人血小板功能抗体及其片段的识别

目的研究特发性血小板减少性紫癜（ITP）患者血小板膜糖蛋白（GP）特异性IgG抗体及其片段的免疫活性及对血小板聚集功能的影响.方法用改良单克隆抗体特异性俘获血小板抗原技术（MAIPA）检测84例慢性ITP患者血浆中抗GPⅡb/Ⅲa、GPⅠb/Ⅸ及GPⅥ自身抗体,比浊法血小板聚集试验筛选出自身抗体阳性并能抑制血小板聚集的患者,用蛋白A柱纯化其血浆IgG抗体并用胃蛋白酶制备F（ab＇）2片段.检测纯化的IgG抗体及其酶切片段与血小板GP的结合活性及对正常人血小板聚集功能的影响.结果 （1）84例ITP患者血浆中,48例（57.1%）抗GPⅡb/Ⅲa和/或GPⅠb/Ⅸ和/或GPⅥ自身抗体阳性,其中7例（14.6%）明显抑制了二磷酸腺苷、瑞斯托霉素或胶原诱导的血小板聚集;（2）纯化的IgG及F（ab＇）2片段具有与相应血小板GP的结合活性;（3）4例患者纯化IgG及F（ab＇）2片段具有抑制血小板聚集的功能.结论 F（ab＇）2片段是IgG自身抗体的功能片段,它不但保留了良好的抗原结合活性且可部分抑制血小板聚集功能,为人源化血小板糖蛋白特异性抗体的制备奠定了基础.     

Acute appendicitis in Henoch-Schönlein purpura: a case report

Common complications of Henoch-Schönlein purpura (HSP) that lead to surgical intervention include intussusception, perforation, necrosis, and massive gastrointestinal bleeding. Acute appendicitis is rarely seen as a complication of HSP. A seven-year-old boy was admitted for arthralgia, abdominal pain, hematochezia, melena, and purpuric rash on the lower extremities. On admission day abdominal ultrasonography was normal, but on day 5, he became pyrexial and developed right iliac fossa pain and tenderness with guarding. Ultrasonography showed distended appendix surrounded by hyperechoic inflamed fat. On exploration an acutely inflamed, necrotic appendix was removed and grossly there was an appendiceal perforation in the appendiceal tip. Microscopically some of the small blood vessels in the submucosa showed fibrinoid necrosis with neutrophilic infiltrations. The authors report the case of a child who developed acute perforative appendicitis requiring appendectomy while on treatment for HSP.     

Childhood idiopathic thrombocytopenic purpura in the Nordic countries: epidemiology and predictors of chronic disease

AIM: To describe the epidemiology of idiopathic thrombocytopenic purpura (ITP) in the Nordic countries, to define clinical subgroups and to investigate factors predicting chronic disease. METHODS: A prospective registration was done from 1998 to 2000, including all children with newly diagnosed ITP aged 0-14 y and at least one platelet count <30 x 10(9)/l. RESULTS: 506 children were registered and 423 followed for 6 mo. The incidence was 4.8/10(5) per year. Most children were aged 0-7 y (78%), with a predominance of boys, while patients aged 8-14 y had equal representation of the two sexes. There were seasonal variations determined by variations in postinfectious cases with sudden onset. The platelet count was <10 x 10(9)/l in 58%, but bleeding manifestations were mild or moderate in 97%. The insidious form (symptoms for more than 2 wk) was more frequent in older children and girls, showed little seasonal variation, had milder manifestations and ran a chronic course in more than half the cases. Intracranial haemorrhages did not occur in the first 6 mo after diagnosis. Chronic ITP developed in 25%. The strongest predictor of chronic disease was insidious onset of symptoms (OR 5.97). CONCLUSION: In the Nordic countries, ITP mainly affects children aged 0-7 y, with a winter bulk of postinfectious cases superimposed on a steady occurrence of non-infectious cases. Clinically, it may be useful to distinguish between children with sudden versus insidious onset of symptoms rather than between different age groups.     

儿童特发性血小板减少性紫癜小巨核细胞的研究

目的 探讨巨核细胞生长发育、小巨核细胞的出现在儿童特发性血小板减少性紫癜(idiopathic thrombocytopenia purpura,ITP)中的意义。方法 对46例儿童ITP进行常规骨髓形态学、骨髓涂片CD41免疫酶标染色检测,并采用ELISA方法检测血小板相关抗体的含量。并分为急性ITP(acute ITP,AITP)组和慢性ITP(chronic：ITP,CITP)组进行统计学分析。结果 46例中36例为AITP,1O例为CITP;45例骨髓巨核细胞计数增高或正常,1例降低者为CITP。小巨核细胞检出率为98％,(45／46),I型淋巴样小巨核细胞检出率为35％(16／46)。CITPI型淋巴样小巨核细胞的检出率及计数高于AITP,其他三型小巨核细胞计数低于AITP,产板巨核细胞比例以及各型小巨核细胞数量与血小板相关抗体的含量无相关性。结论 儿童ITP骨髓巨核细胞系存在不同程度病态造血,其原因可能并非完全由于免疫因素,部分病例可能以骨髓巨核细胞自身异常为主。I型淋巴样小巨核细朐的出现以及数量的高低可存一定程度上提示ITP病程的长短和预后。     

Thrombocytopenic purpura as a presenting manifestation of tubercular lymphadenitis

A 11-year-old girl presented with thrombocytopenic purpura along with cervical lymphadenopathy. There was no hepatosplenomegaly; no evidence of microangiopathy; bone marrow examination was normal. Acid fast bacilli were seen in fine needle aspiration cytology of the lymph node. A diagnosis of tubercular lymphadenitis in association with immune thrombocytopenia was made, and the platelet count recovered with antitubercular therapy. This report illustrates immune thrombocytopenia as a rare manifestation of childhood tuberculosis; the authors also discuss other causes of thrombocytopenia in childhood tuberculosis.     

Plasmapheresis in a very young infant with atypical hemolytic uremic syndrome

Atypical hemolytic uremic syndrome (HUS) is a heterogeneous group of disorders, the pathogenesis of which is unclear. Plasma transfusions and plasmapheresis are widely used modes of therapy for adults with this life-threatening syndrome. There is very limited experience in using plasmapheresis therapy in children and infants with atypical HUS. Plasmapheresis, which is considered a relatively safe procedure in adults and older children, may be hazardous in neonates and very young infants and can result in severe complications. We report a 2-month-old infant with idiopathic atypical HUS, who was successfully treated with a 1-month course of plasmapheresis during the acute phase of the disease. Appropriate preparations as well as several adjustments were made in order to meet the special needs of this very young infant who, to the best of our knowledge, is the youngest reported patient with atypical HUS to undergo plasmapheresis. Plasmapheresis therapy of the infant was not associated with any complications of the procedure and resulted in marked clinical improvement. We conclude that plasmapheresis in neonates and in very small infants is technically feasible, can be performed without major complications, and may be of benefit in individual cases. Received: 11 November 1999 / Revised: 3 August 2000 / Accepted: 10 August 2000     

Toll样受体在儿童ITP的表达及临床意义

目的探讨外周血血小板表面天然免疫分子TLR2、TLR4表达的变化及相关性在儿童特发性减少性紫癜(ITP)发病中的意义。方法采用流式细胞学技术检测24例急性特发性血小板减少性紫癜(AITP)患儿、20例慢性特发性血小板减少性紫癜(CITP)患儿和20例对照儿童外周血血小板表面TLR2、TLR4的水平。采用SPSS13.0进行统计学分析,P<0.01为差异有统计学意义。结果AITP组、CITP组患儿CD41+TLR2+、CD61+TLR4+阳性率显著低于对照组(P<0.01);并且AITP组显著低于CITP组(P<0.01);两者的表达呈正相关(rs=0.65,P<0.01)。结论联合检测外周血血小板表面TLR2和TLR4表达的变化有助于进一步研究儿童ITP的发病机制,有望成为ITP患儿早期分型的指标之一。     

霉酚酸酯治疗成人紫癜性肾炎的疗效观察

目的探讨新型免疫抑制剂霉酚酸酯(MMF)联合糖皮质激素治疗成人紫癜性肾炎的临床疗效。方法对26例紫癜性肾炎的患者在激素(泼尼松)治疗的基础上加用MMF治疗1年。结果24h尿蛋白定量、尿红细胞计数、血清尿素氮、血清肌酐,在治疗6个月及1年后均有明显改善(P<0.05)。副作用:胃肠道反应(26.92%)、肺炎(15.39%)。结论MMF联合糖皮质激素治疗成人紫癜性肾炎效果较好,副作用轻,但需注意合并症发生。     

过敏性紫癜表现及治疗效果与淋巴细胞凋亡的关系

目的通过观察79例过敏性紫癜(HSP)患儿治疗前后淋巴细胞凋亡情况,进一步阐明淋巴细胞凋亡(LA)在HSP中的表现及与各种治疗效果的关系。方法采用原位末端标记技术,对过敏性紫癜患儿治疗过程中淋巴细胞凋亡分组进行测定,在光学显微镜下观察,计算出凋亡比率,然后将急性期与正常组比较、病情较轻的HSP皮肤型与病情较重的HSP复合型比较、恢复期与正常组比较、常规方法治疗未愈的治疗前后比较、常规方法治疗及加用免疫抑制剂方法的治疗前后比较,各组均采用SPSS统计软件进行t检验统计学处理。结果发现急性期患儿组较正常对照组淋巴细胞凋亡情况有显著延迟(P<0.01),恢复期患儿组较急性期患儿组淋巴细胞凋亡情况有明显改善(P<0.01),恢复期患儿组与正常对照组淋巴细胞凋亡情况无明显的差异(P>0.05),经常规方法痊愈的HSP皮肤型淋巴细胞凋亡延迟情况已得到改善(P<0.01),但急性期未痊愈的HSP复合型淋巴细胞凋亡延迟情况未得到改善(P>0.05),急性期病情较重的HSP复合型较病情较轻的HSP皮肤型淋巴细胞凋亡延迟明显(P<0.05),应用雷公藤、激素、甲氨喋呤等免疫抑制剂后淋巴细胞凋亡加速(P<0.01)。结论急性期过敏性紫癜存在淋巴细胞凋亡的延迟,且症状较重HSP较症状较轻HSP的淋巴细胞凋亡延迟明显;经治疗达临床痊愈的恢复期过敏性紫癜淋巴细胞凋亡情况已达正常水平;常规方法治疗可使症状较轻的HSP淋巴细胞凋亡延迟得以改善,但对症状较重的复合型HSP的病例,须加用免疫抑制剂如雷公藤、激素、甲氨喋呤等使淋巴细胞凋亡加速,从而达到治愈目的。