溶血性贫血的诊断治疗现况

溶血性贫血的诊断治疗现况中国医学科学院北京协和医院血液科李蓉生溶血性贫血是由于红细胞的破坏加速而骨髓造血功能代偿不足时发生的贫血。临床上表现为贫血、黄疸及肝脾肿大，依据其发病原因分为红细胞内在异常（包括红细胞膜异常、酶缺陷及球蛋白生成障碍）或红细胞外...     

后天获得性溶血性贫血

溶血性贫血是一种常见的贫血类型,根据其病因和发病机制可将之分为先天性和后天获得性两种.后天获得性溶血性贫血病因复杂、类型多样,包括免疫性溶血性贫血,感染所致的溶血性贫血,化学、物理、生物毒素所致的溶血性贫血,机械创伤和微血管病性溶血性贫血,阵发性睡眠性血红蛋白尿症.只有掌握正确的诊断思路,应用恰当的检测手段才能明晰其类型,做到准确诊断、正确治疗.     

溶血性贫血诊断进展

溶血性贫血(hemolytic anemia,HA)是指红细胞遭破坏而寿命缩短,超过骨髓造血代偿时发生的一类贫血。临床上有急性或慢性HA的症状和体征,实验室检查有贫血、红细胞破坏增多、骨髓代偿性增生及红细胞缺陷或寿命缩短的证据。近年来由于各种检测技术的发展,特别是流式细胞术和分子生物学手段的应用,使HA的诊断取得了长足进步。现以血管外溶血中常见的自身免疫性溶血性贫血(AIHA)和血管内溶血中常见的阵发性睡眠性血红蛋白尿(PNH)为例,就其诊断进展进行阐述。     

小儿溶血性贫血的临床观察

目的以临床分析为基础,总结小儿急性溶血性贫血（简称急性溶贫）的临床特征。方法对所收治的83例急性溶贫患儿的病因、临床表现、实验室检查、治疗及转归等情况进行回顾性分析。结果急性溶贫患儿男性多于女性,男：女比为2．2：1;发病时间：每月均有发病,春季发病最多;病因以红细胞G6PD缺乏症最多（32．53％）,其次为各种诱因导致地中海贫血溶血急性加剧（31．33％）;主要诱因是服用蚕豆或呼吸道感染临床以中、重度贫血多见（62．65％）;平均住院天数5．80d。98．8％的病人病情稳定出院。结论急性溶贫患儿以男性居多,G6PD缺乏症、地中海贫血急性加剧为主要病因;临床上以中、重度贫血多见,诊断处理及时,近期预后良好。     

G6PD缺乏对地中海贫血产前筛查血液学指标的影响

目的 探讨葡萄糖6磷酸脱氢酶(G6PD)缺乏对地中海贫血基因携带者平均红细胞容积(MCV)、红细胞平均血红蛋白含量(MCH)的影响.方法 2006-06-2008-12产前筛查的孕妇及其丈夫中行地中海贫血基因分析确诊为地中海贫血基因携带者,常规行G6PD酶活性测定及血细胞分析.分为G6PD缺乏组及G6PD正常组,比较两...     

广西地中海贫血不同基因型血常规参数MCV MCH及血红蛋白电泳检测结果的差异分析

目的通过对广西地区几种常见地贫基因型的血常规参数,即平均红细胞体积(mean corpuscular volume,MCV)、平均红细胞血红蛋白量(mean corpuscular hemoglobin,MCH)和血红蛋白电泳检测结果进行对比,探索数值差异与基因型之间的相关性。方法采用Gap-PCR法进行α地贫(缺失型)基因检测,采用反向斑点杂交法进行α地贫(非缺失型)和β地贫的基因检测;使用全自动血细胞分析仪进行血细胞分析;使用全自动毛细管电泳仪测定血红蛋白各项指标。结果研究发现静止型α地贫中α~(QS)α/αα基因型的MCV、MCH最低;轻型α地贫中--~(SEA)/αα基因型血液学表现最为严重,差异有统计学意义(P0.05)。轻型α地贫和中间型α地贫Hb A2减少,而轻型β地贫及复合型地贫Hb A2增加,差异有统计学意义(P0.05)。Hb CS带仅出现在α~(CS)α/αα、-α3.7/α~(CS)α、--~(SEA)/α~(CS)α等含有CS点突变的基因型中,但α~(CS)α/ααβ~(CDs41-42)/β~N却未表现出Hb CS带,差异有统计学意义(P0.05)。在中间型α地贫(血红蛋白H病)中,--SEA/α~(CS)α、--~(SEA)/-α4.2和--~(SEA)/-α3.7三种基因型血红蛋白电泳结果均呈现不同程度的Hb-H带,而--SEA/αWSα中却并未发现血红蛋白电泳Hb-H带,差异有统计学意义(P0.05)。复合型地贫(轻型α+轻型β)同复合型地贫(静止型α+轻型β)相比,其贫血程度较轻,差异有统计学意义(P0.05)。结论 MCV、MCH和血红蛋白电泳的结果与基因表型有一定的相关性,对于临床医师在进行地贫遗传咨询时有很好的指导意义,对于地贫防控也具有重要意义。     

青霉素致免疫溶血性贫血1例

现将我院收治1例使用青霉素后发生严重的免疫溶血性贫血患者，报道如下：1病例介绍患者，女，62岁，因咳嗽、咳痰诊为“肺部感染”，在本单位医务室经皮试无反应后肌注青霉素80万，2次／d，2天后解大量浓茶水样尿，于1995年1月3日入院。既往曾有过青霉素过敏史，重度贫血貌，全     

溶血性贫血急性症候群的诊断和治疗

溶血性贫血急性症候群的诊断和治疗南京医科大学附属医院敖忠芳溶血性贫血的急性症侯表现为溶血危象（ＨｅｍｏｌｙｔｉｃＣｒｉｓｉｓ）。其特征是急性、重度的溶血，导致内脏器官（心、脑）的急性缺血，或伴血栓形成，及心、肾器官功能急性衰竭。若不及时诊断和治疗，易...     

溶血性贫血相关性肺高压

溶血性贫血是肺高压重要的相关因素和危险因素,但至今还缺乏溶血性贫血相关性肺高压的大规模临床研究报道。随着溶血性贫血患者生存时间的明显延长,肺动脉高压已成为镰刀细胞贫血、地中海贫血及其他血红蛋白病患者最常见的致残、致死并发症。本文复习了近年有关溶血性贫血相关性肺高压的临床研究资料,以提高对该病的认识。     

MCV、EOFT及Hb电泳联合检测在地中海贫血筛查中的价值

目的探讨平均红细胞体积（MCV）、红细胞脆性试验（EOFT）和Hb电泳联合检测在地中海贫血（地贫）筛查中的价值。方法对经分子生物学技术确诊的195例地贫患者（地贫组）和109例非地贫者（对照组）同时进行MCV、EoFr和Hb电泳测定,并对两组三种方法单项检测阳性率及联合检测的灵敏度（Se）、特异度（Sp）等作相关统计学分析。结果地贫组三种方法单项检测阳性率均显著高于对照组（P〈0．001）;除MCV和Eovr平行联合检测的Se与MCV单项检测的Se无显著差异外,其他平行联合检测的Se均显著高于各单项检测的Se,系列联合检测的Sp均显著高于各单项检测的Sp（P均〈0．05）。结论MCV、EOFT及Hb电泳联合检测可降低地贫漏检率,提高诊断的Se与Sp。     

Mechanism of autoimmune hemolytic anemia in chronic lymphocytic leukemia

Chronic lymphocytic leukemia (CLL) is a malignant clonal expansion of CD5+B lymphocytes. The CD5+B lymphocytes have been postulated to produce autoantibodies. CLL patients may demonstrate features of autoimmunity including autoimmune hemolytic anemia. However, the origin of the autoantibodies causing the hemolysis is not clear. The present studies were performed to determine whether these autoantibodies are the products of the neoplastic B-CLL clones. Immunoglobulins (Ig) were eluted from washed red blood cells (RBC) obtained from two CLL patients at the time they had autoimmune (DAT-direct antiglobulin test - positive) hemolytic anemia. The light chain phenotypes of these eluted autoantibodies were determined and found to be monotypic with exact correlation to the light chain expressed on the surface of the B-CLL clones. Elutions from RBC of DAT negative patients or normal volunteers failed to demonstrate measurable amounts of Ig. In contrast, Ig eluted from RBC obtained from SLE patients with DAT positive hemolytic anemia found to be polyclonal autoantibodies exhibiting both light chain types. Furthermore, CD5+B lymphocytes obtained from the same two CLL patients (DAT+) produce, in vitro understimulation with phorbal myristate acetate (PMA), monoclonal antibodies which react and bind to RBC. Thus these studies provide direct evidence demonstrating that the antibodies causing the autoimmune hemolytic anemia in our two CLL patients are the products of the B-CLL neoplastic clones.     

When autoimmune hemolytic anemia complicates chronic lymphocytic leukemia

Autoimmune hemolytic anemia often develops in patients with chronic lymphocytic leukemia, particularly elderly women. It is heralded by a drop in the hematocrit, elevation of reticulocytes, development of jaundice, or a rise in the indirect fraction of serum bilirubin. Evidence of hemolysis supports the diagnosis, and a positive result of the Coombs test confirms it. Survival time is considerably shorter in patients who have both diseases than in those with chronic lymphocytic leukemia alone. Presenting symptoms in patients with the two diseases may include weakness, dizziness, fever, or hemorrhagic phenomena. If the anemia is severe, palpitations, otic pulsations, and cardiac decompensation are common. Physical examination may show enlargement of reticuloendothelial structures. On the other hand, some patients may be essentially asymptomatic. The hemolytic process must be treated as a separate entity, as even vigorous treatment of the leukemia often does not control it. Corticosteroid therapy is preferred, with splenectomy as a second line of defense. If the patient is not a good surgical risk, chemotherapy should be considered. Transfusions are usually incompatible but should be risked if progressive congestive failure, neurologic disturbance, angina, or signs of an impending infarct are present.     

Methyldopa-induced immune hemolytic anemia and chronic active hepatitis

Immune hemolytic anemia and chronic active hepatitis developed concurrently in a 76-year-old man after prolonged therapy with methyldopa. Both complications disappeared following cessation of treatment with the offending drug and a short course of a high-dose corticosteroid (prednisone). Inhibition of T-suppressor lymphocyte function by methyldopa may be instrumental in the emergence of autoimmune complications following usage of the drug.     

Rituximab therapy for chronic lymphocytic leukemia-associated autoimmune hemolytic anemia

Autoimmune hemolytic anemia (AIHA) is a well-known complication of chronic lymphocytic leukemia (CLL). In recent years the anti-CD20 monoclonal antibody rituximab has been used for the therapy of steroid-refractory AIHA and autoimmune thrombocytopenia, either idiopathic or in association with CLL. We report the results of rituximab treatment for 14 patients suffering from CLL-associated AIHA. They developed a direct antiglobulin test positive AIHA at a mean time of 47 months (range 0-135 months) from the diagnosis of CLL. In 3 cases AIHA was diagnosed at the same time as CLL. Only 1 patient had fludarabine-related AIHA. All patients received steroids as first-line treatment. At a mean time of 46 days (range 1-210 days) from the diagnosis of AIHA all patients received rituximab at a dosage of 375 mg/m(2)/weekly for 4 weeks. All patients except 3 (2 died of cardiac failure or sepsis soon after the third cycle and 1 HCV-positive patient experienced a rise in serum amino transferases) completed the scheduled four programmed cycles. First injection side effects of rituximab were minimal. All but 2 patients showed an increase in hemoglobin levels in response to rituximab (mean value 3.6 g/dl; range 0.7-10 g/dl) and a reduction in the absolute lymphocyte count and lymph nodes and spleen volume. Nine patients required packed red cell transfusions before starting rituximab; 5 no longer needed transfusions just after the second cycle and another patient after the fourth cycle. Three patients (22%) were considered to fully respond and 7 (50%) only responded partially. At a mean follow-up of 17 months, 8 patients were still alive, 6 of them transfusion-free. Our results prove that the anti-CD20 monoclonal antibody is an effective and well-tolerated alternative treatment for CLL-associated AIHA.