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。     

Autoimmune Hemolytic Anemia Associated with Anti-f: Report of a Case

An autoimmune hemolytic anemia occurring in a 60-year-old woman is described. The patient's blocd group is A, Rh-negative ( cde/cde ). Her serum was found to contain high titer anti-D and anti-C, which may have arisen as a result of isoimmunization by a pregnancy and a blood transfusion 17 years earlier. An eluate from the red cells contained an incomplete warm autoantibody of anti-f specificity.     

自身免疫溶血性贫血患者抗体筛选观察

为了观察自身免疫溶血性贫性(AIHA)患者的同种抗体，用氯奎放散试验检测自身抗体掩盖的同种抗体，用乙醚放散试验检测自身抗体的特异性。结果表明：38例AIHA病人中，间抗阳性者19例．其中含同种抗体者7例(抗-D1例，抗-E4例，抗-CE2例)，自身抗体具有D-型特异性者5例(抗-E3例．抗-C1例．抗-c 1例)。结论：采用氯奎放散试验、乙醚放散试验等检测被自身抗体掩盖的同种抗体，对AIHA患者的安全输血很重要。     

New Therapies for the Treatment of Warm Autoimmune Hemolytic Anemia

In this review article we provide a critical insight into recent reports evaluating innovative therapies for warm type autoimmune hemolytic anemia (wAIHA). Among published articles, we selected two reports on the use of the proteasome inhibitor bortezomib in association with dexamethasone or rituximab, one study on the spleen tyrosine kinase inhibitor fostamatinib, and a retrospective study on recombinant erythropoietin (rEPO). Among recent scientific communications, we discussed a report on the phosphoinositide 3-kinase delta inhibitor (PI3Kδi) parsaclisib. All studies highlighted a good efficacy although to be confirmed in larger trials and with limitations due to the heterogeneity of wAIHA patients enrolled, the small number of subjects, the concomitant medications allowed, and the short follow-up. Ongoing trials include new B-cell/plasma-cell targeting agents such as the Bruton tyrosine kinase inhibitors ibrutinib and rilzabrutinib, and the anti-CD38 MoAbs daratumumab and its analogue isatuximab. Further drugs in clinical trials target the complement cascade in wAIHA with complement activation, such as the C3 inhibitor pegcetacoplan and the C1q inhibitor ANX005. Finally, an interesting and non-immuno-toxic strategy is to remove the pathogenic autoantibodies via blocking the neonatal Fc receptor, by intravenous nipocalimab and subcutaneous RVT-1401. Such novel agents targeting the several immunopathological mechanisms acting in wAIHA and their possible combination, will increase the therapeutic armamentarium and possibly fill the gap of wAIHA relapsed after/refractory to rituximab. Moreover, these new target therapies may represent a tool for the unmet need of very acute cases.     

Bovine Blood and Neuromuscular Paralysis as a Bridge to Recovery in a Patent with Severe Autoimmune Hemolytic Anemia

Hemoglobin solutions have several advantages as substitutes for erythrocyte transfusions. They have a prolonged shelf life, do not require cross‐matching, are associated with few transfusion reactions, and are effective in delivering oxygen to the tissues. Despite the potential clinical utility of these solutions, they have not achieved widespread use. HbOC‐201 (Hemopure, Biopure, Cambridge, MA, USA) is a bovine hemoglobin solution that has been FDA approved for compassionate use in Jehovah''s witness patients requiring transfusions. Here we report a case of a patient with hemoglobin H disease who developed a severe life‐threatening mixed warm and cold antibody mediated autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) who was successfully treated with HbOC‐201 as an adjunct to blood transfusion.     

Sirolimus for Refractory Autoimmune Hemolytic Anemia after Allogeneic Hematopoietic Stem Cell Transplantation: A Case Report and Literature Review of the Treatment of Post-Transplant Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia (AIHA) may occur after any type of allogeneic hematopoietic stem cell transplantation (HCT), even ABO-matched transplantation. It tends to be refractory to standard corticosteroid treatment and requires multiple transfusions. Though, there is no consensus regarding the optimal treatment for post-transplant severe AIHA. We present a pediatric patient with refractory AIHA after umbilical cord blood transplantation. She developed severe AIHA at 3 months after transplantation and was unresponsive to multiple treatment modalities, including corticosteroids, intravenous immunoglobulin, plasma exchange and rituximab, resulting in persistent transfusion dependency. Sirolimus, a mammalian target of rapamycin inhibitor, was started on day 67 after the onset of AIHA, and this patient was successfully rescued without any complications. Sirolimus induces apoptosis in autoreactive lymphocytes, increases regulatory T cells and has been reported to have a positive effect on AIHA following solid organ transplantation (SOT). We reviewed the literature regarding post-transplant AIHA in the PubMed database and evaluated the treatment outcome of sirolimus in AIHA after SOT.     

抗CD20单克隆抗体治疗难治性自身免疫性溶血性贫血

本研究初步观察抗CD20单克隆抗体利妥昔（rituximab）用于治疗难治性自身免疫性溶血性贫血（AIHA）的疗效和安全性。对1例用糖皮质激素、脾切除治疗无效的AIHA患者,采用利妥昔单克隆抗体,375mg/m2,每周1次,共4次,观察溶血症状的改变并监测血红蛋白（Hb）水平及其它检验指标,同时观察有无不良反应发生。结果表明,首次治疗后11天乳酸脱氢酶（LDH）、总胆红素（TBIL）、直接胆红素（IBIL）逐渐下降,第45天降至正常范围;首剂治疗25天后Hb水平比治疗之前升高到95-100g/L以上。治疗后已4月余,患者仍处于缓解状态。治疗过程中未发生明显不良反应。结论：抗CD20单克隆抗体利妥昔用于治疗难治性自身免疫性溶血性贫血是有效而且安全的。     

自身免疫性溶血性贫血患者外周血B-1a细胞的研究

目的：研究自身免疫性溶血性贫血患者外周血B-1a细胞的变化。方法：用CD5CD19双标抗体和流式细胞仪检测20例正常对照、13例自身免疫性溶血性贫血（AIHA）发病初期患者、4例脾切除术后AIHA患者以及10例外伤后脾切除患者的外周血B细胞和13-1a细胞占外周血单个核细胞的百分比。结果：正常对照外周血昏1a细胞占外周血单个核细胞的比例为（0．78±0．16）％，AIHA患者发病初期外周血B-1a细胞百分比为（2．58±0．63）％，AIHA患者脾切除术后外周血B-1a细胞百分比降至（0．58±0．37）％，外伤后脾切除患者外周血B-1a细胞百分比为（0．65±0．43）％。结论：AIHA患者外周血B-1a细胞比例明显增高，且AIHA患者接受脾切除手术溶血得到控制后，B-1a细胞比例明显下降。     

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.     

自身免疫性溶血性贫血自身抗体血型特异性的鉴定及其临床意义

本文采用乙醚放散法,以O型R_1R_1,R_2R_2和rr为试剂红细胞,鉴定了16例自身免疫性溶血性贫血患者自身抗体的血型特异性,结果发现4例自身抗体具有血型抗原特异性,其中抗-E1例,抗-C1例,抗-e2例,为其选择相合的血液输注,避免了输血反应.进行自身抗体血型特异性的鉴定,是防止医源性溶血的有力措施.     

Successful Pregnancy in a Patient with Hodgkin's Disease Complicated by Warm Autoimmune Hemolytic Anemia and Anti-e Alloimmunization

A patient with Hodgkin's disease is described, whose first pregnancy was complicated by warm autoimmune hemolytic anemia and anti-e allo-immunization probably due to previously transfused blood. Therapy with large doses of prednisone produced a reduction in both autoantibody and allo-antibody titers, and the patient was subsequently delivered of an infant having only mild hemolytic disease of the newborn.     

AIHA患者应用两种配血方法的输血疗效比较

目的 通过比较自身免疫性溶血性贫血(AIHA)患者分别采用微柱凝集法和体外溶血试验两种配血方法进行输血的疗效,为AIHA患者寻找一种安全有效的配血方法。方法 选取北京大学深圳医院2010年1月～2014年12月诊断为AIHA并且需要输血治疗的患者75例,随机分为A组40例与B组35例,A组采用微柱凝集法配血共输血65人次,B组采用体外溶血试验配血共输血63人次,比较两组患者输血疗效。结果 两组患者输血治疗后RBC计数与Hb水平均有上升,TBIL含量均下降。B组患者RBC计数,Hb水平升高幅度及TBIL含量下降幅度明显高于A组患者; 两组Hb水平输血前比较差异无统计学意义(P>0.05),输血后比较差异有统计学意义(P<0.05),表明B组输血疗效优于A组。A组输注总有效率为67.69%,B组输注总有效率为93.65%,B组总有效率明显高于A组,两组比较差异有统计学意义(P<0.05)。结论AIHA患者采用体外溶血试验配血方法可以筛选到更适合的供者红细胞,确保患者输血安全有效。     

从临床医师角度改进实验室信息系统服务

实验室信息系统服务于临床的过程中,检测项目的优化组合使工作效率得到提高;室内标本交接制度的建立有效解决了取报告时间不同的项目的申请;报告的合并解决了同一病人多张报告的难题;对临床意见反馈的及时跟进、与临床的积极沟通使实验室及时了解临床需要.通过以上工作,完善了实验室信息系统,提高了服务质量.     

Pain Research Update from a Genetic Point of View

Abstract:   Molecular biology investigates the genetic causes of many diseases. Currently, molecular biology in pain research lags behind the investigations of the molecular basis of mental disorders. A significant challenge in pain genetic research is the fact that pain involves emotional factors. Tools available for pain measurements and interindividual comparisons have been imperfect. Another problem relates to research ethics. Unlike animal studies, there is very limited ability to evoke experimental pain in a group of humans with precisely defined age, sex, medication, and pain experience. Nevertheless, pain investigations at the gene level have commenced. Recent progress in molecular biology has enabled gene expression modulation in animal models using "knockout,""oligo-antisense," and viral vector techniques. These methods enable investigation, at molecular level, as to which of the approximately 30,000 genes of the human genome might be involved in pain mediation, which of these are polymorphic, and which polymorphisms are responsible for interindividual differences in pain perception. Recently, the genetic bases of familial hemiplegic migraine and congenital insensitivity to pain with anhidrosis have been shown. In the last 6 years, genetic pain research has focused on potential gene therapy for patients with chronic pain. Results of these studies are encouraging and potentially applicable to clinical practice in the near future.     

应用流式细胞术对自身免疫性溶血性贫血患者自身抗体掩盖下血型同种抗体检测的研究

目的 在自身免疫性溶血性贫血(自免溶贫)患者血样的检测中,自身抗体往往易被同种抗体掩盖,本研究试图通过对流式细胞术与传统血清学方法的比较研究,分析两者对自身抗体中混合同种抗体的检测能力差异,为自免溶贫患者输血前检测提供更敏感可靠的方法.方法 随机选择使用5例人源抗-D及4例抗-E分别与经验证不具血型特异性的自身抗体按比...     

支架与手术--神经科专科医生的观点

尚无充分证据支持血管内治疗可替代动脉内膜切除术而成为治疗颈动脉狭窄的标准疗法.最近的Cochrane系统评价也未发现颈动脉血管内治疗与手术在主要疗效方面存在差异.随机试验的证据表明,血管内治疗颈动脉狭窄在避免颅神经病变和创伤性血肿等小的并发症方面有优势.长期随访结果表明,两者卒中发病率没有差别,但可信区间范围很宽,可能包含了这两种治疗方法在风险和获益两方面存在实质差异的概率.应用保护装置的支架术预防脑卒中发生与传统手术相比无差别,且还有导致额外并发症的风险.