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.     

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.     

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。     

抗幽门螺杆菌治疗对慢性特发性血小板减少性紫癜患者疗效观察

目的通过对特发性血小板减少性紫癜(ITP)患者行抗幽门螺杆菌(HP)感染的临床研究,观察ITP与HP感染之间的关系以及抗HP在ITP患者治疗中的作用。方法2003-07~2003-12对解放军301医院17例慢性ITP患者采用14C尿素呼气试验进行HP检测,女11例,男6例,病程4个月至8年,并对阳性患者行奥美拉唑、克拉霉素及阿莫西林三联根除HP治疗,观察血小板情况。结果本组17例患者中HP阳性13例,阴性4例,抗HP后转阴11例,其中8例血小板水平较前有所提高。结论抗HP治疗确实可提高部分慢性ITP患者的血小板数。     

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     

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。     

High-dose dexamethasone therapy in chronic idiopathic thrombocytopenic purpura

 A high-dose pulse of dexamethasone has been described as a current option for the treatment of refractory idiopathic thrombocytopenic purpura (ITP), but the results are controversial. Here we describe the use of a high dose of dexamethasone (40 mg per day for 4 days every month) in 18 patients with chronic ITP. The median age of the patients was 42.5 years (range, 16–77 years); 13 were female and five male. The duration of the disease ranged from 5 to 480 months, and splenectomy was carried out in six of the 18 patients.The overall results obtained revealed a satisfactory response (platelet counts higher than 50×10⁹/l) in eight of the 18 patients. However, a long-term remisson was achieved in only three of the eight patients with a follow up of 7–16 months. We were not able to identify any clinical or laboratory prognostic parameters or previous treatment which would allow one to predict a successful outcome of this treatment. These results suggest that a high dose of dexamethasone may provide an alternative, be it a poor one, for the treatment of refractory IPT, in which the use of a low-cost drug with limited side effects is an important consideration. Received: 18 March 1996 / Accepted: 2 July 1996     

Dapsone for refractory chronic idiopathic thrombocytopenic purpura

Summary. Fifteen patients with refractory chronic idiopathic thrombocytopenic purpura (ITP) were treated with dapsone (lOOmg/d) for 1-31 months. The overall response rate to dapsone was 40%. Five patients responded in 1 month and one patient in 2 months. No pretreatment characteristics -sex, age, platelet count or duration of ITP - were correlated with response to dapsone. Treatment was well tolerated. The most frequent adverse effect was dose-related haemolytic anaemia. In our experience, dapsone provides an inexpensive and well-tolerated alternative for patients with ITP who had inadequate reponses to conventional therapy.     

Evidence for a light chain restriction of glycoprotein Ib/IX and Ilb/IIIa reactive antibodies in chronic idiopathic thrombocytopenic purpura (ITP)

To address the assumption of clonally restricted antibodies in immune thrombocytopenias we studied sera from 19 patients with chronic ITP known to possess antibodies reactive with glycoprotein (GP) Ib/IX and/or GPIIb/IIIa. These sera were re-analysed using the standard monoclonal antibody immobilization of platelet antigens (MAIPA) assay and 16 patients exhibited IgG antibodies reactive with GPIIb/IIIa; seven patients showed also a reactivity with GPIb/IX. Employing a light-chain-specific MAIPA assay, 75% (12/16) of these sera displayed GPHb/ Ilia-specific antibodies that were light chain restricted; only 13% (2/16) of the GPIIb/IHa reactive sera showed a mixed kappa and lambda phenotype. A light-chain-restricted phenotype was also seen for the GPIb/IX reactive antibodies. To further substantiate these findings, the MAIPA assay was modified in order to avoid interference from human anti-mouse antibodies. A high frequency of light-chain restricted platelet antibodies was also found using the modified MAIPA technique. These results support the hypothesis of a clonal B-cell expansion in immune thrombocytopenias, producing antibodies with a restricted idiotype repertoire and reacting with a limited number of epitopes.     

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.     

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.     

慢性特发性血小板减少性紫癜自身抗体克隆性分析

目的 了解慢性特发性血小板减少性紫癜(ITP)自身抗体克隆性生成特性。方法 采用改良的单克隆抗体免疫固定特异血小板抗原(MAIPA)法检测43例慢性ITP患者血清血小板膜糖蛋白(GP)特异性IgG抗体及其重链亚型和轻链表型,采用PCR技术分析患者淋巴细胞免疫球蛋白重链基因重排。结果 43例中16例(38％)血清中至少存在一种抗GP(GPⅡb／Ⅲa、GPⅠb、GPⅠa、GPⅣ、GPⅤ)IgG抗体。73％(8／11)的血清糖蛋白特异性自身抗体表现重链限制性,仅表达一种重链亚型;80％(16／20)的糖蛋白特异性抗体仅表达一种轻链表型;6例患者的糖蛋白特异性抗体既表现为轻链限制性又表现为重链限制性。PCR分析显示,3例存在淋巴细胞重链基因重排。结论 部分慢性ITP患者GP特异性自身抗体源于寡克隆B淋巴细胞增生。     

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。     

High-dose intravenous IgG for chronic idiopathic thrombocytopenic purpura in adults

Summary Sixteen adult patients of mean age 48 years with chronic ITP were studied for platelet response to high-dose (0.4 g/kg body weight per day for five consecutive days) intravenous polyvalent intact IgG in the absence of any concurrent treatment. The platelet count returned to normal values in nine patients, a partial response (rise in the platelet count between 50 and 150 ×10⁹/l) was observed in three cases. One patient refractory to any other treatment went into a sustained remission. In the other responsive patients the response was only transient. Among seven splenectomised patients only three responded to IgG infusions versus nine in the non-splenectomised group. The length of ITP history appeared as a more critical factor for the response to IgG than previous splenectomy.     

慢性特发性血小板减少性紫癜患者血清转化生长因子-β1测定的意义

目的探讨血清转化生长因子-β1（TGF-β1）在慢性特发性血小板减少性紫癜（ITP）中的临床意义。方法采用双抗体夹心酶联免疫吸附法（ELISA）检测38例初治慢性ITP患者治疗前后血清中TGF-β1水平。结果慢性ITP患者治疗前TGF-β1水平明显高于对照组（P〈0．01）。经治疗缓解后血清TGF-β1水平与治疗前比较明显下降（P〈0．01）。未缓解者TGF-β1水平与初诊患者比较差异无统计学意义（P〉0．05）。TGF-β1水平与血小板数呈负相关（r=-0．342,P〈0．05）,与巨核细胞数呈正相关（r=0．409,P〈0．01）。结论TGF-β1参与了慢性ITP的发病,检测TGF-β1水平变化有助于对慢性特发性血小板减少性紫癜患者病情的判断,可作为疗效观察的辅助指标。     

Parvovirus B19 infection and idiopathic thrombocytopenic purpura*

 The potential association of human parvovirus B19 infection with idiopathic thrombocytopenic purpura (ITP) was studied. All 60 adult patients presenting with ITP at the University Hospital Rotterdam – Dijkzigt during a 12-year period (41 with acute ITP, 19 with chronic ITP) were included. Patient files were retrospectively analyzed. Stored serum samples were tested for parvovirus B19-specific IgG and IgM antibodies, and for parvovirus B19 DNA. In only one patient (1.7%) was evidence of recent B19 infection found. Parvovirus B19 is not a frequent cause of adult ITP and should be tested for only when there are other indications of possible parvovirus B19 involvement. Received: 9 October 1995 / Accepted: 14 November 1995     

特发性血小板减少性紫癜患者外周血单个核细胞中糖皮质激素诱导的亮氨酸拉链蛋白mRNA表达的研究

目的探讨特发性血小板减少性紫癜（ITP）患者使用糖皮质激素治疗前后外周血单个核细胞中GILZmRNA的表达及作用。方法选择未使用糖皮质激素治疗的ITP患者23例（病例对照组）、使用糖皮质激素治疗后的患者22例（治疗组）及健康体检者22例（正常对照组）作为研究对象,采用RT—PCR法检测其外周血单个核细胞中GILZmRNA的表达,同时采用ELISA法检测其血清中IFN-γ、IL-10的表达。结果与正常对照组相比,病例对照组GILZmRNA表达明显降低（P〈0．01）,IFN-γ表达明显升高（P〈0．01）,IL-10表达明显降低（P〈0．01）,而治疗组GILZmRNA、IFN-γ、IL-10表达差异则无明显统计学意义（P均〉0．05）。结论糖皮质激素可以上调TP患者外周血单个核细胞中GILZmRNA表达;GILZmRNA表达上调可明显抑制Th1细胞因子分泌,增强Th2细胞因子分泌,纠正ITP患者血清中Th1／Th2的失衡。     

特发性血小板减少性紫癜患者治疗前后Th1／Th2水平变化及其临床意义

目的：研究特发性血小板减少性紫癜（ITP）患者药物治疗前后辅助性T淋巴细胞（Th）1/Th2细胞因子水平的变化情况及其临床意义。方法：应用流式细胞仪检测60例ITP患者药物治疗前后干扰素γ（IFN-y）、白细胞介素（IL）-2、IL-4、IL-10水平,分析Th1/Th2细胞因子水平的变化情况,并结合疗效作相关性分析。以60名健康体检者为对照。结果：药物治疗有效的ITP患者治疗后Th1细胞因子IL-2、IFN-1水平较治疗前及对照组均显著升高（P〈0．01）。而Th2细胞因子IL-4及IL-10水平在3组间均无显著性差异（P＞0．051．Th1/Th2比例升高。ITP患者Th1细胞因子IL-2、IFN．叮水平在治疗显效组表达均明显高于良效及进步组和无效组（P〈0．05）,良效及进步组与治疗无效组比较亦有显著性差异（P〈0．05）;经相关性分析,患者的疗效与IFN-γ、IL-2均呈正相关。结论：ITP患者药物治疗后Th1细胞因子IL-2、IFN-γ表达升高,且Th1与ITP患者疗效密切相关。