再生障碍性贫血患者细胞因子水平观察

为进一步探讨细胞免疫对ＡＡ发病的影响，阐明细胞因子在ＡＡ患者中变化的基础与临床意义，采用酶联免疫试剂盒ＥＬＩＳＡ法对３８例ＡＡ患者及２０例正常人外周血单个核细胞（ＰＢＭＮＣ）培养上清诱生Ｇ－ＣＳＦ，ＩＬ－６，ＴＮＦα，ＩＦＮα及ＩＬ－８水平进行测定，同时采用改良ＡＰＡＡＰ法观察外周血Ｔ细胞亚群及ＨＬＡ－ＤＲ抗原表达，结果ＡＡ患者ＰＢＭＮＣ培养上清中Ｇ－ＣＳＦ阳性率减低，ＩＬ－６，ＴＮＦαＩＦＮα及     

32例再生障碍性贫血的微循环及血液粘度

对３２例再障患者的甲襞、球结膜微循环及血粘度进行观察，结果表明：再障组均有不同程度的微循环异常改变（１００％），与正常对照组比较差异显著（Ｐ＜０．０１）；再障组全血粘度、全血还原粘度明显低于正常对照组（Ｐ＜０．０１），再障患者的Ｈｂ与全血粘度及血浆比粘度呈正相关。     

Missed diagnosis in hematological patients—an autopsy study

Autopsy findings of missed diagnoses that would probably have changed management or prognosis occur in up to 29% of cases in general hospitals. Such proportions may be higher in subsets of patients with complex diseases. We reviewed 2908 consecutive autopsies performed over a period of 29 months in a large-volume hospital, analyzing 118 autopsies of patients with hematological malignancies or severe aplastic anemia. A review of macroscopic reports as well as microscopic examination of tissue samples was performed. Medical records were reviewed for clinical diagnoses. Discordances between clinical and autopsy diagnoses were classified using Goldmans criteria. Additionally, we searched for clinical parameters correlated with occurrence of class-I discrepancy using a multivariate method. Median age was 46.5 years, and 25.4% had received a hematopoietic stem-cell transplant. Overall, 11.9% (6.6–19.1%) of patients died before conclusion of the hematological diagnosis and 33% (24.6–42.3%) died with no active hematological disease. We found class-I discrepancy in 31.3% (23.1–40.5 %) of cases. The most common among these diagnoses were hematological disease, pneumonia and gastrointestinal bleeding. In a univariate analysis, being elderly (P=0.04) was positively correlated with the finding of class-I discrepancies; while, having received previous specific hematological treatment (P=0.0005) or hematopoietic stem-cell transplants (P=0.013), or being admitted to a specialized hematology unit (P=0.0006) were negatively correlated to the occurrence of such discrepancies. Multivariate analysis showed that care in a specialized hematology unit (OR 0.34, 0.12–0.93) was independently associated with lower occurrence of discrepancies. We concluded that critical diagnoses are often missed in highly complex hematological patients especially in the absence of admission to specialized hematology units.     

Bone marrow mast cell reaction in preleukaemic myelodysplasia and in aplastic anaemia

Summary The relationship of bone marrow mast cell counts to prognosis was investigated in 48 patients with preleukaemic myelodysplasia, in 59 patients with aplastic anemia and in a DMBA induced myelodysplasia/leukaemia rat model. In patients with myelodysplasia terminating in overt leukaemia the number of mast cells per square millimeter was not correlated to duration of the preleukaemic course. Leukaemia development probabilities of patients at risk were not different for low and elevated mast cell counts. In aplastic anaemia, however, a lower bone marrow mast cell count was related to a higher survival probability and longer survival time. In the animal model no significant differences could be found between myelodysplastic, leukaemic, and control animals.     

再生障碍性贫血患者淋巴细胞表型变化

目的：研究再生障碍性贫血（AA）患者骨髓（BM）及外周血（PB）淋巴细胞及其活化相关分子的表达及临床意义。方法：采用单色和双色免疫荧光标记法，流式细胞仪分析AA患者的BM和PB中淋巴细胞膜分子的表达。结果：AA患者BM和BP中CD8^ 细胞增加，CD4／CD8比例下降，BM在CD25^ 细胞和HLA－DR^ 细胞增多，急性AA增加尤为显著（P＜0．01），BM中CD16^ 或CD56^ 细胞也明显增多（P＜0．05），双标记分析提示T细胞主要为CD8^ 细胞：急性AA患者CD8^ -CD25^ 细胞显著增多（P＜0．01），AA患者BM中淋巴细胞活化相关分子表达增多，尤其4－1BB^ ,CD95L^ 和CD40L＋细胞显著增多（P＜0．01），结论：AA患者BM中淋巴细胞活化相关膜分子增多，是AA免疫功能异常及最终导致造血功能衰竭的原因之一。     

抗T细胞单克隆抗体对再生障碍性贫血患者TNF和IL—2水平的影响

为探讨抗Ｔ淋巴细胞克隆抗体对再生障碍性贫血患者免疫功能的调节作用，采用放射免疫检测２５例ＡＡ患者ＭｃＡｂ－Ｔ治疗前后血清肿瘤坏死因子和白细胞介素－２（ＩＬ－２）水平及其中１０例周围血单个核细胞体外诱生ＴＮＦ和ＩＬ－２水平的变化。     

国产十一酸睾酮并中药治疗慢性再生障碍性贫血的疗效观察

目的　观察国产十一酸睾酮治疗慢性再生障碍性贫血的疗效及其副作用。分析患者雄激素治疗的疗效与骨髓增生程度的关系。方法　将 60例慢性再生障碍性贫血患者单纯随机分为两组 ,十一酸睾酮治疗组 3 0例 ,肌注十一酸睾酮 ,2 5 0mg/次 ,1次 /周 ,血红蛋白正常后 ,改为 1次 / 2周 ,2个月后 1次 / 4周。康力龙对照组 3 0例 ,口服康力龙 2mg,3次 /d。两组患者均按辨证分型给以中药汤剂治疗。观察治疗前后患者骨髓增生程度的变化及副作用。结果　治疗组和对照组有效率分别为 76 7%和 73 3 % (P >0 0 5 ) ,而肝功能损害率分别为0 %和 2 0 0 % ( ,P <0 0 5 ) ,声音变粗分别为 5 6 7%和 2 0 0 % (P <0 0 0 5 ) ,毛发增多分别为 5 6 7%和 3 0 0 %(P <0 0 5 ) ,痤疮发生率分别为 4 6 7%和 3 6 7% (P >0 0 5 ) ,女性闭经发生率分别为 10 0 0 %和 3 5 3 % (P<0 0 0 5 )。本组 60例患者骨髓增生程度治疗前后无明显变化 (P >0 0 5 ) ,雄激素治疗疗效与骨髓增生程度无明显相关 (Hc=5 5 9,P >0 0 5 )。结论　十一酸睾酮是一种安全、有效的雄激素制剂 ,且肝功能损害率极低 ,值得在临床推广应用。雄激素治疗有效的病例其造血功能的恢复可能是残存造血岛的代偿性造血功能增加的结果 ,而非骨髓造血的普遍恢复     

基于数据挖掘的低增生性骨髓增生异常综合征与再生障碍性贫血分类模型研究

目的 构建低增生性骨髓增生异常综合征（hypo-MDS）与再生障碍性贫血（AA）鉴别诊断的决策树、贝叶斯、卷积神经网络、改进的支持向量机四种模型并选择出最优模型。方法 收集2010—2019年华北理工大学附属医院的AA与hypo-MDS患者的病例资料,使用统计学方法筛选指标,将处理后的样本以4[DK]∶1随机分为训练集和测试集,构建决策树、贝叶斯、卷积神经网络、改进的支持向量机四种模型,采用五折交叉验证法多次重复验证,通过灵敏度、AUC等指标评价鉴别诊断效果。结果 hypo-MDS患者红细胞、血红蛋白含量等指标低于AA患者,成熟单核细胞比例等指标高于AA患者,年龄和职业分布也存在差异(P＜0.05）;最终选出21个特异性指标。四种模型的分类效果比较:灵敏度分别为82.56%、65.12%、87.21%、79.07%;AUC分别为0.81、0.68、0.82、0.83;准确率分别为75.32%、69.48%、77.27%、74.03%。对卷积神经网络的误判病例分析得出年龄、血成熟淋巴细胞等7个指标均存在差异（P＜0.05）。结论 在决策树、贝叶斯、卷积神经网络、改进的支持向量机四种诊断模型中,卷积神经网络具有最佳分类效果。     

骨髓腔内输注复方丹参注射液治疗再生障碍性贫血的临床研究

目的 :观察骨髓腔内输注复方丹参对再生障碍性贫血 50例的疗效 .方法 :采用骨髓腔内输注复方丹参液并中医辨证的方法治疗再生障碍性贫血 ,并对其作用机制进行了探讨。结果 :治愈 2 4例 ,缓解 8例 ,明显进步 1 3例 ,无效 5例 ,总有效率 90 %。结论 :复方丹参注射液骨髓腔内输注与中医辨证结合治疗再障 ,能提高临床疗效     

中西医结合治疗再生障碍性贫血——附30例临床观察

目的：探讨中西医结合治疗再生障碍性贫血的疗效。方法：采用中医补肾健脾,益气活血的克障汤,辅以西药综合治疗。结果：本组共治疗再生障碍性贫血３０ 例,经２６ 个月治疗,总有效率８０％ 。结论：中西医结合治疗疗效明显优于单纯使用西药组,早期诊断、早期治疗和坚持长期治疗是本病早日康复的关键。