小儿自身免疫性溶血性贫血23例临床分析

了解小儿自身免疫性溶血性贫血与其他免疫性疾病或遗传性疾病的关系 ,以回顾分析 2 3例小儿自身免疫性溶血性贫血的临床资料 ,结果显示 ,2 3例中有 6例合并于其他免疫性疾病 ,占 2 6% ;有 4例合并于遗传性血液病 ,占 17 4%。     

小儿自身免疫性溶血性贫血23例临床分析

了解小儿自身免疫性溶血性贫血与其他免疫性疾病或遗传性疾病的关系，以回顾分析23例小儿自身免疫性溶血性贫血的临床资料，结果显示，23例中有6例合并于其他免疫性疾病，占26%；有4例合并于遗传性血液病，占17.4%.     

地中海贫血并自身免疫性溶血性贫血1例、并红细胞G-6-PD 3例

我科收治了地中海贫血合并自身免疫性溶血性贫血1例、合并红细胞葡萄糖-6-磷酸脱氢酶缺陷(G-6-PD)3例患儿,现报告如下.     

自身免疫性溶血性贫血的病因、临床与诊断

自身免疫性溶血性贫血(AIHA)是由于体内产生与自身红细胞抗原起反应的自身抗体,使红细胞破坏而引起的一种溶血性疾病。一、病因小儿AIHA以继发性为多见。本院与上海新华儿童医院及上海市儿童医院61例中,有原因可查或有相关因素者占77%,其中感染性疾病如呼吸道感染、婴儿肝炎综合征、乙型肝炎、肠道感染、传染性单核细胞增多症及水痘等33例,系统性红斑狼疮及幼年型类风湿共3例,发生在牛痘接种后1例,用药物后3例。     

儿童自身免疫性溶血性贫血治疗选择

儿童自身免疫性溶血性贫血是一种较少见的获得性自身免疫性疾病。准确的分型及病因诊断,是其诊断及治疗依据。目前儿童自身免疫性溶血性贫血主要采用经验性治疗,糖皮质激素为温抗体型一线药物。对于反复及难治患者,近年来利妥昔单抗等二线治疗在儿童中报道增加,取得一定疗效。本文就儿童自身免疫性溶血性贫血诊疗方法的选择进行总结,供临床医师参考。     

系统性红斑狼疮病的多发性血栓形成

患者为5岁女孩。4岁时患系统性红斑狼疮伴自身免疫性溶血性贫血,予以氢化可的松及输注洗涤浓缩细胞治疗。康复出院后,继服强的松龙1年,各项有关检查均无异常。在停用激素3个月时,病情复发,重新口服强的松龙2mg/kg/d。1周后左侧上肢和下肢局部抽搐,脑CT显示右额顶部梗塞,脑脊液检查正常,诊断为脑狼疮。给予甲基强的松龙200mg/日,共     

溶血性贫血的输血治疗

红细胞遭到破坏造成寿命缩短称为溶血。当造血不足以代偿溶血时出现的贫血称为溶血性贫血。儿科临床常见的溶血性贫血有地中海贫血、红细胞葡萄糖-6-磷酸脱氢酶（G-6-PD）缺乏症、自身免疫性溶血性贫血（AIHI）。     

甲基强的松龙治疗Evans综合征2例报告

Evans综合征又称自身免疫性溶血性贫血(AI-HA)合并原发性血小板减少性紫癜。儿童Evans综合征治疗问题的报告较为少见,现将我院收治的2例患儿用大剂量甲基强的松龙治疗的体会报告如     

儿童自身免疫性溶血性贫血29 例临床特点和治疗分析

目的探讨自身免疫性溶血性贫血的病因及治疗。方法回顾分析2013年1月至2015年12月收治的29例自身免疫性溶血性贫血患儿的临床资料。结果 29例患儿中,原发性10例、继发性19例,其中11例发生于感染后。主要临床表现为面色苍白、黄疸、尿色加深及肝脾肿大,21例Coombs试验阳性。29例患儿中,肾上腺皮质激素治疗反应良好22例;治疗效果不佳7例,在联合丙种球蛋白治疗后效果良好。结论自身免疫性溶血性贫血治疗首选药物为肾上腺皮质激素,丙种球蛋白可提高疗效。     

胸腺切除治疗自身免疫溶血性贫血

20年来,关于胸腺在人体免疫能力的重要作用方面认识逐渐提高了。胸腺与人体某些自身免疫如:重症肌无力、类风湿性关节炎、全身性红斑狼疮等的联系也有了一定的认识。本文报导1例7个月婴儿患自身免疫溶血性贫血,用类固醇、免疫抑制剂和脾切除治疗均无效,采用胸腺切除后治愈。     

以血液系统损害为首发症状的儿童系统性红斑狼疮

目的探讨以血液系统改变为首发或主要表现的儿童SLE的临床特点、治疗方案及预后。方法对2005年6月-2011年6月收治以血液系统改变为主并最终确诊为SLE的38例患儿进行回顾性分析。其血液学改变按白细胞改变、贫血及血小板减少进行分析,并随访6～40个月。结果本组患儿血液系统改变中贫血28例(73.7%)、白细胞改变24例(63.2%)、血小板减少15例(39.5%),距确诊SLE的平均时间为8.5(0～24)个月。针对患儿不同症状选择免疫治疗,本组30例患儿SLE基本无活动,6例轻度活动,2例中度活动,无重度活动。15例PLT减少患儿中10例恢复正常,5例PLT维持在安全水平,8例已停药观察。16例自身免疫性溶血性贫血患儿中13例未再出现溶血发作,死亡1例;另2例患儿间断有轻度溶血发作。2例SLE相关再生障碍性贫血治疗显效。1例SLE相关纯红细胞再生障碍性贫血得到有效控制。结论儿童SLE很隐匿,初期常表现为血液系统损害,值得重视,对治疗效果欠佳患儿或青春期前后女童应警惕SLE,延长随访期限,评估病情以选择治疗方案和疗程。     

Systemic lupus erythematosus presenting with mixed‐type fulminant autoimmune hemolytic anemia

We report the case of a 9‐year‐old girl who presented with mixed‐type fulminant autoimmune hemolytic anemia (AIHA) at the onset of systemic lupus erythematosus (SLE). On admission, laboratory investigations indicated very severe anemia (Hb, 2.7 g/dL) with reticulocytosis and positive direct/indirect Coombs tests. In addition, agglutinative reaction was clinically observed. Based on further examinations, the patient was diagnosed with AIHA complicated with SLE, and mixed‐type AIHA was clinically identified. With oral prednisolone and methylprednisolone pulse therapy, the patient entered remission.     

Rituximab in Steroid Refractory Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia is rare in children and infants and steroids are the corner stone of therapy. Management of the patients with steroid refractory/dependent disease is difficult .Rituximab is being used in the treatment of a variety of autoimmune diseases including Autoimmune hemolytic anemia (AIHA),especially in adults but there is scarce data regarding the use of this agent in pediatric AIHA patients.The authors report two cases of steroid refractory AIHA, who responded to rituximab with review the literature of its use in pediatrics.     

小儿溶血性贫血49例临床分析

目的分析49例溶血性贫血患儿的病因及治疗。方法对2006年1月-2010年1月期间在我院住院的49例溶血性贫血患儿的临床特点进行回顾性分析。结果本地区溶血性贫血以G-6-PD缺乏症最多(59%),自身免疫性溶血性贫血次之(22%),符合小儿溶血性贫血多与红细胞内在因素异常有关这一特点。结论本组病例中G-6-PD缺乏是引起溶血性贫血的主要病因,疗效好。对自身免疫性溶血性贫血肾上腺糖皮质激素是首选药物,输血时应严格配血。     

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.     

Autoimmune Hemolytic Anemia as an Initial Symptom in Childhood Hodgkin'S Disease

The association of lymphoproliferative disorders with autoimmune disease has been well recognized in the adult population.¹ The most common correlation of autoimmune disease and malignancy is the presence of Coombs-positive hemolytic anemia and Hodgkin's disease with a reported incidence of 2.7%.² In childhood, however, during the last 20 years less than 10 cases of Hodgkin's disease and autoimmune hemolytic anemia (AIHA) have been reported.³     

Autoimmune hemolytic anemia in children

The clinical and hematological profile and treatment outcome of children with warm autoimmune hemolytic anemia (AIHA) were assessed using retrospective case record analysis. There were 26 (17 idiopathic; 9 secondary) patients with a median age of 11 years. Pallor (100%), fever (39%), and jaundice (59%) were the main presenting complaints. Jaundice was much more common in idiopathic (70%) compared to secondary (44%). Direct antiglobulin test was negative in 3 patients. Oral prednisolone produced remission in 81% patients. Four patients relapsed after a median period of 7 months (2 months to 2 year) after response. All responded to a second course of steroids in median 14 days. One child required cyclosporin A in addition. No correlation was found between response and parameters such as age, sex, jaundice, low pretreatment hemoglobin, reticulocyte count, total leukocyte count, platelet count, subtype of AIHA, and hepatosplenomegaly. Relapse correlated with increased duration between the onset of symptoms and treatment. This study indicates that oral prednisolone is an effective therapy for autoimmune hemolytic anemia. In refractory cases cyclosporine A may be useful.     

Autoimmune Hemolytic Anemia as an Initial Symptom in Childhood Hodgkin'S Disease

The association of lymphoproliferative disorders with autoimmune disease has been well recognized in the adult population.¹ The most common correlation of autoimmune disease and malignancy is the presence of Coombs-positive hemolytic anemia and Hodgkin's disease with a reported incidence of 2.7%.² In childhood, however, during the last 20 years less than 10 cases of Hodgkin's disease and autoimmune hemolytic anemia (AIHA) have been reported.³     

Exchange transfusion for severe autoimmune hemolytic anemia

A 10-year-old patient with severe autoimmune hemolytic anemia (AIHA) presented with heart failure. Hemoglobin levels remained dangerously low despite multiple blood transfusions and corticosteroid therapy. A two-volume exchange transfusion promptly restored and maintained satisfactory hemoglobin levels. An exchange transfusion should be considered in selected patients with AIHA when severe hemolysis results in life-threatening anemia, and repeated blood transfusions are unsuccessful in maintaining safe hemoglobin levels. Such an approach may be especially applicable in children in whom plasma-pheresis may not be suitable because of technical limitations.     

Rituximab for the treatment of post-bone marrow transplantation refractory hemolytic anemia in a child with Omenn's syndrome

Omenn's syndrome is a rare severe combined immunodeficiency that kills affected subjects before the end of the first year of life unless patients are treated with bone marrow transplantation (BMT). Unfortunately, post-BMT patients may develop autoimmune diseases, such as autoimmune hemolytic anemia (AIHA), which sometimes fails to respond to standard therapies. Rituximab is a chimeric, human, immunoglobulin G1/k monoclonal antibody specific for the CD20 antigen expressed on the surface of B lymphocytes. Rituximab is currently only labeled for treatment of B-cell lymphoproliferative disorders, such as B-cell non-Hodgkin's lymphoma and follicular lymphoma; however, it is also employed in the treatment of a variety of disorders mediated by auto-antibodies, such as AIHA and transplant-related autoimmune disorders. Herein, we describe the case of a 23-month-old male child with Omenn's syndrome, who had undergone BMT and was successfully treated with rituximab (375 mg/m(2) intravenously, weekly for three times) for refractory post-BMT hemolytic anemia. Our findings evidence that rituximab should be considered for treatment of post-BMT AIHA refractory to traditional therapy also in children with primary immunodeficiencies; furthermore, rituximab might represent a means to obtain remissions without the toxic effects associated with corticosteroid and immunosuppressive agents.