造血干细胞移植患者腹部超声特点

目的 探讨血液病患者造血干细胞移植治疗后腹部超声特点及相关临床意义.方法 对51例接受造血干细胞移植患者进行常规腹部超声检查,观察腹腔脏器声像图变化.结果 同移植前比较,患者在移植后发生弥漫性肝实质损害、肝静脉纤细、胆囊壁增厚、胆汁淤积、脾体积缩小、肠蠕动异常及腹水等一系列变化,差异有显著性意义（P〈0.05）;而肝大小、肾实质损害、脾内部回声及胰腺变化则差异无显著性意义（P〉0.05）.结论 造血干细胞移植后患者常发生一系列腹腔脏器超声特征性变化,常规腹部超声检查能为移植相关并发症早期诊断提供及时、可靠的诊断依据.     

Allogenic hematopoietic stem cell transplantation in sickle cell disease

Sickle cell disease (SCD) is one of the most common monogenic disorders worldwide and affects approximately 100,000 people in the United States alone. SCD can cause numerous complications, including anemia, pain, stroke, and organ failure, which can lead to death. Although there are a few disease-modifying treatments available to patients with SCD, the only current curative option is a hematopoietic stem cell transplant (HSCT). In this review, we will discuss the different approaches to allogeneic HSCT in the treatment of SCD and the outcomes of these approaches.     

血小板相关参数在造血干细胞移植后巨核系重建中的应用

目的探讨血小板相关参数以了解造血干细胞移植（HSCT）在巨核系重建中的意义。方法对28例HSCT患者的巨核系重建情况与血小板相关参数进行回顾性分析。结果28例移植患者在移植后15d内骨髓处于空虚期，骨髓穿刺涂片及活检显示均未发现巨核细胞，血小板计数与移植前比较下降明显（P〈0．05）；HSCT后恢复期患者巨核系成功重建且骨髓造血功能恢复22例（22／28），骨髓穿刺涂片及活检显示巨核细胞均超过5个／片，恢复的时间平均为63．7d，巨核细胞分类以颗粒型巨核细胞为主，巨核细胞数量与BPC无明显相关（r=0．364，P〉0．1）。经多因素方差分析及t检验，恢复期BPC较空虚期明显增高，且差异存在统计学意义（P〈0．05）；空虚期、血小板输注后及恢复期的MPV之间差异无统计学意义（P〉0．05）；恢复期PDW较空虚期、血小板输注后增高，差异存在统计学意义（P〈0．05），且PDW增高早于BPC增高出现。结论HSCT后，血小板及其相关参数中，PDW变化最早且增高明显，这为停止输注血小板和骨髓穿刺证实HSCT后巨核系重建是否成功提供了一个早期的重要的实验搴判断指标。     

Evaluation of xerostomia in hematopoietic cell transplantation by a simple capacitance method device

GOALS: Hematopoietic cell transplantation (HCT) may lead to the development of xerostomia. However, there have been few reports of xerostomia in HCT patients based on objective data. We investigated moisture in the oral mucosa in patients undergoing HCT by the capacitance method using a convenient device, Moisture Checker for Mucus(R) (MCM; Life Co., Ltd., Saitama, Japan). SUBJECTS AND METHODS: Thirty-six patients undergoing HCT at Okayama University Hospital of Medicine and Dentistry (Male = 22, Female = 14; age = 41.6 +/- 16.2 years old) were enrolled in this study. Moisture in the oral mucosa was measured by MCM in accordance with the manufacturer's instructions. The results were obtained as MCM values (%), which are the weight percentage of water content in the oral mucosal epithelium. As controls, moisture of the oral mucosa was also examined in healthy volunteers (Male = 27, Female = 35; age = 43.0 +/- 14.6 years old). MAIN RESULTS: Throughout the examination period, MCM values were significantly lower in patients who underwent HCT than in controls. The degree of mucosal moisture in HCT patients showed wide interindividual differences. CONCLUSION: The degree of mucosal moisture in HCT patients was low and showed wide interindividual differences. Evaluation of xerostomia using such a device may contribute to appropriate oral care with saliva substitute.     

Three cases of Candida fermentati fungemia following hematopoietic stem cell transplantation

Bloodstream infection with non-Candida albicans Candida species is one of the serious complications among patients with hematological malignancies who receive long-term prophylactic antifungal agents. Here we describe three cases of Candida fermentati (C. fermentati) candidemia after allogeneic stem cell transplantation for hematological malignancies. Case 1 is fluconazole-breakthrough C. fermentati fungemia, which was well controlled with liposomal amphotericin B. Case 2 and 3 were caspofungin-breakthrough C. fermentati fungemia. In case 2, blood culture turned negative for Candida responding to liposomal amphotericin B. Although in vitro susceptibility data for the isolated pathogen suggested the efficacy of both caspofungin and liposomal amphotericin B in all three cases, clinically liposomal amphotericin B seemed to have been more effective for eradication of the pathogen from blood stream. C. fermentati needs to be considered as a possible cause for breakthrough candidemia among post-transplant patients with prolonged antifungal prophylaxis. Discrepancy between in vitro and in vivo susceptibility to antifungals, especially to echinocandins, might provide a clue for the optimal choice of antifungals for C. fermentati infections.     

杀伤细胞免疫球蛋白受体配型在造血干细胞移植中的应用

自然杀伤(NK)细胞在造血干细胞移植(HSCT)对于移植物植入、抵抗感染,以及抗肿瘤效应方面均起着重要作用.杀伤细胞免疫球蛋白受体(KIR)作为NK细胞上的重要受体家族,具有基因组成多样性、等位基因多态性的特点.KIR与人类白细胞抗原(HLA)-Ⅰ类分子结合后,对移植后NK细胞增殖起重要调控作用.笔者从KIR家族基因系统,相应配体及对各类型HSCT的影响方面进行综述,旨在探讨KIR配型对HSCT的临床意义.     

儿童造血干细胞移植后的生存质量研究进展

随着造血干细胞移植（HSCT）技术、造血干细胞来源体系的不断完善及支持治疗的不断改进,移植成功率也在逐年上升,越来越多的儿童从 HSCT 中获得长期生存,造血干细胞移植后患儿的生存质量在近年来也逐渐被重视。针对 HSCT 患儿生存质量的研究在国内仍为空白,而国外学者通过各种量表研究发现：（1）HSCT患儿远期总体的生存质量（QOL）良好;（2）移植前后患儿QOL变化规律：移植前患儿的QOL已经下降,且预处理后会立即进一步下降,但移植4～24个月后会提高;（3）与非移植治疗的白血病患儿相比,HSCT白血病患儿有更多的晚期不良事件,QOL低于非HSCT患儿及正常儿童;（4） HSCT患儿QOL较公认的影响因素主要包括患儿的家庭功能、患儿本身的能力（如社会功能）以及是否存在慢性移植物抗宿主病（cGVHD）,而多数研究显示移植时的年龄、性别、原发病、身高等不影响患儿的生存质量。需要指出的是,以上结论均是由国外研究得出,而我国HSCT治疗后患儿的QOL情况有待进一步研究。     

主要ABO血型不合异基因造血干细胞移植后纯红细胞再生障碍

目的 研究主要ABO血型不合异基因造血干细胞移植（allo-HSCT）后患者纯红细胞再生障碍（PRCA）的发病情况及危险因素。方法 分析移植后患者PRCA的发病危险因素，比较抗A凝集素与抗B凝集素对红系造血恢复的影响。结果 100例ABO血型主要及主次要均不合allo-HSCT患者中，12例发生PRCA。A供O者9例，A供B者1例，B供O者2例。有抗A凝集素的患者（10例）较有抗B凝集素的患者（2例）易发生PRCA（P〈0．05）。PRCA的发生不影响急性移植物抗宿主病（GVHD）或巨细胞病毒（CMV）感染的发生。发生PRCA时血型转换的中位时问为150．5d，显著长于无PRCA发生患者（60．0d）（P〈0．05）；红系恢复的中位时间为203．5d，显著长于无PRCA发生患者（76．0d）（P〈0．05）。有抗A凝集素的患者血型转换中位时间为90．0d，显著长于有抗B凝集素的患者（55．0d）（P〈0．05）；红系恢复中位时间为98．0d，长于有抗B凝集素者（80．0d）（P〉0．05），但差异无统计学意义。结论 PRCA是ABO血型不合移植的合并症之一。A供O是主要ABO血型小合allo-HSCT后PRCA发病的危险因素。     

非清髓异基因造血干细胞移植患者早期造血细胞嵌合体检测及其临床意义

目的　研究非清髓异基因造血干细胞移植 (NAST)早期造血细胞嵌合体变化规律 ,探讨分子植入 (ME)与造血恢复、移植排斥的关系。方法　应用短串联重复序列 (STR) PCR技术检测 6例血液病患者NAST后外周血及骨髓中造血细胞嵌合体。结果　NAST后 5例早期移植成功的病例其外周血造血细胞嵌合体变化特点为 :移植后 1天 ( 1天 )供者造血细胞嵌合比例为 13%～ 6 0 % , 7天嵌合比例均 >5 0 % , 14天达到完全供者造血嵌合 (FDC) , 2 1～ 2 8天嵌合比例稳定在 80 %～ 10 0 %。这 5例ME时间平均为 6天 ,较造血恢复时间提前 5d(P >0 .0 5 )。而 1例在 7天嵌合比例 <5 0 % , 14天为完全受者细胞 ,但其在 14天亦恢复自身造血。结论　NAST早期造血细胞嵌合体检测及确定ME对判断植入、预测移植排斥具有重要意义。NAST后如 7天供者造血细胞嵌合比例 <5 0 %、 14天仍未达到ME ,预示着发生移植排斥的可能性较大     

造血干细胞移植后真菌感染的防治

目的 回顾性分析我院造血干细胞移植患者感染并发症的发生及防治情况.方法 分析我院1990年9月至2000年3月收治的150例次各种类型造血干细胞移植病例感染并发症的发生及防治情况,根据移植类型、感染发生与否、预防用药、病原学及感染部位等分组并进行统计学分析.结果 150例次病例感染的发生率为89.3%(134/150),3例患者死于感染,感染病死率为2%(3/150),这3例均为真菌感染.80例预防或治疗中用过泰能或头孢他啶的病例,真菌感染发生率为32.5%(26/80),而未用过泰能或头孢他啶的病例真菌感染的发生率仅为15.7%(11/70),差异有统计学意义(X2=12.0471,P<0.05).12例真菌感染病例采用小剂量两性霉素B(10 mg/d)治疗,治愈率为100%.结论 预防性应用广谱强力的抗生素并不能减少感染发生率,反而可能增加真菌感染的机会;小剂量两性霉素B是治疗真菌感染新的、有效而安全的方法 .     

Development and current use of in hematopoietic stem cell transplantation in children and adolescents in Poland: Report of the Polish pediatric study group for hematopoietic stem cell transplantation of the Polish society for pediatric oncology and hematology

The purpose of the survey was to evaluate the development and current use of hematopoietic stem cell transplantation (HSCT) in Poland between 1989–2016. The data for analysis (indication, number of performed HSCT, HSCT type, donor type, and stem cell source, year) have been collected annually using a standardized form. In Poland, between 1989–2016, the number of pediatric transplant beds grew from one to 40 and number and rate of transplants increased annually from 1/year (0.8/10 million) to 186/year (248/10 million). During the analyzed time period 2506 HSCTs were performed, including 1718 (68.6%) allogeneic transplants (allo-HSCT) with142 in 2016 and 788 (31.4%) autologous transplants (auto-HSCT) with 44 in 2016. Among 1718 allo-HSCT, 74% were performed for malignancy (ALL 47.2%, AML 26.2%, MDS 10.8%, CML 8.1%, NHL/HD 6.1%, others 2.5%), and 26% for non-malignant disorders (SAA 41%, congenital immunodeficiencies 35.4%, hereditary bone marrow failure 16%, metabolic disorders 7%). Among 788 auto-HSCTs, 30.8% were done for hematological malignancy (NHL 41.2%, AML 23.9%, HD 17.7%, ALL 15.6%, other 1.5%), while the remaining 69.2% for solid tumors (neuroblastoma 59.8%, Ewing’s sarcoma 20.4%, other 19.8%). In Poland, between 1989–2016, the infrastructure indispensable to perform HSCT in every child with indication for this therapeutic procedure was created, and HSCT became an important part of pediatric treatment, especially in pediatric oncology, hematology, and in primary immunodeficiencies.     

血缘关系供者外周血干细胞移植中移植物细胞组分对造血重建移植物抗宿主病的影响

目的 探讨HLA全相合血缘关系供者外周血干细胞移植(PBSCT)中移植物细胞组分对恶性血液病患者移植后造血重建、移植物抗宿主病(GVHD)的影响.方法 回顾性分析我科107例接受HLA全相合血缘关系供者PBSCT的恶性血液病患者,其移植物细胞组分与移植后患者造血重建、GVHD的关系.结果 移植物各细胞组分与粒细胞重建时间无关;单个核细胞(MNC)、CD34+细胞数与血小板重建时间呈负相关(r值分别为-0.32和-0.21,P值均＜0.05).CD34+、CD34+CD38-细胞数与急性GVHD发生呈负相关(r分别为-0.24和-0.29,P值均＜0.05).淋巴细胞各亚群数量与急性GVHD发生均无明显关系.CD25+ CD4+、CD3+、CD4+ CD3+细胞数及CD4+/CD8+细胞比值与慢性GVHD发生均呈正相关(P值均＜0.05),且相关系数均大于0.4,其中CD25+ CD4+细胞数与慢性GVHD相关系数高达0.78.CD34+、CD34+ CD38-细胞数与慢性GVHD发生无明显关系.结论PBSCT中输入MNC、CD34+、CD34+ CD38-细胞数增加到一定阈值后,增加细胞数并不能进一步有效促进患者造血重建,反而有可能因输入淋巴细胞数增加而增加患者慢性GVHD、广泛慢性GVHD的发生率.     

Review of attrition and adherence in exercise studies following hematopoietic stem cell transplantation

PurposeImplementing exercise programs in people receiving high-dose chemotherapy followed by bone marrow (BMT) or hematopoietic stem cell transplantation (SCT) presents unique challenges. This review examines subject attrition rates and reasons for attrition as well as adherence to exercise interventions following BMT/SCT.MethodsStudies published between January 1985 and December 2012 that prospectively tested an exercise intervention following BMT or SCT were included in the review. Evaluation criteria included: (1) exercise modality; (2) the amount of supervision required to implement the intervention; (3) timing of the intervention; (4) subject attrition rates and reasons for attrition; and (5) exercise adherence rates.ResultsOf the 20 studies reviewed, most tested an aerobic exercise intervention or a combination of aerobic and strength training. Supervised exercise sessions were more commonly used than unsupervised sessions. The overall attrition rate was 18% for the 998 subjects enrolled in the studies. Major reasons for attrition included death, change in health status, protocol issues, personal issues with subjects, and lost to follow-up/no reason provided. Authors of supervised exercise programs rarely published exercise adherence information. Unsupervised exercise programs relied mainly on self-report to document adherence.ConclusionExercise research following BMT/SCT is becoming more sophisticated as researchers build upon the expanding literature base. Questions regarding subject attrition and adherence to exercise interventions must be addressed to identify interventions that are likely to be successful when translated into clinical practice. Subject attrition from exercise studies following BMT/SCT is relatively low. Adherence information for exercise interventions needs to be regularly addressed.     

人类白细胞抗原（HLA）基因分型在造血干细胞移植中的应用

目的提高造血干细胞移植供、受者HLA配型的精确度，有效防止移植物抗宿主病Graft—vs host disease（GVHD）的发生。方法采用准确、简便的DNA提取方法和PCR—SSP基因分型方法。建立HLA—A，B，DR基因分型方法。结果对167例临床标本进行基因水平的研究发现。DNA提取方法可以满足此项研究对样本的要求。HLAPCR—SSP基因分型方法具有良好的稳定性、可靠性和特异性。结论HLA基因分型方法准确、特异、重复性好．可作为临床造血干细胞移植配型和正常人群无关供者筛选的常规方法。对（GVHD）的发生必将起到重要的预防作用。     

非血缘关系异基因造血干细胞移植66例分析

目的对66例血液病患者进行非血缘关系异基因造血干细胞移植（allo-HSCT），探索提高移植疗效的措施。方法慢性粒细胞白血病（CML）患者24例，急性白血病（AL）患者40例，其他血液病患者2例，经预处理治疗后，进行人类白细胞抗原（HLA）基本相合的非血缘关系骨髓移植（BMT）48例，外周血干细胞移植（PBSCT）18例：部分患者采用长程加强的移植物抗宿主病（GVHD）的预防方案（将环孢菌素A提前至预处理开始时使用，同时加用霉酚酸酯）。结果64例患者达到完全稳定的供者植入，WBC植活中位时间15d（BMT组16d；PBSCT组12d，P〈0．01）。45例患者发生急性GVHD（aGVHD），累积发生率为71．16％，其中28例患者发生Ⅰ～Ⅱ度GVHD，累积发生率57．15％；17例患者发生Ⅲ～Ⅳ度GVHD，累积发生率32．25％；COX模型分析得出HLA配型及移植方式是影响aGVHD发生的因素，HLA配型相合、采用G-CSF动员的PBSCT可以降低aGVHD，尤其是重度GVHD的发生。可供分析的36例患者中有21例发生慢性GVHD。66例接受移植的患者中复发6例，死亡27例，5年的预期生存率为52．91％。用COX模型分析得出aGVHD以及aGVHD与GVHD的预防方案的交互冈素是影响生存率的惟一因素，其相对危险度分别为1.517和1.255。结论提高非血缘关系allo-HSCT疗效的关键是控制aGVHD，而选择HLA配型相合的供者，加强移植早期的免疫抑制，可以减少aGVHD的发生     

地中海贫血造血干细胞移植供体及预处理方案选择的研究进展

地中海贫血为常染色体隐性遗传疾病,是由于基因突变导致血红蛋白的a或β珠蛋白链生成障碍引起的血液系统疾病.目前,造血干细胞移植(HSCT)是唯一可以治愈地中海贫血的方法.但是,由于受到HSCT供者与患者人类白细胞抗原(HLA)相合程度的限制,以及HSCT后的移植排斥与移植相关不良反应等并发症与预处理方案相关,因此选择合适的造血干细胞供体与预处理方案,对提高HSCT治疗地中海贫血的疗效至关重要.笔者拟就无关供者HSCT、亲缘供者HSCT、脐血移植(UCBT)及预处理方案的研究现状与研究进展进行综述.     

供,受者感染乙型肝炎病毒对造血干细胞移植的影响

目的 分析血液病患移植前供、受感染乙型肝炎病毒（ＨＢＶ）对造血干细胞移植（ＨＳＣＴ０临床结果的影响。方法 对我院１９８６年１０月￣１９９８年１２月间进行ＨＳＣＴ前供、受感染ＨＢＶ的２６例患临床资料进行回顾性分析。结果 ①移植后３例患发生ＶＯＤ,发生率（１１．５％）明显高于供、受无ＨＢＶ感染的患（１．４％）（Ｐ〈０．０５）;②５例输注ＨＢｓＡｇ（＋）供造血干细胞患２例发生乙型肝炎;     

供受者乙型肝炎病毒感染对造血干细胞移植后受者临床影响的研究

目的 分析感染乙型肝炎病毒(HBV)的供、受者异基因造血干细胞移植(allo-HSCT)后,受者HBV血清学标志变化及对临床结果 的影响.方法 对2002年9月至2008年11月79例alloHSCT治疗前供、受者HBV感染患者的临床资料进行回顾性分析.结果 ①移植前供、受者HBV感染对受者预后无明显影响.②HBsAg阳性组患者20例,13例(65.0%)出现HBV激活,时间为移植后1(0.5～10)个月,9例(45.0%)并发乙肝相关肝炎.③HBsAg阴性组患者35例,4例(11.4%)移植后HBsAg转为阳性,即出现乙肝血清学转换.其中1例伴随严重慢性移植物抗宿主病(cGVHD).④HBsAg阳性组患者移植后出现HBV激活及移植后100 d内肝功能损害的比例均明显高于HBsAg阴性组患者(P<0.05).⑤2例(10.0%)HBsAg阳性患者在移植后清除体内HBV,其供者乙肝血清学标志均为HBsAb阳性.结论 供、受者HBV感染不是allo-HSCT的禁忌证;HBsAg阳性是移植后发生HBV激活的高危因素,对于这类患者应进行规范性拉米夫定预防性治疗;HBcAb/HBeAb阳性患者移植后可能出现乙肝血清学转换,在免疫抑制剂减量过程中应密切监测其乙肝血清学标志变化;alloHSCT可以通过过继免疫治疗清除患者体内HBV.     

急性白血病患者第一次完全缓解期自体和异基因造血干细胞移植疗效的比较

目的　对急性白血病 (AL) (不包括急性髓系白血病M3 型 )患者第 1次完全缓解 (CR1)期自体造血干细胞移植 (auto HSCT)和异基因造血干细胞移植 (allo HSCT)的疗效进行比较。方法　AL CR1期HSCT患者 14 0例 ,其中HLA相合同胞供者allo HSCT 4 6例 ,auto HSCT 94例 ,预处理方案包括全身照射 环磷酰胺 (TBICy)、白消安 环磷酰胺 (BuCy)以及马法兰 阿糖胞苷 环磷酰胺(MAC)方案。allo HSCT组予以环孢菌素A(CsA)或联合甲氨蝶呤 (MTX)或FK5 0 6预防移植物抗宿主病 (GVHD) ,auto HSCT组自体骨髓净化 39例 ,移植后免疫治疗和 (或 )维持化疗 38例。结果　 14 0例患者移植后均获髓系造血重建 ,中位随访时间 70 0 (18～ 5 5 6 3)d ,auto HSCT组与allo HSCT组比较 :移植后 5年无白血病生存 (LFS)率 [分别为 (5 1.5± 5 .4 ) %和 (5 2 .8± 7.6 ) % ]相近 (P >0 .0 5 ) ;累积移植相关死亡率 [分别为 (14 .4± 4 .1) %和 (37.6± 7.8) % ]后者显著增高 (P <0 .0 5 ) ;累积复发率 [分别为 (5 2 .0± 5 .5 ) %和 (2 6 .3± 6 .9) % ]前者明显增加 ,但无显著性差异 (P >0 .0 5 )。auto HSCT组中自体骨髓净化和移植后治疗患者与未经相应处理患者比较 ,5年LFS率显著提高 ,分别为 (6 2 .8± 6 .8) %和 (38.4± 8.4 ) % ,P <     

Visceral leishmaniasis in hematopoietic cell transplantation: Case report and review of the literature

Visceral leishmaniasis has been recognized as an opportunistic infection affecting people with cellular-immunity impairment, including hematopoietic cell transplantation (HCT) recipients. We describe the case of a young Italian man with Hodgkin lymphoma, who developed visceral leishmaniasis after multiple lines of chemotherapy and allogenic HCT. Literature review of visceral leishmaniasis in HCT recipients was also performed. Eleven patients (median age 50 years, 9 male) developed visceral leishmaniasis after allogenic (n = 9) and autologous (n = 2) HCT. Most of them presented with fever and pancytopenia. Bone marrow examination was the main diagnostic technique; liposomal amphotericin B was the treatment of choice. Four out of eight patients (for whom data are available) experienced visceral leishmaniasis relapse. Visceral leishmaniasis in HCT recipients is a rare event that should be suspected in patients with persistent fever, pancytopenia and possible exposure to Leishmania spp., remembering that – as well as South-East Asia, East Africa and South America - it is endemic in several European regions.