儿童自身免疫性溶血性贫血和Evans综合征复发及其相关因素分析

目的研究和探讨儿童自身免疫性溶血性贫血（AIHA）和Evans综合征复发率及其相关因素。方法采用同期病例对照法，比较不同因素与复发的相关性。结果40例患者中有7例复发，复发率为17．5％，Coombs阴性患者复发率为66．66％，Coombs阳性患者复发率为13．51％。复发发生在首次发病的2个月到2年7个月之间，4例在停药后，2例在激素减量中。复发诱因中感染因素占4例（57．14％），主要为呼吸道和消化道感染；无诱因者3例（42．86％）。复发组和未复发组患儿的性别、抗体的类型、是否存在其他自身免疫性疾病、患病时的血红蛋白水平和网织红细胞计数等均与复发无关（P 〉 0．05）。细胞免疫功能的检查结果显示复发组CD3增高、CD8降低，经统计学分析两组存在显著差异（P 〈 0．05）。结论儿童AIHA和Evans综合征的复发率远低于成人，复发患者免疫功能异常较未复发患者显著。     

造血器管及血液疾病

053667儿童自身免疫性溶血性贫血和Evans综合征复发及其相关因素分析／景虹…∥中国小儿血液．-2005,10(3)．-113- 116 40例中有7例复发,复发率为17．5％,Coombs阴性患儿复发率为66．66％,Coombs阳性患者复发率为13．51％。复发诱因中感     

IL-4可变串联重复序列区基因多态性在小儿激素敏感性肾病综合征复发中的意义

目的探讨IL-4基因VNTR区的基因多态性与小儿激素敏感性肾病综合征复发的关系。方法选择激素敏感性肾病综合征患儿55例,按发病后1年疾病复发情况分为频复发组28例和非频复发组27例(40例有肾活检资料)。对照组115例,选自健康献血员。采用聚合酶链式反应(PCR)及PCR产物测序方法,检测患儿与对照组IL-4基因VNTR区的基因型别。对48例患儿于发病初(频复发组23例,非频复发组25例)检测其血清IgE水平。结果(1)激素敏感性肾病综合征患儿和对照组IL-4基因VNTR区的基因型的分布差异无统计学意义(P>0·05)。(2)频复发组B1B1分布频率(96·4%)明显高于非频复发组(66·7%)(P<0·05)。(3)发病初期血清中IgE水平:频复发组为(1·98±0·23)g/L,非频复发组为(1·05±0·19)g/L(P<0·05)。B1B1组IgE水平为(1·68±0·18)g/L,B1B2和B2B2组为(0·81±0·21)g/L(P<0·05)。结论激素敏感性肾病综合征患儿携带IL-4VNTR区的B1B1基因型者,容易表现为频复发。     

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目的探讨米多君对血管迷走性晕厥儿童的治疗效果。方法将2003-07—2004-12在北京大学第一医院儿科就诊的46例晕厥反复发作、直立倾斜试验(HUT)阳性的血管迷走性晕厥患儿,分为米多君组、美托洛尔组及基础治疗组。首先应用HUT评价患儿的治疗反应及调整药物,所有患儿随访6个月后,如没有晕厥的发作者则停药,并继续随访。进一步评价患儿晕厥复发情况及药物的不良反应。结果米多君组、美托洛尔组及基础治疗组3组患儿HUT转阴率分别为75·0%、65·0%及20·0%。米多君组及美托洛尔组患儿的HUT转阴率明显高于基础治疗组(P均<0·05),而给药治疗的两组患儿的HUT转阴率差异无显著性(P>0·05)。在随访过程中,米多君组及美托洛尔组晕厥复发率分别为22·2%及30·7%,而基础治疗组的晕厥复发率为80·0%,前两组晕厥复发率显著低于后组(P均<0·05)。前两组之间的复发率差异无显著性(P>0·05)。结论米多君可有效治疗血管迷走性晕厥儿童。     

三色流式细胞术在120例儿童急性白血病免疫分型中的应用

目的探讨三色流式细胞术在儿童急性白血病AL免疫分型中的应用价值,了解儿童AL抗原表达规律和免疫亚型的分布情况。方法采用CD45/侧散射(SSC)双参数散点图设门方法进行三色流式细胞术细胞表面及浆内分化抗原的分析。结果120例儿童白血病免疫分型可分为4类:未分化型占0·8%,急性髓细胞性白血病(AML)占35·0%,急性淋巴细胞白血病(ALL)占57·5%,混合型急性白血病占6·7%。69例儿童ALL中,B淋巴细胞白血病(B-ALL)占75·3%,T淋巴细胞白血病(T-ALL)占24·7%。AML伴淋巴系抗原表达为28·8%,主要表达CD5、CD7及CDl9(均为9·6%)。B-ALL伴髓系抗原CDl3、CD33表达分别为40·4%和5·8%,T-ALL伴髓系抗原CD13、CD33表达分别为35·3%和5·9%。结论应用流式细胞术几乎能对所有儿童急性白血病进行准确分型,对儿童白血病患者的治疗方案选择及预后判断等均有重要价值。     

儿童130例人巨细胞病毒感染的血液学有关表现

目的探讨儿童人巨细胞病毒(humancytomegalovirus,HCMV)感染的血液学表现。方法回顾性分析130例人巨细胞病毒感染患儿的临床特征、血象、骨髓象和免疫学指标的变化及治疗和转归。结果130例中有64例出现外周血象的改变(49·2%)。其中贫血46例(35,4%),血小板减少35例(26·9%),粒细胞减少4例(3·1%),全血细胞减少5例(3·8%),外周血出现异型淋巴细胞11例(8·5%)。29例出现血液系统并发症(22·3%),包括传染性单核细胞增多综合征、特发性血小板减少性紫癜(ITP)、自身免疫性溶血性贫血(AIHA)、病毒相关性噬血细胞综合征(VAHS)等。部分行免疫功能检查的患儿中,27·5%的病人存在体液免疫异常,71·4%存在细胞免疫异常。结论儿童是HCMV感染的易感人群。HCMV几乎可以感染人体所有组织,造血系统受累,可引起多种造血功能紊乱,甚至发生多种CMV感染相关性血液系统疾病。早期足疗程地应用更昔洛韦,配合对症、支持、免疫调节等综合治疗,可明显提高疗效。     

儿童Evans综合征8例临床分析

目的探讨儿童Evans综合征的诊断及治疗。方法 2004年3月-2013年7月郑州大学第一附属医院儿科共收治儿童Evans综合征8例,分析临床特征及治疗效果。结果 8例患儿中6例男性,2例女性,首次诊断中位年龄1岁(4个月~3岁10个月)。8例患儿中,5例直接抗人球蛋白试验(DAT)阳性(62%);3例行血小板抗体(HPA)检查,均阳性(100%);8例患儿均行骨髓穿刺检查,提示增生性贫血5例(62%),免疫性血小板减少症2例(25%),骨髓象正常1例(13%);3例行细胞免疫功能检查,均有B淋巴细胞(CD19~+)比例增高(100%),其中2例合并有Th淋巴细胞(CD3~+CD4~+)比例下降(67%)。8例患儿中,5例同时接受糖皮质激素及丙种球蛋白(IVIG)治疗,IVIG用量均为500mg/(kg·d)×5d,糖皮质激素应用地塞米松磷酸钠/甲泼尼龙琥珀酸钠/泼尼松,4例达CR或PR(总有效率80%),1例无效;单一应用IVIG和糖皮质激素治疗各1例,均无效(0/2);1例仅经过抗感染治疗后达PR。中位随访时间18(14~24)个月,随访期间2例复发,最长维持缓解期为3个月。截止至末次随访时间,2例死亡,3例持续CR,3例失访。结论 Evans综合征难治且易复发,患儿可因出血或感染而死亡。初发病例激素与IVIG联用疗效肯定;复发或耐药病例,长期单用激素疗效不满意,且易合并严重感染,建议多药联合治疗。     

抗凋亡基因Bcl-2在儿童急淋白血病中表达与早期治疗反应关系的初步研究

目的探讨骨髓细胞抗凋亡基因Bcl-2表达蛋白在儿童急性淋巴细胞白血病(ALL)患者中的表达状况与临床相关危险因素及其早期治疗反应的关系。方法采用S-P免疫组织化学方法,检测32例ALL患者治疗前后骨髓细胞Bcl-2的表达。选择同期住院的非白血病患者15例正常骨髓作为对照。结果Bcl-2蛋白在ALL中表达明显高于对照组(t=3·2558、P<0·01);治疗前后高危组、中危组、低危组间比较均有显著性差异(F分别为3·4317、4·1132,P均<0·05,早期治疗反应差与早期治疗反应好组间比较均有显著性差异(t分别为2·9462、2·3747,P<0·01、<0·05);治疗反应好组,一个疗程后Bcl-2蛋白表达水平显著下降(t=1·7309;P<0·05),治疗反应差组,一个疗程后Bcl-2蛋白表达水平下降,但较化疗前无显著性差异(t=1·1875;P>0·05),仍显著高于对照组病例(t=2·4164;P<0·05)。结论抗凋亡基因Bcl-2蛋白在儿童急淋白血病患者中存在高表达,与临床危险因素有关,可以作为一个影响儿童ALL疗效、预后,评定危险程度的新的生物学指标。     

玉屏风散治疗单纯性肾病综合征儿童继发性免疫功能低下

目的研究玉屏风散防治儿童单纯性肾病综合征(SNS)继发性免疫功能低下的疗效。方法选择小儿SNS 78例,抽签法随机分为治疗组和对照组,在予免疫抑制剂治疗同时,治疗组另加用玉屏风散。观察2组治疗前后SNS患儿并感染和复发、血清IgG和IgA水平、T细胞亚群变化。结果治疗组SNS并感染的发病率为55.3%,较对照组85%明显降低;治疗组SNS复发率39.5%,也较对照组67.5%明显减少;治疗组SNS血清IgG和IgA水平由治疗前(3.88±1.22)g/L和(0.83±0.14)g/L恢复为治疗后(10.06±1.89)g/L和(1.38±0.10)g/L,且明显高于对照组的(6.23±1.55)g/L和(0.85±0.13)g/L;治疗组SNSCD4 、CD8 细胞比率和CD4 /CD8 比值分别由治疗前(37.91±3.89)%(、30.69±3.08)%和(1.24±0.49)明显恢复为治疗后(42.38±4.89)%、(26.01±2.20)%和(1.63±0.41),并明显优于对照组治疗后(39.87±3.91)%(、29.76±3.55)%和(1.34±0.24)。结论玉屏风散治疗儿童SNS继发性免疫功能低下有可行性。     

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目的了解喘息与非喘息型病毒性肺炎患儿淋巴细胞亚群的改变。方法对2002—2004在北京友谊医院就诊的急性期喘息型病毒性肺炎患儿37例及非喘息型病毒性肺炎患儿32例,采用流式细胞仪对其外周血淋巴细胞亚群进行检测。并以30例健康儿童作为对照组。结果喘息组与非喘息组病毒性肺炎患儿急性期外周血T辅助淋巴细胞亚群Th1细胞百分率分别为(12·61±7·19)%及(17·32±9·92)%,与对照组[(9·16±9·90)%]比较差异均有显著的统计学意义(P<0·001);喘息组与非喘息组患儿Th1/Th2比值均高于对照组(P<0·05,P<0·01)。此外与非喘息组比较,喘息组CD4+/CD8+较高(P<0·05),但其他各项指标差异均无统计学意义。结论病毒性肺炎时不论是否发生喘息,均呈现Th1应答优势。喘息型病毒性肺炎患儿除CD4+/CD8+较非喘息型病毒性肺炎高外,其他各项淋巴细胞亚群差异无明显统计学意义。     

Autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura in pediatric solid organ transplant recipients, report of five cases and review of the literature

Cytopenias are common among pediatric SOT; however, autoimmune cytopenias are infrequently reported. We report five cases of autoimmune cytopenias in pediatric LT patients: two with isolated IgG-mediated AIHA, two with ITP, and one with Evans syndrome (ITP and AIHA). All patients were maintained on tacrolimus as immunosuppression. Viral illness commonly preceded the autoimmune cytopenias. All patients responded well to medical therapy (steroids, intravenous immunoglobulin, and rituximab) and lowering tacrolimus serum level. Prognosis appears to be worse when more than one cell line (e.g., Evans syndrome) is affected, and/or there is no preceding viral illness. A critical literature review of autoimmune cytopenias in children following SOT is conducted. Autoimmune cytopenias are a rarely reported complication of pediatric SOT, but clinicians taking care of pediatric transplant recipients need to be aware of this complication.     

微量血检测红细胞表面抗体诊断小儿自身免疫性溶血性贫血研究

目的：　采用微量血检测自身免疫性溶血性贫血(AIHA)患儿的红细胞表面抗体,对其方法学与诊断学进行探讨。方法：　对145例疑为AIHA患儿用微量方法检测直接Coombs试验及红细胞表面单价抗体,并对其中30例病人进行了静脉血脱纤维蛋白后的Coombs试验,对两种方法进行比较。结果：　 145例标本共检出AIHA 13例 ,所有患者Coombs试验阳性,其中单价抗体IgG阳性有7例,IgG +C31例 ,IgG +IgM +C33例,IgM +C32例。同时对30例检测对象进行了微量血与脱纤维蛋白血的对比,其中 4例阳性者,两种检测方法一致;2 6例阴性结果中微量血假阳性率3.85% ,脱纤维蛋白血假阳性率为7.69%,二者差异无统计学意义(P>0.05)。对两种方法洗涤后红细胞上清液分析显示,3次洗涤后上清液中所含总蛋白差异无显著性(P>0.05)。结论：　用微量血检测红细胞表面抗体诊断AIHA与静脉脱纤维蛋白血检测结果基本一致,采血少,操作方便,且易于复查     

Autoimmune hemolytic anemia

Objective  To study the clinico-hematological profile and treatment outcome in children suffering from auto immune hemolytic anemia (AIHA). Methods  Twelve children were diagnosed with auto immune hemolytic anemia over a period of four years. Direct antiglobulin test was positive in all the cases. Other causes of hemolytic anemia like thalassemia syndromes, hereditary spherocytosis, G6PD deficiency were excluded by appropriate tests. The children were followed up for 6 months to 4 years. Results  The age ranged from 7 mth to 9 yr with a mean age of 4.51 yr. All patients had pallor as the presenting complaint followed by splenomegaly (83.3%), jaundice (66.7%), fever (50%) and bleeding manifestations (16.7%). 9 patients had primary disease and 3 had secondary disease. Tubercular infection was seen in 2 patients with secondary disease. Jaundice was seen equally in both the groups. Oral prednisolone produced remission in 83.3% cases. 4 patients (3 in primary and one in secondary group) had relapse after initial response. All responded to a second course of steroids but had subsequent relapses and developed a chronic course. Conclusion  Autoimmune hemolytic anemia is an uncommon cause of hemolytic anemia in children. Tubercular infection is an underlying pathology in cases of secondary autoimmune hemolytic anemia. Although oral steroids induce remission in most of the cases, relapses are common.     

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目的观察槐杞黄颗粒辅助治疗原发性肾病综合征(PNS)患儿对淋巴细胞亚群、免疫球蛋白及感染次数的影响。方法 2008-01-01—2009-10-31中国医科大学附属盛京医院小儿肾脏风湿免疫科将住院的101例PNS患儿随机分为A组(62例)和B组(39例),选取正常体检儿童22名为正常对照组。A组采用糖皮质激素联合槐杞黄颗粒治疗,B组单独服用激素。分别于治疗前和治疗3个月、6个月检测患儿的淋巴细胞亚群、免疫球蛋白及记录感染次数、肾病复发次数和药物副反应。结果 (1)A、B组治疗前,CD8+高于正常对照组(P<0.05),CD4+、CD4+/CD8+和NK细胞均明显降低于正常对照组(P<0.05),IgA与IgG均低于正常对照组(P<0.05)。(2)治疗3个月,A组(39例)与B组(30例)之间CD8+细胞差异无统计学意义,两组均明显高于正常对照组,差异有统计学意义(P<0.01);B组CD4+及CD4+/CD8+显著低于A组和正常对照组(P<0.05);A组与B组NK细胞均高于治疗前,B组低于同期A组(P<0.05);IgG明显升高但组间差异无统计学意义。(3)治疗6个月,A组(23例)、B组(9例)间各指标均差异无统计学意义,均低于正常对照组(P<0.01)。(4)感染次数,A组发生上呼吸道感染6例次,肺炎2例次,肾病综合征复发4例次。B组发生上呼吸道感染10例次,肺炎3例次,泌尿系感染2例次,肾病综合征复发6例次。1例患儿服槐杞黄颗粒后出现较严重腹泻(排除感染性腹泻)。结论 PNS患儿治疗前细胞、体液免疫功能降低、紊乱;槐杞黄颗粒辅助治疗过程中,可能通过提高NK细胞和T辅助淋巴细胞活性,减少感染并有减少肾病综合征复发的趋势,且副反应轻微。     

Infantile cytomegalovirus-associated autoimmune hemolytic anemia

Autoimmune hemolytic anemia (AIHA) is a hematologic disorder that is rarely seen in infants and young children. Most cases are associated with viral or bacterial infection, but the immunologic events leading to hemolysis are poorly understood. We describe two infants with severe cytomegalovirus (CMV)-associated warm antibody AIHA. One case was immunohematologically analyzed and showed suggestive evidence that endogenous anti-CMV IgG antibodies were the pathogenic antibodies leading to hemolysis, implicating a possible causal relationship between AIHA and CMV infection. Both patients were ultimately treated with intravenous CMV immune globulin, with subsequent improvement. These cases suggest that investigation for the presence of CMV in infantile AIHA is warranted and that CMV immune globulin should be considered as a therapeutic option.     

Successful treatment of tacrolimus‐related pure red cell aplasia and autoimmune hemolytic anemia with rituximab in a pediatric cardiac transplant patient

Acquired pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) are rare complications of immunosuppression in pediatric solid organ transplant patients. We report a 14‐month‐old female child who developed Coombs positive hemolytic anemia and reticulocytopenia while on tacrolimus after cardiac transplantation. She was successfully treated with rituximab after failing treatment with corticosteroids and intravenous immunoglobulins. Clinicians should consider PRCA differential diagnosis in a patient presenting with reticulocytopenia and hemolysis. In addition, the coexistence of PRCA with AIHA, and the response to therapy with rituximab, supports a common immune‐mediated pathogenesis for both disorders.     

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目的通过测定Tourette综合征(TS)患儿血清抗链球菌溶血素O(ASO)、T淋巴细胞亚群和自然杀伤细胞(NKC),观察链球菌感染与TS的关系、TS细胞免疫功能状态及免疫调节剂的治疗作用。方法2005-05—2005-10对首都儿研所神经科门诊就诊的58例TS患儿分别采用透射比浊法检测ASO,双色免疫荧光法检测T淋巴细胞亚群及NKC。以同期健康体检儿童为对照组。对细胞免疫异常者随机分为两组,分别予免疫调节剂或免疫调节剂 多巴胺受体阻滞剂治疗。结果(1)TS患儿ASO阳性率较对照组显著增高(P<0.05);(2)TS患儿中,ASO阳性组CD4 显著低于ASO阴性组(P<0.05);TS患儿CD3 、CD4 、CD4 /CD8 显著低于对照组(P均<0.05),NKC显著高于对照组(P<0.05);(3)细胞免疫异常的TS患儿,单纯采用免疫调节剂治疗或免疫调节剂 多巴胺受体阻滞剂治疗,两组间疗效差异无显著性(P>0.05)。结论部分TS患儿发病与链球菌感染有关;TS患儿存在细胞免疫功能改变,表现为T淋巴细胞亚群平衡失调和NKC升高,链球菌感染可能与之有关;单纯免疫调节剂治疗可能对链球菌感染触发的TS有效。     

Attenuated form of Evans syndrome among pediatric ITP patients

Long term follow up of adult patients with immune thrombocytopenic purpura (ITP) have shown evolvement of secondary autoimmune diseases such as SLE, Evans syndrome, autoimmune neutropenia, Graves disease etc. We studied 30 cases of pediatric ITP patients for evidence of hemolysis to assess the possibility of Evans like syndrome. Measurement of free serum haptoglobin, a sensitive indicator of red cell destruction was used after careful exclusion of micro angiopathic hemolysis, SLE or overt Evans Syndrome. Results showed abnormally low level of free serum haptoglobin in 11 of the 30 (36.7%) patients compared to that in 20 age matched controls (P < 0.001) as an evidence of hemolysis. Our data in pediatric patients is similar to that reported in adult ITP cases and support the observation of Evans made 50 years ago that there is a spectrum like relationship between primary thrombocytopenia and hemolytic anemia. Thus the concept of attenuated form of Evans syndrome could be considered, in group of patients with ITP in pediatric age group.     

单纯性肾病综合征患儿外周血单个核细胞病毒分子表达的研究

目的　探讨外周血单个核细胞 (PBMC)呼吸道病毒感染与糖皮质激素敏感型单纯性肾病综合征 (SSNS)的发生和反复的关系。方法　采用免疫组化、原位杂交、酶联免疫吸附和病毒分离技术 ,对青岛大学医学院附属医院儿科和滨州医学院附属医院 2 0 0 0年 1月至 2 0 0 2年 12月收治的 36例初发组、15例反复组和 2 1例缓解组SSNS患儿检测了PBMC病毒分子表达和血清中病毒特异性IgM抗体水平。结果　初发组与反复组比较 ,PBMC中呼吸道合胞病毒抗原和肠道病毒基因表达的阳性率差异均无显著性 ;而初发组与缓解组和对照组比较及反复组与缓解组和对照组比较 ,PBMC中两种病毒感染的阳性率差异均有显著性 (P <0 0 5 ,P <0 0 1)。初发组与其他各组比较 ,不同病毒的特异性IgM抗体阳性率明显增高 ( P <0 0 5 ) ;而反复组与缓解组和对照组比较 ,多种病毒的特异性IgM抗体的阳性率差异均无显著性。 结论　初发和反复SSNS患儿的PBMC均易被病毒感染 ,糖皮质激素的应用并不影响细胞内病毒抗原和基因的表达 ,但可使病毒特异性IgM抗体的产生明显减少 ;PBMC内病毒抗原或基因表达阳性可做为SSNS有反复倾向的重要分子标志之一。     

Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance

Thrombocytopenia and autoimmune hemolytic anemia (Evans syndrome) with the presence of both warm and cold autoantibodies (mixed type) are rare in the pediatric age group. This condition may be associated with other autoimmune disorders and is notoriously difficult to treat. This case describes an adolescent male who presented with rapid onset Evans syndrome and diabetes. After failing to respond to high dose prednisone and intravenous immunoglobulin, the patient was successfully treated with monoclonal antibody against CD20 (anti-CD20). This suggests that anti-CD20 is a valuable treatment for severe warm and cold antibody mediated Evans syndrome, and possibly for select cases of antibody mediated diabetes.