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。     

33例获得性免疫性溶血性贫血的实验室结果分析

目的探讨实验室检测结果分析对获得性免疫性溶血性贫血诊断价值。方法33例获得性溶血性贫血,应用常规的实验方法。结果24例温抗体型自身免疫性溶贫(WAIHA),直接抗人球蛋白试验(Coombs试验)阳性。3例阵发性睡眠性血红蛋白尿(PNH),酸化血清溶血试验(Hams试验)阳性。1例冷凝集素综合征(CAS),冷凝集素滴度明显增高。结论应用最常规实验方法来诊断鉴别获得性免疫性溶血性贫血,是非常重要的,其特点快速,简便,成本低,便于实验室推广应用。     

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。     

直接抗人球蛋白试验阳性的类型鉴别及临床意义

目的　通过直接抗人球蛋白试验 (DAT)阳性的类型鉴别 ,有助于临床对自身免疫性溶血性贫血 (AIHA)的正确诊断 ;对血库交叉配血不合的处理有指导意义。方法　DAT阳性标本分AIHA组和非AIHA组 ,进行免疫分型 ,然后把IgG或IgG C3的标本进行放散试验 ,利用放散液与标准抗体筛查细胞进行间接抗人球蛋白试验 (IAT)。结果　AIHA组患者的放散液均同标准抗体筛查细胞反应 ,非AIHA组除了 1例SLE患者外均为阴性反应。结论　DAT阳性患者可以分为两类 :自身抗体引起和免疫复合物引起     

IgG型抗Ce联合抗体致自身免疫性溶血性贫血2例

2009年3-4月湖北省襄樊市中心血站分别接到市中医院和市中心医院的自身免疫性溶血性贫血患者标本各1例.通过血清学试验,笔者发现这2例患者的自身抗体均为IgG型抗-Ce联合抗体.     

骨髓单个核细胞Coombs分型试验在衄细胞减少症中的临床意义

目的 观察骨髓单个核细胞Coombs分型试验对全血细胞减少症的临床意义。方法 对51例全血细胞减少症患者进行骨髓单个核细胞Coombs分型试验检测，骨髓单个核细胞Coombs分型试验阳性患者给予泼尼松或加用环孢素A治疗。结果 51例血细胞减少患者，骨髓单个核细胞Coombs分型试验阳性40例，阴性11例。阳性患者中，单纯IgG阳性16例，单纯C3阳性4例，IgG＋C3阳性4例，单纯IgM阳性4例，IgG＋IgA阳性4例，抗IgG＋IgM阳性8例。泼尼松或加用环孢素A治疗有效率87．5％。结论 骨髓单个核细胞Coombs分型试验对血细胞减少症患者的鉴别诊断和指导治疗有重要的作用。     

ABC-ELISA检测AIHA患者EAIgG的结果分析

目的　探讨亲和生物素化酶复合物 酶联免疫吸附试验 (ABC ELISA)法定量检测红细胞结合IgG(EAIgG)在自身免疫性溶血性贫血 (AIHA)中的诊断价值。 方法　采用ABC ELISA法定量检测EAIgG。共检测 36名正常对照、38例AIHA患者 (其中 6例Coombs′试验阴性的AIHA患者 )的EAIgG值 ,并同时与直接抗人球蛋白试验 (DAT)结果比较。结果　AIHA患者的EAIgG值显著增高 ,与正常对照组相比 ,差异有显著性 (P <0 .0 0 1) ;6例Coombs′试验阴性的AIHA患者EAIgG均值 (3.93± 1.77)fg/RBC ,Coombs′试验阳性的AIHA患者EAIgG均值 (2 .89± 1.72 )fg/RBC ,两者相比差异无显著性 (P >0 .0 5 )。 结论　ABC ELISA法定量检测EAIgG有助于AIHA的诊断 ,特别对Coombs′试验阴性的AIHA患者更具确诊价值。     

微柱凝胶卡式法Coombs试验在自身免疫性溶血性贫血诊断中的应用

目的 采用微柱凝胶卡式检测方法进行直接Coombs试验,探讨其在自身免疫性溶血性贫血( AIHA)诊断中的临床意义.方法 对临床怀疑为AIHA的128例患者标本同时用试管法和微柱凝胶卡式法进行直接Coombs试验,对两种检测方法进行比较.检测患者同期血红蛋白水平、网织红细胞比例、胆红素及游离血红蛋白水平,比较不同型别患者间的差异.结果 应用微柱凝胶卡式法进行直接Coombs试验阳性检出率为88.4％,试管法阳性检出率为37.7％,患者红细胞包被不同免疫球蛋白时溶血程度不同.结论 微柱凝胶卡式法较试管法敏感,操作简单,易标准化,结果判读客观,可长期保存.对AIHA的诊断具有临床意义.     

39例Coombs试验阳性自身免疫性溶血性贫血临床分析

目的对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.方法采用上海市红十字中心生产的三种抗血清,进行直接抗人球蛋白试验(DAT),结合相关溶血系列检查.结果发病年龄以青壮年为主且女性多于男性.类型分布以IgG C3型多见,且溶血、贫血程度重,余依次为C3型、IgG型.结论 AIHA免疫分型可判断疾病的严重程度以及为临床治疗提供依据.     

ABC-ELISA检测AIHA患者EAIgG的结果分析

目的探讨亲和生物素化酶复合物-酶联免疫吸附试验(ABC-ELISA)法定量检测红细胞结合IgG(EAIgG)在自身免疫性溶血性贫血(AIHA)中的诊断价值.方法采用ABC-ELISA法定量检测EAIgG.共检测36名正常对照、38例AIHA患者(其中6例Coombs′试验阴性的AIHA患者)的EAIgG值,并同时与直接抗人球蛋白试验(DAT)结果比较.结果 AIHA患者的EAIgG值显著增高,与正常对照组相比,差异有显著性(P<0.001);6例Coombs′试验阴性的AIHA患者EAIgG均值(3.93±1.77)fg/RBC,Coombs′试验阳性的AIHA患者EAIgG均值(2.89±1.72)fg/RBC,两者相比差异无显著性(P>0.05).结论 ABC-ELISA法定量检测EAIgG有助于AIHA的诊断,特别对Coombs′试验阴性的AIHA患者更具确诊价值.     

Clinical application of a flow cytometric direct antiglobulin test

BACKGROUND: The clinical application of flow cytometric direct antiglobulin test (FC-DAT) has rarely been evaluated for patients with various diseases including immune and nonimmune hemolytic anemia.
STUDY DESIGN AND METHODS: Blood samples from 380 patients with a variety of diseases were studied using the tube direct DAT and FC-DAT. The results of tube DAT and FC-DAT were compared. The predictive values of DAT for hemolysis were evaluated.
RESULTS: Of 57 patients with autoimmune hemolytic anemia (AIHA), 6 of the 17 with a negative tube DAT (immunoglobulin G [IgG]) had a positive FC-DAT (IgG) and 23 of the 36 patients with a negative tube DAT (complement 3d [C3d]) had a positive FC-DAT (C3d). In 57 patients with AIHA, the incidence of positive results of FC-DAT (IgG) and tube DAT (IgG) were similar (42 positive vs. 40 positive); but in 323 patients without AIHA, the incidence of positive FC-DATs (IgG) was higher than that of tube DAT (IgG; 47 positive vs. 9 positive). The higher incidence of positive FC-DAT (C3d) than that of tube DAT (C3d) was seen in patients with AIHA (42 positive vs. 21 positive) as well as in patients without AIHA (61 positive vs. 5 positive). Both DAT (IgG) and DAT (C3d) positive has highest positive predictive value for hemolysis, followed by DAT (IgG) alone positive and DAT (C3d) alone positive.
CONCLUSIONS: FC-DAT is a complementary test for diagnosing AIHA. There is a synergistic effect of the red blood cell–bound IgG and complement in predicting hemolysis.     

84例自身免疫性溶血性贫血IgG抗体亚型与临床意义的分析

目的　研究温抗体型自身免疫性溶血性贫血ＩｇＧ亚型及其临床意义。方法　回顾性分析８４ 例患者ＩｇＧ亚型与临床特点的关系。结果　８４ 例患者中ＩｇＧ１ ＋ＩｇＧ３ ＋ Ｃ３ｄ 型占４５．０％ ,ＩｇＧ１ ＋ＩｇＧ３型占１５ ．５％ ,ＩｇＧ１ ＋ Ｃ３ｄ型占１１．０％ ,Ｃ３ｄ型占１０ ．７％ ,ＩｇＧ１ 型占９．５％ ,ＩｇＧ３ ＋ Ｃ３ｄ占８．３ ％ 。各型中均以女性为主,合计占６９ ．５％ 。ＩｇＧ３ 阳性各型的临床表现相似,各项临床指标异常最严重;ＩｇＧ１ 阳性的各型次之;Ｃ３ｄ 型最轻。Ｈｂ＜６０ ｇ／Ｌ、总胆红素＞ ４０ μｍｏｌ／Ｌ、ＦＨｂ＞ ６０ ｍｇ／Ｌ的患者百分率三组间比较,差异有显著性（Ｐ＜０ ．０５） 。ＩｇＧ１ 型与ＩｇＧ３ 型都随Ｃｏｏｍｂｓ 试验积分增高,其临床表现及溶血程度加重。ＩｇＧ３ 阳性的患者治疗有效率为６８ ．２％ ,ＩｇＧ１ 阳性患者有效率为１００％ 。结论　ＩｇＧ 亚型中以ＩｇＧ１ ＋ＩｇＧ３ 为主。ＩｇＧ３ 阳性的患者临床表现及溶血程度严重,治疗效果差     

Influence of immunohematological markers on severity of in vivo hemolysis in human warm autoimmune haemolytic anemia

Autoantibody production in autoimmune haemolytic anemia (AIHA) is the result of the loss of self-immunological tolerance of the host. Here we investigated the various immunohematological markers that may influence the severity of in vivo hemolysis in warm AIHA (WAIHA). Complete direct antiglobulin test (DAT) evaluation and immunohematological characterization were performed in 247 patients of WAIHA following departmental protocols. Clinical and laboratory details of patients were obtained from patient file. The median age of WAIHA patients was 47 years with a female preponderance. Lymphoproliferative diseases were the major underlying causes of secondary WAIHA. The mean haemoglobin (Hb) and reticulocyte count (Retic) were 6.43 gm/dL and 7.58% respectively. Single autoantibody bound to red cells was investigated in 151 patients. The main IgG subclass was IgG1. Multiple autoantibodies like IgG+ C, IgG+IgA and IgG+IgA+C were found in 87 (35.2%) patients. Free autoantibodies were observed in 112 patients with a median indirect antiglobulin test (IAT) reactivity of 2+. Derangement of haematological and biochemical values was statistically significant with increase in DAT reactivity, presence of multiple autoantibodies on red cells, coating of red cells by IgG3 or multiple IgG subclass, higher DAT dilution and increasing IAT reactivity. We conclude that several important but simple immunohematological parameters may influence the degree of in vivo hemolysis in WAIHA. Since a set of common haematological and biochemical test determines the severity of in vivo hemolysis therefore a comprehensive clinical and immunohematological evaluation is advisable for a correct diagnostic and therapeutic workup of WAIHA.     

首发症状Coombs试验阴性自身免疫性溶血性贫血的非何杰金淋巴瘤(英文)

非何杰金淋巴瘤(NHL)伴发自身免疫性溶血性贫血(AIHA)或AIHA发生在NHL诊断之前或治疗过程中已有不少报道,但以Coombs试验阴性AIHA为首发症状的NHL鲜有报道。在此,本文报道1例1.5年反复溶血发作的Coombs试验阴性AIHA后合并NHL的患者。患者女性,69岁,根据病史和实验室检查诊断为Coombs试验阴性AIHA,给予强的松治疗后血红蛋白恢复正常,停药后溶血反复复发2次,强的松治疗仍有效。第3次复发时强的松治疗无效,并出现颈部淋巴结肿大,病理检查确诊为NHL。给予6个疗程的CHOP方案化疗,NHL治愈,但溶血仍不能控制,给予小剂量利妥昔单克隆抗体(rituximab,RTX)治疗后,溶血很快停止,此后给予3次小剂量RTX进行维持治疗,NHL和AIHA呈持续缓解状态。结论:本文报告了一例十分罕见的非何杰金淋巴瘤,其发病时的主要临床症状为Coombs阴性自身免疫性溶血性贫血。     

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.     

Clinical features and treatment of primary autoimmune hemolytic anemia in childhood

Background and aim

Autoimmune hemolytic anemia (AIHA) is characterized by autoimmune destruction of erythrocytes. In this retrospective study, the clinical, laboratory features and treatment responses of patients with primary AIHA were evaluated.

AIHA患者外周血Tfh/Treg细胞的检测及临床意义

目的 研究自身免疫性溶血性贫血(AIHA)患者外周血CD4⁺CXCR5⁺T细胞(Tfh)及CD4⁺CD25⁺FoxP3⁺T细胞(Treg)数量和比例及相关细胞因子的变化,探讨其在AIHA发病机制中的作用。方法 选择30例AIHA患者,分离外周血单核细胞(PBMC),采用流式细胞术(FCM)检测Tfh及Treg细胞的数量,ELISA法测定血清中IL-21,IL-10的水平及IL-21/IL-10比值变化,与同期选择的健康对照组比较。结果 AIHA患者PBMC中Tfh细胞及IL-21因子的水平均高于健康对照组,而Treg细胞及IL-10水平低于对照组,差异均有统计学意义(P<0.05),Tfh/Treg与IL-21/IL-10比值均升高(P<0.05),且IL-21/IL-10比值变化与患者病情呈正相关(P<0.05)。结论 Tfh及Treg细胞的比例异常及相关细胞因子IL-21/IL-10比值变化可能参与AIHA患者的免疫紊乱机制。     

P31Mixed‐Type Autoimmune Haemolytic Anemia (AIHA)

The diagnosis of mixed‐type AIHA is based on demonstrating the presence of ‘warm’ IgG auto‐antibody and high thermal amplitude ‘cold’ IgM auto‐antibody reacting at or >30 °C. Mixed‐type AIHA is uncommon. In general, patient with mixed‐type AIHA responds to steroids and reported to be associated with SLE and lymphoma. Awareness of this condition is important as management may be different from either treating warm AIHA or cold haemmagglutinin disease (CHAD). We report two unusual cases of mixed‐type AIHA. Management and outcome of these cases are discussed. An 88‐year‐old, patient was admitted with low Hb and raised bilirubin. He was already on steroids 15 mg daily for joint pain. In view of the strong red cell agglutination in the blood film (CHAD) was suspected initially and were investigated to exclude the secondary causes of CHAD. Three days later Hb dropped to 6.6 g dL^?1. Samples were forwarded to the reference laboratory. The DAT was positive with IgG, IgM and C3d. Serological evidence of mixed‐AIHA was confirmed at the reference laboratory. In view of the ongoing haemolysis, steroids were increased to 40 mg daily, and transfusion was given, together with intravenous immunoglobulin (IVIg). Haemolysis ceased and the patient was discharged on decreasing doses of steroid. This case was unusual as the patient developed AIHA while on steroids medication. An 85‐year‐old patient, was admitted with Hb level of 5.7 g dL^?1 and evidence of haemolysis. Laboratory investigation confirmed mixed‐type AIHA. A CT scan showed splenomegaly, with small volume lympadenopathy, which were too small to biopsy. A bone marrow biopsy showed no evidence of lymphoma infiltration. The patient was treated with steroids, IVIg, followed by chemotherapy (cyclophosphamide, vincrestine and steroids) with no response. Rituximab (357 mg m^?2 weekly for 2 weeks) resulted in no response. The patient received 33 units of red cells over a 9 week period. She underwent splenectomy with resolution of haemolysis and Hb sustained at 11 g dL^?1. Splenic biopsy revealed T cell angioimmunoblastic Non‐Hodgkins lymphoma. Splenectomy should be considered in resistant mixed‐type AIHA.