异基因外周造血干细胞移植与免疫抑制治疗重型再生障碍性贫血的临床比较

目的 比较异基因外周造血干细胞移植(Allo-PBSCT)与免疫抑制治疗(IST)重型再生障碍性贫血(SAA)的疗效与并发症.方法 25例SAA患者接受了两种方法治疗:PBSCT组12例接受了同胞HLA全相合的PBSCT,预处理方案为:环磷酰胺(Cy)联合兔抗人胸腺免疫球蛋白(ATG);IST组13例治疗方案为:ATG/环孢素A(CsA).比较两种治疗方法的近期疗效、远期疗效与并发症.结果 PBSCT组的中性粒细胞计数、Pt和Hb的恢复时间[分别为(13.5±2.3)、(23.5±4.1)、(82.7±6.1)d]快于IST组[分别为(32.6±3.5)、(73.8±6.2)、(296.4±12.5)d](P<0.05),但1年的近期疗效比较差异无统计学意义(P>0.05).两组3年生存率和总生存率比较差异均无统计学意义(P>0.05),总复发率比较差异有统计学意义(P<0.05).结论 Allo-PBSCT和IST都是治疗SAA的有效手段,但Allo-PBSCT具有造血重建快、复发率低、并发症并没有增加等特点,临床上可作为优先选择.     

Incidence of aplastic anemia in Turkey: A hospital-based prospective multicentre study

The incidence of aplastic anemia among hospitalized adult patients was prospectively determined in this first study in Turkey. New cases of aplastic anemia among patients 14 years and older who were admitted to the study centers were included in a 3 year survey. Seventy-three patients fulfilled the diagnostic criteria, yielding a mean annual incidence rate of 1.14 cases in 10³ admissions. The male-to-female ratio of the cases (1.6:1) differed from the almost equal ratio of the larger population of Turkey. The median age was 30 years and females were younger at diagnosis. The age distribution of the cases was different from that of the population; showing two incidence peaks in both sexes. The majority of the patients (89%) had severe disease.     

Antihypertensive drugs and the risk of idiopathic aplastic anaemia

AIMS: A recent report has raised concern that nifedipine may be associated with an increased risk of aplastic anaemia. This large population-based study evaluated the risk of idiopathic aplastic anaemia in users of calcium channel blockers compared with that of other antihypertensive drugs. METHODS: The study was based on information derived from the General Practice Research Database. We conducted a follow-up study with a nested case-control analysis of 322 448 subjects who received antihypertensive drugs. Cases were people who had a first-time diagnosis of aplastic anaemia during January 1, 1988 through September 30, 1997. The risk estimate of aplastic anaemia was calculated for all antihypertensive drugs. For the nested case-control analysis, six controls were matched to each case on age, sex and general practice attended. Odds ratios compared the risk of idiopathic aplastic anaemia for all antihypertensive drugs relative to nonusers. RESULTS: There were 13 cases of newly diagnosed idiopathic aplastic anaemia. The estimated risk of aplastic anaemia per 100 000 users was 0.8 (95% CI 0.1, 4.7) for calcium channel blockers, 1.4 (95% CI 0.5, 4.1) for beta-adrenoceptor blockers, 2.3 (95% CI 0.6, 8.6) for angiotension-converting enzyme (ACE) inhibitors and 5.9 (95% CI 1.6, 21.5) for users of other antihypertensive drugs. In the case-control analysis of 13 cases and 77 controls, the odds ratio was 0.3 (95% CI 0.02, 3.3) for calcium channel blockers, 0.5 (95% CI 0.1, 2.5) for beta-adrenoceptor blockers, 0.7 (95% CI 0.1, 5.6) for ACE inhibitors, 1.2 (95% CI 0.1, 11.8) for users of other antihypertensive drugs and 0.7 (95% CI 0.1, 7.2) for users of multiple drugs with a calcium channel blocker compared with nonusers. CONCLUSIONS: The present study suggests that the use of calcium channel blockers is not associated with an increased risk of aplastic anaemia.     

A Case of Severe Aplastic Anaemia Successfully Treated by Retransplantation from the Same Donor

A 16-year-old girl with severe aplastic anaemia was successfully treated with retransplantation of bone marrow from an HLA-identical sibling after rejection of the first transplantation from the same donor. Cyclophosphamide was used for the first transplantation and cyclophosphamide, 300 rad total-body irradiation and antilymphocyte globulin were used for the second transplantation. Permanent engraftment was achieved after the retransplantation with normalization of haemopoiesis, which has lasted for over 17 months. The patient is now in excellent clinical condition with minimal signs of chronic graft versus host disease.     

Successful recovery of aplastic anemia following orthotopic liver transplantation for non-A-E acute liver failure

This report describes a teenager who developed aplastic anemia (AA) because of non-A-E acute liver failure (ALF) requiring orthotopic liver transplantation (OLT). His AA did not recover spontaneously and he required treatment with ATG 9 months post-OLT. Bone marrow recovery occurred 4 months after immunotherapy and coincided with further intensification of immunusuppression required to treat early chronic rejection of the liver graft. Three years post-OLT he remains well with good bone marrow and liver function. Intensification of immunosuppression can lead to successful resolution of AA associated with non-A-E ALF.     

Analysis of T-cell repertoire and mixed chimaerism in a patient with aplastic anaemia after allogeneic bone marrow transplantation

We analysed 26 T-cell receptor (TCR) beta chain subfamilies (VB) of a patient with aplastic anaemia (AA) who underwent allogeneic bone marrow transplantation (allo-BMT). The patient developed pancytopenia at d 80. The patient's T cells were skewed in 10 of 26 TCR-VB on d 83. These TCR-VB, especially VB15, which were almost entirely CD8-positive cells, were skewed throughout her clinical course. Chimaerism analysis of the CD8-positive cells indicated that they were of recipient origin. Therefore, some immune responses induced by the recipient CD8-positive T cells had an important role in pancytopenia in AA patients after allo-BMT.     

干细胞因子受体基因在再生障碍性贫血患儿中的表达

目的探讨再生障碍性贫血（再障）患儿造血干细胞因子受体（C-KIT）基因Exon9、11、13的表达和序列构象及与发病机制的关系。方法应用逆转录-聚合酶链反应（RT-PCR）和聚合酶链反应单链构象多态分析（PCR-SSCP）和序列测定技术,测定再障与正常对照儿童各15例C-KIT基因Exon9、11、13的表达水平和结构是否存在变异。结果1.再障组C-KIT基因Exon9、11、13阳性表达率（40.0%）与正常对照组（46.67%）比较无显著性差异（P〉0.05）;2.经PCR-SSCP分析和基因序列测定C-KIT基因Exon9、11、13均未见碱基序列改变或碱基数目增多、缺失。结论C-KIT基因Exon9、11、13表达与儿童再障发病关系不大,提示儿童再障的发病可能不是由于Exon9、11、13突变引起。     

再生障碍性贫血的免疫抑制治疗

免疫抑制疗法是再生障碍性贫血的主要治疗方法，本文简要综述其主要药物的作用机制、应用方法，合理用量及疗效、相互之间的协同作用。所综述的药物包括抗人胸腺球蛋白／抗淋巴细胞球蛋白(ATG／ALG)，环孢菌素A(CSA)，ALG／ATG与CSA联合应用，强化免疫抑制治疗联合造血生长因子(IIST HGFs)，抗T淋巴细胞单克隆抗体(McAb—T)，大剂量环磷酰胺(HD—CTX)，大剂量甲基强的松龙(HD—MP)和大剂量免疫球蛋白(HD—IVIG)。本综述目的是指导再生障碍性贫血患者的临床治疗。     

补髓生血冲剂对慢性再障IL-6 IL-8水平的影响

应用补髓生血冲剂治疗慢性再生障碍性贫血 (简称CAA)患者 ,观察其骨髓造血细胞IL 6及IL 8水平的变化。采用原位杂交法检测治疗前后阳性细胞指数变化 ,并与正常组相比较。结果 ,治疗组疗前IL 6、IL 8阳性细胞指数明显高于正常组 ,疗后较疗前指数明显降低 ,与正常组相比差异不显著。IL 6及IL 8水平异常与CAA发病有关。补髓生血冲剂对这两种细胞因子有调节作用