用1升全血中平均血红蛋白密度鉴别诊断缺铁性贫血和地中海贫血特征

目的探讨1升全血中平均血红蛋白密度(MDHLWB)鉴别诊断缺铁性贫血(IDA)和地中海贫血特征(TT)的临床价值。方法根据物理学"密度"的概念建立了一个新的红细胞参数MDHLWB[MDHLWB=(MCH/MCV×10-3)×RBC计数]。随机选取经血液表型分析诊断的150例IDA和166例TT成年病例,用ROC曲线分析确定MDHLWB鉴别诊断TT和IDA的cutoff值。以分子诊断并结合铁代谢指标分析作为金标准,将MDHLWB和文献报道的IDA和TT鉴别诊断指标用于296例临床小细胞低色素贫血个体的鉴别诊断以评估MDHLWB的临床应用价值。结果 MDHLWB鉴别成年男、女IDA与TT的最佳cutoff值分别为男1.736和女1.493。MDHLWB诊断TT的敏感性(SE)、特异性(SP)、阳性预测值(PPV)、阴性预测值(NPV)、诊断效率(EDF)和约登指数(Youden’s index,YI)分别为96.32%、90.98%、92.90%、95.28%、93.92%和0.873,优于其他鉴别诊断指数。结论 MDHLWB可快速、有效地鉴别诊断单纯性的IDA和TT。     

甲巯咪唑治疗甲状腺功能亢进致再生障碍性贫血患者的护理

甲状腺功能亢进简称甲亢,是由多种原因引起的甲状腺激素分泌过多所致的一组常见内分泌疾病.再生障碍性贫血系多种病因引起的造血功能障碍,导致红骨髓总容量减少,代以脂肪髓,造血衰竭,以全血细胞减少为主要表现的一组综合征.甲亢病本身和抗甲状腺药物均可引起白细胞减少,严重者可导致粒细胞缺乏而危及生命.2010年4月～2011年6月,我院收治13例甲巯咪唑治疗甲亢致再生障碍性贫血患者,经过医护人员精心治疗和护理后好转出院.现报告如下.     

儿童重型再生障碍性贫血并发毛细血管渗漏综合征诊治探讨（附2例报告）

目的探讨联合免疫抑制治疗重型再生障碍性贫血（SAA）并发毛细血管渗漏综合征（CLS）儿童的临床特征、发病机制及诊治方法。方法总结2011年我科行联合免疫抑制治疗SAA并发CLS病儿2例的临床特点及诊治过程,并结合相关文献进行分析。结果 2例病儿分别于连续5d应用抗胸腺细胞免疫球蛋白（ATG）结束后38d和1d发生CLS,临床特征有体质量增加、腹围增加、双下肢轻度凹陷性水肿、胸腔积液、心包积液、腹水、非感染性腹泻、低清蛋白血症,单纯输注清蛋白无效,以及无明显低血容量性低血压。经糖皮质激素＋羟乙基淀粉＋呋塞米＋清蛋白治疗后,症状及体征均逐渐消失。结论 CLS是联合免疫抑制治疗SAA过程中的一种罕见并发症,早期诊断和治疗可治愈。     

环孢素A联合康力龙治疗慢性再生障碍性贫血的临床观察

目的观察环孢素A联合康力龙应用于慢性再生障碍性贫血（CAA）的疗效。方法将52例慢性再生障碍性贫血患者随机分为对照组和观察组。对照组：单用康力龙治疗,观察组在对照组基础上联合环孢素A治疗,比较两组的疗效,以及两组治疗前后外周血中性粒细胞、血红蛋白、网织红细胞及血小板的变化情况。结果观察组总有效率76．9％,高于对照组的总有效率（53．8％）（P〈0．05）。两组治疗后中性粒细胞、血红蛋白、网织红细胞、血小板均较治疗前明显改善,且观察组较对照组改善更明显（P〈0．05）。结论环孢素A联合康力龙应用于慢性再生障碍性贫血疗效可靠,安全性高,值得广泛推广和应用。     

血液透析与腹膜透析改善肾性贫血的临床观察

目的比较血液透析(HD)和腹膜透析(PD)改善肾性贫血患者的疗效。方法选择尿毒症行透析治疗患者59例,其中行腹膜透析患者24例(PD组),行血液透析患者35例(HD组)。2组开始治疗时未使用促红细胞生成素,在治疗1个月时均加用促红细胞生成素,治疗2个月时均加用左旋卡尼汀联合促红细胞生成素治疗,比较2组患者透析治疗前与开始透析治疗1个月、2个月、3个月后血红蛋白(Hb)值的变化。结果透析治疗前PD组和HD组的Hb值无明显差异(P>0.05)。透析治疗前后比较,PD组和HD组治疗1个月、2个月、3个月的Hb值均明显升高(P<0.05或P<0.01),治疗3个月>治疗2个月>治疗1个月(P<0.05或P<0.01),且透析治疗1个月、2个月、3个月后PD组的Hb值均高于HD组,差异有统计学意义(P<0.05或P<0.01)。结论无论腹膜透析和血液透析均能改善贫血,但腹膜透析疗效更明显,促红细胞生成素可有效纠正尿毒症患者的肾性贫血,并且联合左旋卡尼汀治疗效果更明显。     

环孢菌素A联合雄激素治疗再生障碍性贫血疗效分析

目的总结环孢素联合雄激素治疗再生障碍性贫血的临床效果。方法选择1997年1月至2010年8月在我院住院的再生障碍性贫血患者208例,根据分型分为SAA组60例,CAA组148例。SAA患者随机分为2组,分别为CSA治疗组和CSA＋雄激素联合治疗组;CAA患者随机分为2组,分别为CSA治疗组和CSA＋雄激素联合治疗组。结果 60例重型再障治疗有效率为33.3%,单用环孢素（CSA）治疗有效率为16.7%,CSA和雄激素联合应用的治疗有效率为44.4%;148例慢性再障治疗有效率为73.0%,单用CSA治疗有效率为66.7%,CSA和雄激素联合应用的治疗有效率为76.0%。结论 CSA和雄激素联合应用为治疗AA的有效方法。     

骨髓增生异常综合症骨髓细胞结构异常与贫血和粒细胞减少相关性研究

本研究旨在探索骨髓增生异常综合症患者贫血和外周血中性粒细胞减少与骨髓幼红细胞和中性幼粒细胞超微结构异常的相关性。采用全自动血细胞分析仪检测74例MDS患者贫血指标和中性粒细胞数,根据贫血程度和外周血中性粒细胞减少程度将MDS患者分别分为正常范围、轻度、中度和重度4组,用透射电子显微镜分析所有患者骨髓中性幼粒细胞和幼红细胞病态及凋亡细胞百分率。结果表明,74例MDS患者中68例贫血(92%),68例患者中51例外周血中性粒细胞减少(75%);电子显微镜下所有MDS患者骨髓幼红细胞和中性幼粒细胞不同程度结构异常;血红蛋白正常组和轻度、中度及重度贫血4组患者骨髓幼红细胞病态率分别为(37±14.7)%、(24±9)%、(32±16)%和(34±21)%,凋亡率分别为(2.25±1.03)%、(4.43±2.60)%、(8.78±4.04)%和(11.67±4.57)%,病态和凋亡率分别与血红蛋白及红细胞比容呈负相关(p<0.05);外周血中性粒细胞正常、轻度、中度和重度减少4组患者骨髓中性幼粒细胞凋亡率分别为(6.00±2.67)%、(9.50±4.42)%、(13.00±3.54)%和(17.00±2.39)%,与外周血中性粒细胞呈负相关(p<0.01);骨髓中性幼粒细胞病态率分别为(12.25±16.31)%、(13.5±10.01)%、(23±8.59)%和(51.67±19.67)%,病态率与凋亡率呈正相关(p<0.01)。结论:MDS患者骨髓幼红细胞和中性幼粒细胞的凋亡和病态发育与贫血、外周血中性粒细胞减少相关,二者可能是MDS无效造血重要成因。     

增生性贫血患儿骨髓单个核细胞转铁蛋白受体2表达情况及其意义探讨

本研究旨在探讨增生性贫血患儿骨髓单个核细胞转铁蛋白受体2(transferrin receptor 2,TfR2)表达情况,分析TfR2 mRNA表达水平与贫血程度、骨髓红系增生程度、基础疾病种类和铁状况等指标的相关关系,评价TfR2受体蛋白在红系造血方面的作用和在增生性贫血诊断方面的应用价值。实验分为两组:实验组系我院儿童血液肿瘤科收治的40例增生性贫血患者,对照组系经骨髓检查排除红系疾病和血液系恶性肿瘤的10例患者。收集研究对象的骨髓抗凝标本,采用荧光定量PCR检测增生性贫血患儿骨髓单个核细胞TfR2 mRNA表达水平,分析其与骨髓增生程度、原发疾病种类以及机体铁状况的关系。结果表明,增生性贫血组骨髓单个核细胞TfR2相对表达量显著高于对照组;增生性贫血组TfR2相对表达量与外周血Hb含量呈负相关关系(rs=-0.715),而与骨髓幼红细胞比例呈正相关关系(rs=0.533)。结论:增生性贫血患者TfR2表达水平增高,与骨髓增生程度、外周血贫血程度密切相关。     

Frequency of pain crises in sickle cell anemia and its relationship with the sympatho-vagal balance, blood viscosity and inflammation

Background

Recent evidence suggests that autonomic nervous system activity could be involved in the pathophysiology of sickle cell disease, but it is unclear whether differences in autonomic nervous system activity are detectable during steady state in patients with mild and severe disease. The aim of the present study was to compare the autonomic nervous system activity, blood rheology, and inflammation in patients with sickle cell anemia according to the frequency of acute pain crisis.

Design and Methods

Twenty-four healthy volunteers, 20 patients with sickle cell anemia with milder disease, and 15 patients with sickle cell anemia with more severe disease were recruited. Milder disease was defined as having no pain crisis within the previous year. More severe disease was defined as having had within the previous year three or more pain crises which were documented by a physician and required treatment with narcotics. The autonomic nervous system activity was determined by spectral analysis of nocturnal heart rate variability. Blood viscosity determination and measurements of several inflammatory markers (interleukin-6, soluble vascular cell adhesion molecule-1, soluble CD40 ligand and sL-selectin) were made on blood samples collected in steady-state conditions.

Results

Results showed that: 1) patients who had suffered more frequent pain crises had lower parasympathetic activity and greater sympatho-vagal imbalance than both controls and patients with milder disease. However, when adjusted for age, no significant difference was detected between the two sickle cell anemia patient groups; 2) patients who had suffered more frequent pain crises had higher blood viscosity than patients with milder disease, and this was not dependent on age.

Conclusions

Results from the present study indicate that both the autonomic nervous system activity and blood viscosity are impaired in patients with sickle cell anemia exhibiting high frequency of pain crisis in comparison with those who did not experience a crisis within the previous year.     

New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children

Background

Autoimmune hemolytic anemia is a rare condition in children. Little is known about its initial presentation and the subsequent progression of the disease.

Design and Methods

Since 2004, a national observational study has been aiming to thoroughly describe cases and identify prognostic factors. Patients from all French hematologic pediatric units have been included if they had a hemoglobin concentration less than 11 g/dL, a positive direct antiglobulin test and hemolysis. Evans’ syndrome was defined by the association of autoimmune hemolytic anemia and immunological thrombocytopenic purpura. Data from patients’ medical records were registered from birth to last follow-up. Autoimmune hemolytic anemia was classified as primary or secondary. Remission criteria, qualifying the status of anemia at last follow-up, were used with the aim of identifying a subgroup with a favorable prognosis in continuous complete remission.

Results

The first 265 patients had a median age of 3.8 years at diagnosis. In 74% of cases the direct antiglobulin test was IgG/IgG+C3d. Consanguinity was reported in 8% of cases and first degree familial immunological diseases in 15% of cases. Evans’ syndrome was diagnosed in 37% of cases. Autoimmune hemolytic anemia was post-infectious in 10%, immunological in 53% and primary in 37% of cases. After a median follow-up of 3 years, 4% of children had died, 28% were still treatment-dependent and 39% were in continuous complete remission. In multivariate analysis, IgG and IgG+C3d direct antiglobulin tests were associated with a lower rate of survival with continuous complete remission (adjusted hazard ratio, 0.43; 95% confidence interval, 0.21–0.86).

Conclusions

This nationwide French cohort is the largest reported study of childhood autoimmune hemolytic anemia. The rarity of this condition is confirmed. Subgroups with genetic predisposition and underlying immune disorders were identified.