骨髓移植后纯红细胞再生障碍性贫血的特点

在HLA表型一致的异基因骨髓移植(bone marrow transplatation,BMT)中,供、受者间ABO血型不合者占10%～15%,纯红细胞再生障碍性贫血(pure red cell aplasia,PRCA)是其主要并发症之一,发病率大约为5%～16%[1,2].本文就PRCA的临床特点、发病机制及治疗作一简要综述.     

异基因外周血干细胞移植后纯红细胞再生障碍性贫血的治疗

患者男,2 3岁。因不明原因头昏、乏力、牙龈出血2周于2 0 0 2年8月2日入院。既往有乙肝病毒小三阳史,5年前在外院行先天性心房间隔缺损修补术治疗。入院后查:重度贫血貌,巩膜无黄染,口腔黏膜血泡,牙龈出血,四肢皮肤较密集出血点,淋巴结无肿大,心肺无异常,肝脾肋下未扪及。血WBC0 8×10 9/L ,中性粒细胞绝对值(ANC) 0 1×10 9/L ,Hb33g/L ,BPC8×10 9/L ,网织红细胞(Ret)计数0 0 5 % ;抗核抗体(ANA) ( ) ,Ham’s试验( ) ;乙肝病毒小三阳,HBV DNA( ) ,丙肝抗体( ) ;骨髓细胞学涂片及病理学活检均为再生障碍性贫血(AA)。临床诊断…     

纯红细胞再生障碍性贫血30例分析

纯红细胞再生障碍性贫血３０例分析福建省血液病研究所黄增敏黄明清周凡福建医科大学附属协和医院张萍容张臣青黄慧芳纯红细胞再生障碍性贫血（ＰＲＣＡ）系因红系祖细胞受损、衰竭导致红细胞系统受抑制而发生的一种贫血。特点是贫血严重，网织红细胞显著减少，白细胞和...     

成人再生障碍性贫血骨髓移植治疗的新进展

再生障碍性贫血是一种造血干细胞障碍导致外周血细胞、骨髓细胞减少的疾病。其病因复杂,治疗方法主要有骨髓移植、免疫抑制疗法及大剂量的环磷酰胺。笔者对其定义、严重性及部分治疗方法进行综述。     

贵阳医学院附院白血病,再生障碍性贫血恶性淋巴瘤患者ABO血型分布

白血病、再生障碍性贫血（简称再障）、恶性淋巴瘤是血液系统的常见病，但发病原因至今尚未完全明确。据报道胃癌等恶性肿瘤伪发病与ABO血型有一定的关联。白血病与ABO血型的关系也曾有报道。为进一步了解这三种疾病的病因与ABO血型的关系，我们调查了本院住院的白血病、再障、恶性淋巴瘤患者ABO血型的分布，并与正常人ABO血型分布比较，以探讨白血病、再障、恶性淋巴瘤与ABO血型的关系。1资料来源病人血型来源于本院确诊病人输血化验单血型检验结果。正常人血型系本院健康职工及学生。】．1病人组：全部患者均系本院住院病人。白血…     

促红细胞生成素引起纯红细胞再生障碍性贫血

近年,随着促红细胞生成素临床应用的增多,其引起纯红细胞再生障碍性贫血的报道有所增加,本文根据国外文献报道,探讨促红细胞生成素引起纯红细胞再生障碍性贫血发病机制,及其诊断、治疗和预防。     

35例纯红细胞再生障碍性贫血的实验室资料分析

纯红细胞再生障碍性贫血（PRCA）是以骨髓单纯红系造血功能衰竭为特征的一组疾病，临床上血红蛋白及网织红细胞绝对值减少，而白细胞及血小板大多正常，本病不常见。为了提高对该病诊断的认识，现将我院对年来诊治的35例PRCA分析如下。1临床资料三．且一般资料：我院于1刃5年Z月一N％年10月共行骨髓穿刺检查8630例，经外周血象及骨髓象检查确诊PRCA35例，占骨髓检查总数的O．4％。其中男性15例，女性20例，年龄8月一67岁，平均年龄万．6岁，14岁以下13例（37．l％），14一刀岁10例（28．6％），31－50岁9例（25．7％），sl岁以上3例（…     

肝炎后再生障碍性贫血

肝炎后再生障碍性贫血(肝炎后再障,HAAA)是一种发生在急性或慢性肝炎后期,以外周血全血细胞减少,骨髓三系细胞增生低下,临床以贫血、出血及感染为特征的重症疾病,治疗方法有限,死亡率高达84.1%。25岁以下患者占肝炎后再障总数的80%,与肝炎的好发年龄有关。欧美地区肝炎后再障占所有再生障碍性贫血再障(AA)的2%~5%,亚洲占4%~10%,其中病毒性肝炎后再障占所有再障的1%左右[1]。1肝炎后再生障碍性贫血的发病机制①肝炎病毒对造血干细胞的影响:肝炎病毒感染骨髓造血细胞后,某些遗传物质能与骨髓造血细胞的遗传物质整合,既可导致骨髓造血干/祖…     

双氯芬酸钠致纯红细胞再生障碍性贫血1例

患者 ,女 ,61岁 ,四肢乏力伴上腹部灼热感 10ｄ于 2 0 0 1年 5月 6日急诊入院。患者 10ｄ前在其他医院因颈椎病口服双氯芬酸钠片 [商品名 :扶他林 ,北京诺华制药有限公司生产 ,批准文号 :京卫药准字 (1996)第 110 0 0 2号 ] 1周 ,症状稍好转 ,但上腹部灼热难受 ,渐出现食欲不振 ,四肢乏力。遂到我院诊治。Ｈｂ3 5ｇ·Ｌ 1。否认近期服用其他药物史 ,否认贫血病史及家族遗传病史。体格检查 :体温 3 6.8℃ ,Ｐ 88次·ｍｉｎ 1,Ｒ 2 0次·ｍｉｎ 1,ＢＰ12 2 /66ｍｍＨｇ(1ｍｍＨｇ =0 .13 3ｋＰａ) ,重度贫血貌 ,全身皮肤、巩膜无黄染 ,全身皮…     

Acute bone marrow aplasia associated with intravenous administration of deferoxamine (desferrioxamine)

The authors report the clinical course and fatal outcome of a case of acute bone marrow aplasia, after intravenous administration of deferoxamine (desferrioxamine) to a 16-year-old girl with homozygous beta-thalassaemia. The type of aplasia was mainly that of a megakaryocytic thrombocytopenia, but the 2 other haemopoetic series were also involved. The absence of any other toxic factors and the quite rapid onset of the bone marrow failure after this type of treatment strongly suggest that intravenous administration of high doses of deferoxamine was the potential toxic factor.     

Case-control study of the association between select HLA genes and anti-erythropoietin antibody-positive pure red-cell aplasia

AIMS: Antibody (Ab)-positive pure red-cell aplasia (PRCA) is a very rare but serious adverse event associated with recombinant human erythropoietin treatment (4.1 reports per 100,000 patient-years) in which patients produce antibodies to recombinant and endogenous erythropoietin, halting red blood cell production. In a previous case series, four Thai subjects with chronic kidney disease and Ab-positive PRCA were reported to have the HLA-DRB1*9 allele. To confirm a possible association of HLA-DRB1*9 and Ab-positive PRCA, we performed a pharmacogenomic analysis using subjects from an earlier case-control study of risk factors associated with Ab-positive PRCA, which had been performed using subjects from Europe or Canada. The primary goal of the analysis was to test the association between HLA-DRB1*9 and Ab-positive PRCA. A secondary goal was to perform an exploratory analysis in order to identify additional HLA alleles potentially associated with Ab-positive PRCA. PATIENTS & METHODS: Subjects were taken from a case-control study of Ab-positive PRCA in chronic kidney disease patients treated in Europe or Canada. Ab-positive PRCA cases (n=24) were matched to controls (n=81) by timing of treatment exposure and, when possible, by location. RESULTS: The allele frequency of HLA-DRB1*9 was 12.5% in cases vs 1.2% in controls (p=0.002). The frequency of the HLA-DRB1*9/other genotype was 25.0% in cases vs 2.5% in controls (p=0.004; OR: 10.8 [95% CI: 2.2-53.7]). Within the exploratory analysis, six additional HLA alleles (HLA-A*25, HLA-B*53, HLA-C*12, HLA-DQB1*3, HLA-DQB1*6 and HLA-DRB1*4) were also found to be associated with Ab-positive PRCA. CONCLUSION: This study confirmed that HLA-DRB1*9 occurs at a significantly higher frequency in Ab-positive PRCA cases than in controls; however, within this sample set, carrying the *9 allele was neither necessary nor sufficient to cause Ab-positive PRCA.     

Immunological reconstitution after allogeneic and autologous bone marrow transplantation

Immunological reconstitution was studied with regard to T cell subsets and their functions in patients who received intensive therapy and autologous or allogeneic marrow transplantation. One of the distinct features was the imbalance of T cell subsets. Long-standing inversion of the OKT4/OKT8 ratio was characteristic in both autotransplant and allotransplant patients. Significant differences were observed in recovery of T cell subsets and mitogenic responses between autotransplant and allotransplant patients and between transplant patients and normal controls. In contrast, low reactivities of mixed lymphocyte culture (MLC) recovered to normal levels within 1 yr in both groups of patients. Then the role of suppressor cells was investigated. During early posttransplant periods, MLC suppressor cells were operative in association with the development of low MLC reactivities because suppressor activity was inversely correlated with MLC reactivities at a significant level. Characterization of these MLC suppressor cells revealed that they were an OKT8- and Ia-positive, radioresistant T cell subset of peripheral blood lymphocytes from autologous and allogeneic marrow recipients. These observations suggest that the imbalance of T cell subsets and their abnormal reactivities may by responsible for the development of immunodeficiency and immunodysregulation in bone marrow transplant patients.     

Intracerebral transplantation of bone marrow with BDNF after MCAo in rat

We tested the hypothesis that a composite graft of fresh bone marrow (BM) along with brain-derived neurotrophic factor (BDNF), transplanted into the ischemic boundary zone (IBZ) of rat brain, facilitates BM cells to survive and differentiate, and improves functional recovery after middle cerebral artery occlusion (MCAo). The fresh BM was harvested from adult rats injected with bromodeoxyuridine (BrdU) as a tracer. Rats (n=37) were subjected to 2h of MCAo, received grafts at 24h and were sacrificed at 7days after MCAo. Test groups consisted of: (1) control - MCAo alone (n=9); (2) injection of phosphate buffered saline (n=4); (3) transplantation of BM (n=8); (4) injection of BDNF (n=7); and (5) transplantation of BM with BDNF (n=9) into the IBZ. Immunohistochemistry was used to identify cells derived from the BM stem cells. Behavioral tests (rotarod motor test; adhesive-removal somatosensory test) were performed before and 7days after MCAo. The data indicate that intracerebral grafting of a combination of BM with BDNF enhances differentiation of BM cells and significantly improves motor recovery of rotarod (P<0.05) and adhesive-removal (P<0.05) tests. We anticipate that BM along with neurotrophic factors may provide a powerful autoplastic therapy for human neurological injury and degenerative disorders.     

Oral eicosapentaenoic acid for acute colonic graft-versus-host disease after bone marrow transplantation

We investigated whether pretreatment with eicosapentaenoic acid, an inhibitor of leukotriene (LT) B4, could ameliorate acute colonic graft-versus-host disease (GVHD) after bone marrow transplantation (BMT). Seventeen patients undergoing unrelated BMT were divided into two groups, with eight patients receiving eicosapentaenoic acid and nine not receiving it. The grade of GVHD after transplantation was compared with that estimated from the pretransplantation LTB4 level. The levels of LTB4 and several cytokines were also monitored. The actual grade of GVHD was lower than that estimated from LTB4 levels in three of the eight patients from the treated group, and there was a significant difference between the treated and untreated groups (p < 0.05, chi 2 test). The levels of LTB4, tumor necrosis factor-alpha (TNF-alpha), and interferon-gamma (IFN-gamma) were all significantly lower in the treated group (p < 0.05, Student's t-test). These findings suggest that eicosapentaenoic acid may ameliorate acute colonic GVHD when administered from before BMT.     

Biological significance of Ss (serum soluble) HLA-class I antigens in bone marrow transplantation

Ss (serum soluble) HLA-class I antigens were investigated in 10 cases of bone marrow transplantation and the relationships among Ss HLA antigens, lymphocyte counts, GvHD and the clinical course were compared. Cs (cell surface) HLA antigens were typed by the NIH standard microcytotoxicity test. Measurement of Ss HLA antigens was performed by both the lymphocytotoxicity inhibition test and solid phase RIA. A good correlation was found between the cytotoxicity inhibition test and solid phase RIA. No correlation was observed between lymphocyte counts and changes in the Ss HLA antigens in the presence of GvHD. In cases with GvHD, Ss HLA antigens were found to correlate with the GvHD. Ss HLA antigens also correlated with levels of lymphocyte counts in cases without GvHD. Ss HLA antigens are an important marker of biological significance in GvHD following bone marrow transplantation.