儿童自身免疫性溶血性贫血治疗进展

自身免疫性溶血性贫血是由各种原因导致机体免疫功能紊乱,产生可与红细胞自身抗原反应的自身抗体和(或)补体,导致红细胞破坏增多并超过骨髓造血的代偿能力。儿童多急性起病,临床表现与发病机制相关。糖皮质激素作为该病的一线治疗,多数患儿对其反应良好。部分患儿因存在激素依赖,激素耐药,或因抗体类型不同导致病程反复,常需采取二线治疗...     

儿童自身免疫性溶血性贫血的治疗进展

儿童自身免疫性溶血性贫血（autoimmune hemolyticanemia,AIHA）通常表现为急性,病程自限性,并预后良好,80％患儿对于激素敏感。但部分患儿则为慢性、难治陆病程,特别是2岁以下或10岁以上患儿,易激素耐药或依赖,从而出现生长发育缓慢、内分泌紊乱等问题。本文将目前国内外治疗AIHA方法及进展做一总结,期望对于临床工作有所帮助。     

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     

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。     

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%.     

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。     

小儿自身免疫性溶血性贫血76例临床分析

目的探讨小儿自身免疫性溶血性贫血(AIHA)的病因及治疗。 方法对1990年1月至2004年10月山东省立医院住院76例AIHA患儿,进行回顾分析。 结果76例中原发18例,继发58例。继发者中继发于上呼吸道感染者27例。临床表现主要为面色苍白、发热、黄疸、尿色加深及肝脾肿大,Coombs试验阳性61例(61/76,80.3%)。原发性者治愈和好转率低于继发性者。 结论肾上腺皮质激素仍为治疗AIHA的首选药物。在单用激素治疗疗效欠佳时,加用丙种球蛋白及丹那唑治疗可缩短病程,并可减轻激素的副反应。对于AIHA患儿尽量不予输血,需要输血时应严格配血。原发病的治疗也是提高疗效的关键。     

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%。     

Pernicious anemia associated with autoimmune hemolytic anemia and alopecia areata

We report a 16-year-old male with a combination of pernicious anemia, auto-immune hemolytic anemia and alopecia areata. Autoimmune hemolytic anemia coexisted with pernicious anemia but was diagnosed only when the anemia failed to respond to cobalamin therapy. Alopecia areata occurred 9 years later.     

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.     

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??Abstract??Autoimmune hemolytic anemia ??AIHA?? is an uncommon acquired immune disorder. The classification of AIHA is based on the pattern of the direct antiglobulin test and on the immunochemical properties of the autoantibody?? but also on the presence or absence of an underlying condition or disease. Different type of AIHA has different treatment and outcome. Treatment for AIHA has long been empirical and first line therapy is corticosteroids?? especially for warm AIHA ??wAIHA??.For relapsed or refractory AIHA?? second-line therapy??such as rituximab??may be a good alternative and has been more reported in childhood patients. In this article?? the classification and the recent progress in therapies for AIHA are discussed to provide treatment recommendations for pediatricians.     

Bortezomib for autoimmune hemolytic anemia after intestinal transplantation

AIHA is rare in the general population and associated with a mortality of 8%. In contrast, AIHA occurs in up to 12.2% of cases after intestinal transplantation and is associated with mortality up to 50%. Treatment entails a “step‐up” approach including corticosteroids, IvIg, plasmapheresis, and rituximab. However, AIHA after transplantation often is refractory to this strategy, contributing to a poor outcome. We describe a child with microvillous inclusion disease who developed AIHA 1 year after multivisceral transplantation that was refractory to standard therapy and was subsequently treated with bortezomib.We observed remission of AIHA within 1 week after the start of bortezomib. Bortezomib was associated with transient diarrhea, leucopenia, and elevated liver enzymes. Three years later, he remains in remission without important complications. Published data on bortezomib for autoimmune cytopenias outside SOT are discussed. This is the first report to support bortezomib as an important therapeutic alternative for AIHA after SOT. The occurrence and treatment of AIHA after SOT, and specifically intestinal transplantation, should be the subject of future registry studies to collect additional experience and explore the optimal therapeutic approach.     

Disseminated intravascular coagulation due to IgM-mediated autoimmune hemolytic anemia

Disseminated intravascular coagulation (DIC) due to red cell hemolysis has been previously attributed to transfusion-related hemolytic reactions, but not to autoimmune hemolytic anemia. We report a case of DIC in a child with complement-fixing IgM-mediated cold-agglutinin autoimmune hemolysis, which resulted in arterial thrombosis and gangrene of the upper and lower extremities.     

Abdominal tuberculosis with autoimmune hemolytic anemia

An eight-year-old male child presented with progressive distension of abdomen, fever, pallor and jaundice with a history of tubercular contact. Investigations were suggestive of abdominal tuberculosis with autoimmune hemolytic anemia. The child responded well to a course of oral steroids with antitubercular treatment. A literature search did not reveal any previous case report of an association between tuberculosis and autoimmune hemolytic anemia.     

Sirolimus rescue for tacrolimus-associated post-transplant autoimmune hemolytic anemia

Autoimmune hemolytic anemia (AIHA) has been reported to occur after renal transplantation, and typically does so in the first few weeks post-transplant. We report on a 3-yr-old child who developed cold AIHA nearly 1 yr after an ABO identical, living donor renal transplant from his mother. Numerous transfusions, pulse steroids, repeat plasma exchange treatments, and IVIG were unsuccessful. Nearly 3 wk into his illness, tacrolimus was changed to cyclosporine, and then to sirolimus, and resulted in a prompt response. He currently has a normal renal function and a normal hemoglobin level on sirolimus monotherapy.     

Giant cell hepatitis with autoimmune hemolytic anemia in a Korean infant

Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AHA) is a very rare disease characterized by early onset and severe clinical manifestations, including immune hemolytic anemia and hepatitis with cholestasis. The prognosis is poor despite aggressive immunosuppressive therapy. We report here the first case of GCH with AHA in East Asia. A 2‐month‐old boy was admitted with jaundice. Blood test indicated abnormal liver function and low hemoglobin. Direct Coombs test and several autoantibodies associated with liver disease were positive, and liver biopsy was consistent with GCH. He was treated with prednisolone and ursodeoxycholic acid, and at the time of writing was in clinical and biochemical remission after prednisolone was stopped.     

IgA-mediated autoimmune hemolytic anemia in an infant

Autoimmune hemolytic anemia (AIHA) is characterized by the presence of autoantibodies, most frequently of the IgG isotype, directed against erythrocyte surface antigens. The direct antiglobulin test (DAT) is the critical laboratory test for the diagnosis of AIHA, but is negative in 3-11% of cases. In these cases of DAT negative AIHA, a wider spectrum of clinical data including more specialized testing for erythrocyte autoantibodies may be required. We describe the unique and challenging case of an infant with corticosteroid-responsive, DAT negative AIHA, in which specialized gel card testing identified an isolated IgA autoantibody on the erythrocyte surface.     

Rubella infection with autoimmune hemolytic anemia

A 4-yr-old boy developed autoimmune hemolytic anemia after rubella infection and clinical manifestations cleared up after decrease in rubella specific IgM titer without any specific therapy.