CAA患者造血干细胞内Ca2+含量、CD95+表达水平及相关因素的研究

目的 :探讨慢性再生障碍性贫血 (CAA)患者骨髓造血干细胞内Ca2 + 含量及CD34、CD95阳性细胞率及其相关因素IFN -r、TNF -a之间的内在联系 ;方法 :我们分别采用钙离子邻%D甲酚酞络合酮直接比色法、流式细胞仪双标记法及放免法 ,检测 30例CAA患者的骨髓造血干细胞内Ca2 + 含量、CD34、CD95阳性细胞率及IFN -r、TNF -a水平 ,并与正常手术组 (14例 )和健康献血员 (14例 )比较。结果 :CAA患者骨髓造血干细胞内Ca2 + 及CD34、CD95阳性细胞率与手术组比较 ,有明显差异 (P <0 .0 1) ,患者外周血清中IFN -r、TNF -a的表达水平也与正常人有较显著的差异 (P <0 .0 1)。结论 :CAA患者其造血干细胞存在凋亡现象 ,是通过IFN -r、TNF -a作用靶细胞后触动靶细胞的CD95表面蛋白受体 ,进而引起靶细胞内的连锁生化反应 ,包括Ca2 + 升高 ,使DNA降解而凋亡     

非清髓性脐血移植治疗成人重型再生障碍性贫血

目的　研究与追踪观察无关供者脐血造血干细胞移植 (Allo CBSCT)治疗成人重型再生障碍性贫血 (SAA)后 ,脐血造血细胞的植入情况及疗效。方法　单份脐血 (3例 )和双份脐血 (3例 )移植治疗成人重型再生障碍性贫血患者共 6例。输入的脐血单个核细胞 (MNC)数为 (1.6～ 10 .7)×10 7/kg(按患者体重计 )。预处理方案由低剂量的环磷酰胺 (CTX)和抗淋巴细胞球蛋白 (ALG)组成。CTX总用量为 6 0mg/kg ;ALG为 12 0mg/kg。移植后供者细胞植入状态的检测方法采用微卫星DNA指纹法或萤光定量PCR分析。结果　 6例中有 5例移植后有供者细胞植入证据 ,均为供、受者细胞混合嵌合状态。 2例接受双份脐血移植者均仅显示单份脐血植入。迄今追踪时间平均 2 1个月 (7～ 5 0个月 ) ,4例造血完全恢复。 2例移植后早期死于重症感染 ,其中 1例曾有早期植入证据。结论　Allo CBSCT治疗成人重型再生障碍性贫血 ,可形成较长期的稳定供、受者造血细胞混合嵌合体。脐血可作为造血干细胞来源的一种选择。     

以“芪英三两三”组方经验为指导治愈顽固慢性再生障碍性贫血1例

慢性再生障碍性贫血属于临床血液系统常见疾病之一，发病率呈逐年升高趋势，病程长，易反复，对患者的生活质量形成严重影响。姜苗教授认为慢性再生障碍性贫血不仅与肾虚相关，且与脾胃升降功能联系密切，强调从脾论治，抓住“虚”“瘀”“毒”3个病理因素，活用“芪英三两三”组方加减，健脾益肾，化瘀解毒。文章以完全治愈1则顽固慢性再障合并继发血小板减少病案为例，探讨姜教授治疗慢性再生障碍性贫血思路及用药方案。     

杨文华教授辨治慢性再生障碍性贫血经验浅析

从慢性再生障碍性贫血的中医病因病机及临证遣方用药特点等方面对杨文华教授的治疗经验进行归纳、总结,以期为临床治疗提供借鉴。杨文华教授认为慢性再生障碍性贫血发病以肾虚精亏为本,脉络瘀阻为标,“肾虚精亏、髓枯血瘀”为病机关键,治疗以“补肾生髓、祛瘀生新”为根本大法,同时注重调补阴阳,善用血肉有情之品、炭类止血药物及中药药对等治疗,临床疗效显著。     

RELATIONSHIP BETWEEN HUMAN PARVOVIRUS B19 INFECTION AND APLASTIC ANEMIA

Objective. To explore the relationship between human parvovirus B19 (HPV B19) infection and aplastic anemia (AA) and to investigate the role of HPV B19 in the occurrence of AA. Methods. The presence of HPV B19 DNA was detected in the peripheral blood samples of 60 patients with AA (children 38 and adults 22) by nested polymerase chain reaction (PCR) assay, and 30 healthy persons were selected as control. Results. Sixteen (26. 7 % ) of 60 AA cases were HPV B19 DNA positive, while all the samples in the control group were negative for HPV B19 (P = 0. 000914). Among the case group, the positive rates of HPV B19 DNA were21.4% (6 /28), 30.0% (3 / 10), 20.0% (1 /5) and 35.3% (6 / 17) in children acute AA (AAA), children chronic AA (CAA), adults AAA and adults CAA patients respectively, which were significant-ly higher than that in the control group, Furthermore, there was no remarkable difference between children AA and adults AA in the 16 HPV B19 DNA positive patients; neither was there between AAA and CAA. Conclusions. HPV B19 infection is not only correlated with the occurrence of children AAA and CAA, but also with adults AAA and CAA, and might be an important viral cause for AA in humans.     

30例中西医结合治疗慢性再障观察

目的观察健脾补肾活血方剂配合西药治疗慢性再生障碍性贫血（CAA）的疗法。方法30例患者均口服强的松、康力龙或环孢素等西药。其中15例在其基础上加用健脾补肾活血煎剂口服。结果15例中西医结合治疗患者总有效率为80%,远期疗效明显,长期口服西药所致的不良反应发生率低。明显优于单纯应用西药组（P〈0.01）。结论中西医结合方法治疗CAA疗效明显,复发率低,西药不良反应减少。     

健脾补肾活血方及其拆方对免疫介导再生障碍性贫血小鼠骨髓造血细胞凋亡的影响

目的:采用细胞培养方法,从细胞凋亡角度探讨健脾补肾活血方对再生障碍性贫血(aplastic anemia,AA)模型小鼠的疗效及其促进骨髓造血的作用机制。方法:复制AA小鼠模型并制备AA小鼠血清,以健脾补肾活血方及其拆方灌胃大鼠制备含中药血清,共同培养正常小鼠骨髓造血细胞,24 h后采用流式细胞仪和透射电镜观察细胞的凋亡情况。结果:AA小鼠血清能诱导正常小鼠骨髓造血细胞发生凋亡,经健脾补肾活血方及其拆方的药物血清作用后,各组小鼠骨髓造血细胞的凋亡率均下降,其中,补肾方的疗效优于健脾方和活血方,健脾补肾活血方(全方)的效果最好。电镜观察造血细胞超微结构的结果亦表明补肾方的作用较好,但以全方作用最佳。结论:健脾补肾活血方及其拆方均可不同程度地降低骨髓细胞凋亡率,可促进骨髓恢复造血功能。     

骨形态发生蛋白诱导髓外造血的实验研究

目的:探讨骨形态发生蛋白(BMP)诱导髓外造血以拯救骨髓造血衰竭的作用。方法:采用60Co-γ+氯霉素(CH)+环磷酰胺(CY)的方法诱导小鼠致死性再生障碍性贫血模型,实验组于诱导再障前6d肌肉内植入重组人-BMP-4(rh-BMP-4),对照组小鼠肌肉内植入琼脂。观察血象、病理形态等变化以及两组再障小鼠的死亡率。同时,给正常小鼠大腿肌肉内植入rh-BMP-4,动态观察植入后局部的形态变化、形成骨及骨髓的形态特点,脾集落形成单位(CFU-S)以及基质细胞干细胞生长因子(SCF)的表达。结果:正常小鼠在BMP肌肉内植入1周内,在BMP周围有大量间质细胞增殖,2周后才出现软骨化骨和骨小结形成,其骨髓基质细胞的SCF表达明显高于自体骨髓;BMP植入组的再障小鼠,不仅血象与对照组相比有明显改善,而且死亡率也明显下降,有46.7%存活超过3个月,其血象完全恢复正常,骨髓和脾脏的组织形态学改变也恢复正常。对照小鼠除1只存活超过3个月外(占6.7%),其余均死于急性再生障碍性贫血。结论:肌肉内植入BMP-4可诱导髓外造血,其机制可能与BMP-4诱导成年自体肌肉内存在的干细胞向造血分化有关,这一结果将为干细胞治疗提供了一条新的思路。     

中医分型治疗慢性再生障碍性贫血临床研究

目的:探讨再造生血颗粒治疗慢性再生障碍性贫血(CAA)肾阴虚型和肾阳虚型患者的临床疗效和作用机理.方法:选取慢性再障患者40例,其中肾阴虚型22例,肾阳虚型18例.均口服再造生血颗粒0.5 a.检测治疗前后骨髓CD34 细胞bcl-2、bax蛋白的表达.结果:治疗后肾阳虚型基本治愈9例,缓解6例,明显进步2例,无效1例,有效率为90.9%.肾阳虚型分别为5例、5例、8例、4例,有效率为81.8%.两组比较,P＜0.05.肾阳虚型患者的疗效明显优于肾阴虚型者.结论:肾阳虚型治疗前bcl-2值明显高于肾阴虚型,bax则相反.     

Is the early cyclosporine A level predictive of the outcome of immunosuppressive therapy in severe aplastic anemia?

Immunosuppressive therapy (IST) has provided an alternative treatment option for cure of aplastic anemia patients who cannot receive bone marrow transplantation. Although there have been many recent studies on the efficacy of antithymoglobulin (ATG) combined with cyclosporine A (CsA), there is no data on the correlation between the variability of CsA levels and the response to IST. Therefore, we retrospectively assessed the factors associated with IST efficacy in patients with acquired severe aplastic anemia (SAA). Sixty‐six patients were treated with ATG combined with CsA for 6 months. In the response group, the CsA levels were increased rapidly to more than 200 ng/mL within the first 2 wk after starting the IST. However, the non‐response group had a pattern of slower increase of the CsA levels. The CsA levels, during the first and second week of treatment with IST, were significantly different in the responders and non‐responders. The factors predictive of response to IST and survival were analyzed. The univariate analysis showed that a younger age at the initiation of IST, a high absolute neutrophil count prior to starting IST, a short interval between the diagnosis and initiation of IST, and high CsA levels during the first and second week of IST treatment were positively associated with the response rate and overall survival. The multivariate analysis showed that these four factors were independent factors associated with a longer patient survival. A high response rate was associated with a short interval between diagnosis and initiation of IST as well as high CsA levels during the first and second week of IST. Therefore, early intensification of CsA levels might improve patient outcome.