器脾切除术(laparoscopic splenectomy by Endo-GIA,LSED)治疗特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的成本-效果。方法收集本院2007年5月～2009年6月因ITP行腹腔镜下脾切除术治疗的患者42例,随机分为LSSP和LSED两组,每组21例,比较两种治疗方法的疗效差异,并进行成本-效果分析。结果 LSSP组的手术时间、术中出血量、术中住院时间分别为(152.8±61.4)min、(202.5±88.4)mL和(5.9±0.7)d,与LSED组(161.5±31.8)min、(219.7±79.6)mL和(6.1±0.4)d相比均无统计学差异(P>0.05),LSSP组治疗总有效率为81.0%,与LSED组(90.5%)相比无统计学差异(P>0.05)。而在手术费用上LSSP组为(7681.7±752.1)元,与LSED组的(10138.5±631.3)元相比差异有显著性(P<0.05)。结论 LSSP治疗ITP的治疗效果与LSED相近,但更节约医疗成本。

金薯叶止血合剂对ITP模型动物的治疗作用

目的:观察金薯叶止血合剂对特发性血小板减少性紫癜(ITP)模型小鼠外周血血小板(PLT)数、骨髓巨核细胞数和巨核细胞数分类、外周血T淋巴细胞亚群变化的影响。方法:用豚鼠抗小鼠血小板血清(APS)连续注射小鼠,造成ITP小鼠模型,分别给金薯叶止血合剂(大、中、小剂量即5g生药.kg-1.d-1、2.5g生药.kg-1.d-1、1.25g生药.kg-1.d-1),正常对照组、模型对照组给予生理盐水,连续给药10d;血细胞分析仪测定PLT,显微镜法观察巨核细胞形态分类,塑料包埋法计数小鼠股骨巨核细胞数,流式细胞仪检测T淋巴细胞亚群。结果:模型小鼠外周血PLT数持续明显减低、骨髓巨核细胞数增多,其中原幼巨核细胞比例明显增高,产板巨核细胞比例明显减低,外周血CD4+CD8-细胞减低,CD8+CD4-和CD4+CD8+细胞明显增高,CD4+/CD8+比值明显降低。给予金薯叶止血合剂后PLT明显增高、巨核细胞数减少、产板巨核细胞比例明显增高,CD8+CD4-和CD4+CD8+细胞比例明显降低,CD4+/CD8+比值升高。结论:金薯叶止血合剂能提升ITP模型小鼠PLT数,提高产板巨核细胞比例,改善T淋巴细胞的失衡,达到治疗ITP的作用。

特发性血小板减少性紫癜患儿血清LXA4、ADAMTS-1水平及其在预后评估中的价值

目的 探讨特发性血小板减少性紫癜(ITP)患儿血清脂氧素A4(LXA4)及含凝血酶敏感素1型基序的解聚素样金属蛋白酶(ADAMTS-1)水平以及二者在ITP预后评估中的价值。方法 将2020年8月至2022年8月于该院确诊为ITP的112例患儿纳入研究作为ITP组。另选取同期于该院体检的106例健康志愿者作为对照组。采用酶联免疫吸附法检测受检者血清LXA4、ADAMTS-1水平。采用Pearson相关分析患儿血清LXA4、ADAMTS-1水平的相关性。对不同病情及预后患儿的血清LXA4与ADAMTS-1水平进行比较。采用受试者工作特征(ROC)曲线分析血清LXA4与ADAMTS-1对ITP预后不良的预测价值。采用Logistic回归分析影响ITP患儿预后的因素。结果 与对照组相比,ITP组血清LXA4与ADAMTS-1的水平均升高(t=16.921、17.360,P<0.05)。Pearson相关分析显示,血清LXA4与ADAMTS-1呈正相关性(r=0.577,P<0.05)。与轻度组相比,中度组与重度组血清LXA4与ADAMTS-1表达水平较高(P<0.05);与中度组比较,重度组血清LXA4与ADAMTS-1水平较高(P<0.05)。预后不良组血清LXA4与ADAMTS-1水平均高于预后良好组(t=7.903、11.480,P<0.05);血清LXA4、ADAMTS-1单独及联合检测用于ITP预后不良预测的曲线下面积(AUC)分别为0.695、0.816、0.882,二者联合预测ITP预后的AUC大于LXA4单独预测的AUC(Z=3.363,P<0.05)和ADAMTS-1单独预测的AUC(Z=2.534,P<0.05)。Logistic回归分析显示,LXA4与ADAMTS-1是ITP预后不良的危险因素(P<0.05)。结论 ITP患儿血清LXA4与ADAMTS-1水平升高,联合检测LXA4、ADAMTS-1水平有助于评估患儿预后。

成人难治及复发ITP的个体化治疗及研究进展

免疫性血小板减少性紫癜(ITP)是血液系统的常见疾病,以出血、外周血血小板减少为主要临床特征。成人常呈慢性病程,其中11%-35%可发展为难治性ITP。调查研究显示,ITP发病与基因多态性有关。目前对于难治/复发性ITP的治疗还没有定论,从一定程度上说,其治疗依赖于患者的治疗需要和对治疗的反应。本综述的目的旨在为目前参差不齐的治疗策略提供临床参考。本文论述了难治/复发性ITP的所有治疗方案,并着重论述了新的疗法,包括抗CD20单克隆抗体、血小板生成素样物质、TPO受体激动剂和造血干细胞移植,在我国泛细胞保护剂也显示了较好的临床疗效。总之,最重要的是根据ITP患者的具体情况给予个体化治疗。

糖皮质激素联合根除幽门螺杆菌治疗ITP的疗效观察

目的探讨糖皮质激素联合根除幽门螺杆菌(HP)治疗特发性血小板减少性紫癜(ITP)的临床疗效,以及对血小板计数和T细胞亚群的影响。方法将94例HP阳性的ITP患者分为两组,每组47例。对照组给予泼尼松治疗;观察组给予泼尼松联合根除HP治疗,观察两组患者的临床疗效及1年内复发率,并检测治疗前、后血小板计数和T细胞亚群变化情况。结果观察组治疗的总有效率为89.36%,对照组为70.21%;观察组1年的复发率为36.17%,对照组为72.34%,两组比较均差异有统计学意义(P<0.05)。两组患者治疗后血小板计数均明显升高,血小板相关抗体(PAIgG)水平均明显降低,但观察组改善更明显(P<0.05)。两组患者治疗后CD3+CD4+%均明显升高,CD3+CD8+%均明显降低,但观察组改善更明显(P<0.05)。结论糖皮质激素联合根除HP治疗ITP临床疗效确切,能够提高血小板计数,改善T细胞亚群比例失调,值得临床推广。

慢性ITP外周血单个核细胞Th1/Th2亚群反应状态及生血灵干预研究

目的探讨辅助性T淋巴细胞亚群Th1、Th2在慢性特发性血小板减少性紫癜(ITP)发病中的作用,观察生血灵对慢性ITP Th1、Th2亚群功能状态的影响。方法以酶联免疫吸附法(ELISA法)检测慢性ITP患者外周血单个核细胞(PBMC)培养上清中Th1型细胞因子IL-2和Th2型细胞因子IL-10的水平。结果慢性ITP患者PBMC培养上清中IL-2和IL-10的含量均较正常对照组明显减低(P均<0.001),生血灵对其具有明显提升作用(P<0.01)。结论慢性ITP患者Th亚群免疫功能低下。生血灵治疗慢性ITP能够增强Th亚群免疫功能,恢复其平衡。

孟鲁司特钠对ITP模型小鼠T淋巴细胞亚群及晚期氧化蛋白产物的影响

目的:探讨孟鲁司特钠对特发性血小板减少性紫癜(ITP)模型小鼠T淋巴细胞亚群、细胞因子及晚期氧化蛋白产物(AOPP)的水平,分析孟鲁司特钠治疗ITP的机制。方法:将40只ITP模型小鼠随机分为对照组、模型组、孟鲁司特钠低剂量组(3 mg/kg)和孟鲁司特钠高剂量组(12 mg/kg)。成功造模后第8天开始,连续14 d给药后,计算血小板数量(PLT)、胸腺、脾脏指数,采用流式细胞仪检测外周血T淋巴细胞亚群,酶联免疫法检测血清中IL-6、TNF-α、AOPP水平。结果:与空白组小鼠比较,模型组小鼠的PLT、脾脏指数、CD8^+、IL-6、TNF-α、AOPP水平显著升高(P<0.05),CD3^+、CD4^+、CD4^+/CD8^+水平显著下降(P<0.05);与模型组比较,孟鲁司特钠低、高剂量组小鼠脾脏指数、CD8^+、IL-6、TNF-α、AOPP水平显著降低(P<0.05),CD3^+、CD4^+、CD4^+/CD8^+水平显著上调(P<0.05)。结论:孟鲁司特钠能够通过调节免疫功能紊乱,消除AOPP积累,降低IL-6、TNF-α水平,对ITP起治疗作用。