温冷双抗体型自身免疫性溶血误定血型1例分析

自身免疫性溶血性贫血(AIHA)是一种获得性免疫性疾病,根据抗体种类可分为温反应性抗体型和冷反应性抗体型。笔者工作中发现1例以冷抗体为主的温冷双抗体型自身免疫性溶血性贫血,造成血型鉴定及配血困难,报告如下。     

自身免疫溶血性贫血

自身免疫溶血性贫血（AIHA）是获得性溶血中最常见者。其中温抗体型占SO％，冷凝集素综合征15％，阵发性冷性血红蛋白尿5％。本病国外最低年发病率为1／80O00，女性稍多于男性，无种族差异。国内AIHA亦不少见，以温抗体型为主。1AIHA的分型AIHA红细胞上致敏成分是不均一的，其分型方法是继抗人球蛋白试验（Coombs）后一大进展。分型，即区分致敏红细胞上?免疫球蛋白的类别以及激活的补体。红细胞上结合的免疫球蛋白通常是IgG，少数为IgM，个案报告有单纯的IgA致敏的，迄今未发现红细胞被lgE或lgD致敏。IgG是引起温型AIHA的主…     

冷凝集素试验阳性的自身免疫性溶血性贫血实验室资料分析

自身免疫性溶血性贫血(AIHA)是一组B淋巴细胞功能异常、产生抗自身红细胞的抗体,使红细胞破坏增加而引起的贫血。国外报道,原发型AIHA占49％,其余为继发型,可继发于免疫系统疾病(系统性红斑狼疮、硬皮病等)、肿瘤细胞疾病(淋巴瘤、急性白血病等)及各种感染。依据自身红细胞抗体的特性可将AIHA分为温抗体型AIHA(红细胞与自身红细胞抗体结合的最佳反应温度为37℃)、冷抗体型AIHA(红细胞与自身红细胞抗体结合的最佳反应温度为4℃)和温冷双抗体型AIHA(在同一患者红细胞上既有温型自身红细胞抗体又有冷型自身红细胞抗体)。冷凝集素试验(CAT)用于检测患者体内是否     

自身免疫性溶血性贫血的诊断

自身免疫性溶血性贫血(autoimmune hemolytic anemia，AIHA)是一种获得性溶血性疾病，患由于免疫功能紊乱产生抗自身红细胞的抗体．与红细胞表面的抗原结合，或激活补体使红细胞加速破坏而致溶血性贫血。国外报道本病约占溶血性疾病患总数的1／3，最低年发病率为1／80000，国内AIHA亦不少见。该病以青壮年为多，女性多于男性。鉴于AIHA是临床上较常见、难根治的贫血．     

自身免疫性溶血性贫血36例临床分析

目的研究自身免疫性溶血性贫血(AIHA)的临床特征。方法对36例AIHA的临床资料进行回顾性分析。结果发病年龄以青壮年为主,且女性多于男性;36例中病因明确者19例(52.8%),其中系统性红斑狼疮6例(31.6%);免疫分型以IgG+C3型多见,余依次为C3型、IgG型;C3型溶血、贫血程度最轻,IgG+C3组次之,含IgA或IgM组溶血、贫血程度最严重;C3型对激素反应效果最好,IgG+C3组次之,含IgM或IgA组疗效最差。结论AIHA免疫分型与临床特征及治疗效果相关,可为临床治疗提供依据。     

164例Coombs试验阳性的自身免疫性溶血性贫血的临床研究

目的:研究自身免疫性溶血性贫血(AIHA)的临床特征,进一步指导治疗。方法:采用回顾性分析患者资料,率的比较采用精确概率法,均值比较采用t检验。结果:AIHA多发生在女性,继发性占49％,以胶原系统疾病为主,Coombs分型以IgG C_3型为主IgG IgM阳性的患者临床表现最严重,环孢霉素A(CsA)联用皮质激素组有效率为90.9％。结论:皮质激素仍为治疗AIHA的首选药物,合用CsA可提高疗效,缩短疗程。     

自身免疫性溶血性贫血患者临床特征和实验室指标分析

目的分析自身免疫性溶血性贫血(AIHA)患者临床特征及实验室指标检测结果。方法收集75例AIHA确诊患者临床资料,回顾性分析临床特征及实验室指标检测结果。结果 AIHA发病年龄以青壮年为主,女性患者所占比例高于男性,临床表现以贫血症状为主。多数患者Coombs试验阳性,IgG+C3d型多见,其次为IgG型和C3d型;总胆红素、乳酸脱氢酶、网织红细胞水平均高于正常参考值。结论分析患者临床特征和实验室指标可快速诊断及分型AIHA,为临床治疗提供可靠的依据。     

自身免疫性溶血性贫血抗体检测临床分析

自身免疫性溶血性贫血(AIHA)为自身抗体引起红细胞破坏的一种溶血性贫血。AIHA可分为三型：温抗体型AIHA、冷抗体型AIHA及阵发性寒冷性血红蛋白尿，其自身抗体分温性与冷性，而直接抗人球蛋白实验阳性为诊断温性抗体必备条件。笔应用广谱抗血清和特异性抗血清相结合的方法，共检测29例AIHA患，现报告如下。     

自身免疫性溶血性贫血的免疫分型及临床分析

目的 对64例自身免疫性溶血性贫血(AIHA)患者血液中红细胞上结合的抗体进行免疫分型,进一步指导临床治疗.方法 采用免疫分型技术及相关溶血性贫血系列检测法.结果 发病较多的是青壮年,女性多于男性,类型分布以IgG C3型多见,且贫血、溶血程度重,余依次为C3型、IgG型.结论 AIHA免疫分型可判定疾病的严重程度以及为临床治疗提供依据.     

血清稀释法在1例免疫性溶血性贫血患者输血中的应用

自身免疫性溶血性贫血(AIHA)是由于机体免疫功能紊乱产生抗自身红细胞抗体,导致自身红细胞破坏加速的一种溶血性贫血。根据抗体在机体内作用的最适温度将AIHA分为温抗体型与冷抗体型[1]。依据病因明确与否分为继发性和原发性两类[2]。AIHA患者体内常常检出一些抗体导致抗体筛查阳性及交叉配血困难,延误临床输血治疗时机。本科室结合患者病情,应用血清稀释法进行抗体鉴定和交叉配血试验,选择合适的血液给予及时的输血治疗,成功救治了1例极重度自身免疫性溶血性贫血患者,现报道如下。     

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　了解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　对治疗后完全缓解的 5 2例AIHA和Evans综合征患者随访 1～ 14年 ,了解其复发率 ,并采用同期病例对照法 ,比较不同因素与复发的相关性。结果　总复发率为 5 7.7% ;首次复发中位时间为缓解后 9个月 ;Coombs试验阴性型复发率为 30 .8% (13例中 4例复发 ) ,温抗体型复发率为 5 4 .0 % (2 4例中 13例复发 ) ,冷温双抗体及冷抗体型复发率为 86 .7% (15例中 13例复发 ) ,合并冷抗体患者复发率明显高于其他两型 (P <0 .0 5 ) ;抗体效价和≥ 10 0的患者复发率为 92 .9% (14例中 13例复发 ) ,抗体效价和 <10 0者复发率为 5 9.1% (2 2例中 13例复发 ) ,随着抗体效价升高 ,复发率明显增高 (P <0 .0 5 ) ;反复感染者易复发 ;加入环孢菌素A(CsA)的治疗方案较传统单用激素方案复发率明显降低 (P <0 .0 1) ;复发与激素、CsA的疗程密切相关 (P <0 .0 1)。结论　AIHA和Evans综合征复发率较高 ;分型施治、减少感染、加用CsA、延长疗程可减少复发。     

Mixed-type autoimmune hemolytic anemia: differential diagnosis and a critical review of reported cases

BACKGROUND: Autoimmune hemolytic anemia (AIHA) is usually classified as either warm or cold type. During the past few decades, mixed types (Mxs) have also been described in a number of cases (6%‐8% of AIHA), often without serologic data to support the diagnosis. In this study, we demonstrate that the incidence of Mx AIHA in our institution is extremely rare. STUDY DESIGN AND METHODS: Between August 1998 and August 2007, all in‐ and outpatients with detectable warm autoantibodies (WABs) were included in this study. Serologic testing was performed using standard techniques for the detection of red blood cell antibodies. RESULTS: From a total of 2194 patients with detectable WABs, only 2 patients (<0.1%) developed both WABs and cold agglutinins (CAs), which in the presence of clinical evidence of hemolytic anemia, satisfies the criteria for Mx AIHA. Only 1 of these patients, however, showed cold and warm hemolysis. Insignificant CAs at temperatures of not more than 24°C were found in 242 patients. CONCLUSION: There is evidence that the presence of CAs with high thermal amplitude and WABs may lead to confusion and misdiagnosis in some patients with AIHA. This study demonstrates that Mx AIHA is less common than previously reported.     

Aquired immune hemolytic anemias

Aquired immune hemolytic anemias are classified in autoimmune hemolytic anemia (AIHA) of warm type, cold agglutinine disease, paroxysmal cold hemoglobinuria, drug-induced immune hemolytic anemia, and paroxysmal nocturnal hemoglobinuria. The autoantibodies in AIHA of warm type react most strongly at 37 degrees C (warm autoantibodies). They are of the IgG, less commonly of the IgM and IgA classes. The cause of autoimmunization remains obscure in 50% of the patients (idiopathic AIHA). In the remaining cases, the AIHA is associated with other diseases. Corticosteroids are the mainstay of therapy, but most patients with AIHA of warm type require additional treatment with azathioprine or other drugs. Cold agglutinins are the cause of hemolysis in about 10% of patients with AIHA. Paroxysmal cold hemoglobinuria (Donath-Landsteiner) is less common and occur in children following infections. Drugs are the cause of hemolysis in about 10% of all cases with AIHA. The true incidence of alloimmune hemolytic anemias including neonatal immune hemolytic anemias is unknown. The paroxysmal nocturnal hemoglobinuria is rare. It is caused by complement activation due to aquired membrane defects.     

61例自身免疫性溶血性贫血患者血型血清学特征及输血疗效评估

本研究旨在分析自身免疫性溶血性贫血患者的血型血清学特征及红细胞不相容输注的疗效及安全性.通过回顾性分析特发性（21例）或继发性自身免疫性溶血性贫血患者（40例）血型血清学特征、输血不良反应发生情况,按照自身抗体类型、接受不同红细胞成分分别评价不相合输血疗效和安全性.结果表明：61例中单独IgM类冷自身抗体8例（13.1％）,单独IgG类温自身抗体50例（82.0％）,IgM冷自身抗体联合IgG温自身抗体3例（4.9％）,合并存在同种抗体18例（29.5％）;其中36例自身免疫性溶血性贫血患者在排除同种抗体干扰情况下共进行不相合红细胞输注113次,总有效率56.6％,总部分有效率15.1％,总无效率28.3％.按输注红细胞成分差异分为ABO同型非洗涤红细胞组和O型洗涤红细胞组,ABO同型非洗涤红细胞组有效率57.6％,部分有效率13.0％,无效率29.4％;O型洗涤红细胞组有效率53.6％,部分有效率21.4％,无效率25.0％,两组输注疗效比较差异无显著性（P＞0.05）.按患者自身抗体类型分为IgM冷自身抗体组和IgG温自身抗体组,其中IgM冷自身抗体组有效率46.2％,部分有效率30.8％,无效率23.0％;IgG温自身抗体组有效率56.7％,部分有效率13.4％,无效率29.9％,两组输注疗效比较差异无显著性（P＞0.05）.所有输血病例均无溶血性输血反应发生.结论：对于重度贫血的自身免疫性溶血性贫血患者在排除同种抗体干扰的情况下,采用同型非洗涤红细胞或O型洗涤细胞输注都是相对安全的,两种方式疗效差异无显著性,选择同型非洗涤红细胞输注更方便、快捷,还能避免O型红细胞的过度使用.     

Serologic findings in autoimmune hemolytic anemia associated with immunoglobulin M warm autoantibodies

BACKGROUND: Autoimmune hemolytic anemia (AIHA) associated with immunoglobulin M (IgM) warm autoantibodies is unusual but often severe, with more fatalities than other types of AIHA. Diagnosing this type of AIHA can be difficult because routine serologic data are not always informative.
STUDY DESIGN AND METHODS: Forty-nine cases of IgM warm AIHA in 25 years were studied by serologic methods.
RESULTS: Routine direct antiglobulin tests (DATs) detected red blood cell (RBC)-bound C3 in 90 percent of cases (65% had C3 but no immunoglobulin G [IgG] on their RBCs) and IgG in 24 percent. IgM was detected on 29 of 47 (62%) patients' RBCs; RBC-bound IgM was detected in 14 of 47 cases by a tube DAT method and in an additional 15 of 21 (71%) cases using fluorescein isothiocyanate anti-IgM and flow cytometry. Eighty-one percent of eluates from patients' RBCs reacted. Warm autoagglutinins were present in 94 percent of serum samples; untreated and enzyme-treated RBCs were hemolyzed at 37°C by 13 and 65 percent of serum samples, respectively. Most agglutinins were optimally reactive at 30 to 37°C. Patients' RBCs were spontaneously agglutinated in 78 percent of cases; washing with 37°C saline or treating RBCs with dithiothreitol resolved this problem. Clear specificity of autoantibody was defined in 35 percent of serum samples.
CONCLUSION: IgM warm AIHA can be confused with cold agglutinin syndrome and "mixed/combined"-type AIHA; a serologic workup by a specialist reference laboratory can help with the diagnosis.     

Réanimation et cytopénies auto-immunes. Diagnostic et modalités de prise en charge

Immune thrombocytopenia (ITP) and autoimmune haemolytic anemia (AIHA) are the two main autoimmune cytopenias in adults. AIHA includes AIHA associated with warm autoantibodies and cold agglutinin-mediated anemia (CAA). Treatment of the most severe forms of ITP is based on high-dose steroids and intravenous immunoglobulins (IVIg). Platelet transfusions are indicated only for life-threatening bleedings. High-dose steroids represent the first-line treatment of warm AIHA. Red blood cell transfusions are effective for symptomatic anemia. Splenectomy should only be performed in the exceptional cases refractory to medical treatment. In CAA, transfusion of warmed packed red blood cells is the unique emergent therapy. Steroids are not effective and should be avoided. Anti-CD20 antibodies represent a promising therapy for warm AIHA and CAA and should be administered if first-line treatment has failed.     

Autoimmune hemolytic anemia

Diagnosis of autoimmune hemolytic anemia (AIHA) requires both serologic evidence of an autoantibody and hemolysis. Based on the characteristic temperature reactivity of the autoantibody to red cell membranes, AIHA is classified into warm AIHA or cold AIHA (cold agglutinin disease and paroxysmal cold hemoglobinuria). Sensitized RBCs are destructed by intravascular and/or extravascular hemolysis. On the basis of etiology, AIHA are classified as idiopathic or secondary. The common cause of secondary AIHA is lymphoproliferative disorders, autoimmune diseases, and infections. The first line therapy of patients with warm AIHA is glucocorticoids and primary treatment for cold AIHA is avoiding cold exposure. The other standard treatments include splenectomy and immunosuppressive drugs. Recently, rituximab, a monoclonal anti-CD20 antibody, has been used in refractory AIHA with excellent responses.     

Early-onset autoimmune hemolytic anemia after cladribine therapy for Waldenström's macroglobulinemia

BACKGROUND: Purine nucleoside analogs are a class of antineoplastic drugs with potent lymphotoxicity against T and B lymphocytes, causing prolonged lymphopenia and linked to delayed immune complications such as opportunistic infections and more recently autoimmune hemolytic anemia (AIHA), seen mostly in patients with chronic lymphocytic leukemia (CLL). A characteristic temporal relation between fludarabine therapy and the appearance of a warm‐reactive immunoglobulin G (IgG)‐mediated AIHA in patients with CLL has been observed and, in some, the AIHA has been fatal. Whether both fludarabine and cladribine cause AIHA is uncertain because AIHA is commonly seen in patients with CLL without the use of these drugs. In contrast, AIHA is encountered in Waldenström's macroglobulinemia (WM) much less frequently, and the autoantibody is usually cold‐reactive and IgM‐mediated. In a few reported cases of AIHA arising in patients with WM after cladribine therapy, there was a latency of 24 to 60 months between therapy and the onset of AIHA, three of which were warm‐reactive and IgG‐mediated. CASE REPORT: A warm‐reacting IgG red cell autoantibody and evidence of hemolysis detected 1 month after completing cladribine therapy for WM, with warm antibody AIHA developing 4 months later, are described. CONCLUSIONS: Cladribine, like fludarabine, is possibly able to produce this complication during or early after therapy. Because the use of purine analogs is becoming increasingly common, it is important to have an awareness of the complications that can arise during and after treatment. Further observations of warm AIHA during cladribine therapy are needed to establish it as a distinct complication.     

Resolution of serologic problems due to cold agglutinin mediated autoimmune hemolytic anemia and its transfusion decision

BackgroundAutoimmune hemolytic anemia (AIHA) is a rare disease characterized by hemolysis caused by autoantibodies against erythrocyte surface antigen. These antibodies can be classified as warm, cold, or mixed types.MethodsWe report two cases of cold agglutinin disease (CAD), which were eventually diagnosed owing to blood group discrepancy. Resolution was achieved after washing the red blood cells (RBCs) with warm saline and absorbing the autoantibodies at 4°C with the washed RBCs. We also assessed the patient''s condition and discussed the strategy of blood transfusion.ResultsThe first case occurred after postoperative chemotherapy for rectal cancer, and the other manifested with anemia from the outset. Direct antiglobulin tests were positive and revealed autoantibodies against C3d only. Cold agglutinin titration was performed, and the titers of both were 1:1024. Eventually, the patient''s condition stabilized without blood transfusion.ConclusionThe serological discrepancies observed in the blood transfusion department can successfully guide blood transfusion decisions in cases of CAD.     

Production of human warm-reacting red cell monoclonal autoantibodies by Epstein-Barr virus transformation

Monoclonal antibody technology has been used in both murine and human systems to produce a variety of antibodies that react with the human red cell (RBC). RBC monoclonal autoantibodies have been obtained from animal models of autoimmune hemolytic anemia (AIHA), but to date no warm-reactive monoclonal autoantibodies have been generated from human B cells. Using the Epstein-Barr virus (EBV) transformation method, clones of RBC autoantibodies were generated from two patients with AIHA. These antibodies reacted preferentially at 37 degrees C, agglutinated or bound to a variety of different RBC phenotypes, and were IgM in nature. The serologic reactivity of one clone showed a relative specificity to e+ RBCs that was similar to that seen in the patient's serum. These results are the first to demonstrate that warm-reactive RBC autoantibodies can be obtained from patients with AIHA using the technique of EBV transformation, and they further substantiate the existence of warm-reactive IgM RBC autoantibodies in the spectrum of warm AIHA.