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[The impact of donor human leukocyte antigen-Bw4 allele on natural killer cell reconstitution and transplant-related mortality in haploidentical transplantation]. [供体人类白细胞抗原-Bw4 等位基因对单倍体移植中自然杀伤细胞重建和移植相关死亡率的影响]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn.121090-20240314-00094
M Zhao, Z L Xu, X X Yu, Y Y Ding, Y J Chang, X H Zhang, K Y Liu, X J Huang, X Y Zhao

Objective: To investigate the impact of donor human leukocyte antigen (HLA) -Bw4 expression on natural killer (NK) cell reconstitution and transplant outcomes in recipients undergoing haploidentical hematopoietic stem cell transplantation (HSCT) from maternal or related donors without ex vivo T-cell depletion. Methods: This study prospectively enrolled 32 patients who received T-replete haploidentical HSCT from maternal or collateral donors (cohort 1) to evaluate the facilitating effect of donor HLA-Bw4 expression on NK cell reconstitution. Furthermore, a retrospective analysis was conducted on 278 patients who underwent T-replete haploidentical HSCT from maternal or collateral donors (cohort 2) to analyze the impact of donor HLA-Bw4 expression on HSCT outcomes. Thus, a comparison was made between the effects of donor HLA-Bw4 expression on HSCT outcomes in patients receiving or not receiving post-transplant cyclophosphamide (PT-Cy) conditioning. Results: Donors expressing HLA-Bw4 alleles facilitated NK cell reconstitution and functional recovery, which remained unaffected by PT-Cy. Donors with HLA-Bw4 expression were associated with reduced transplant-related mortality (TRM), particularly mortality related to infections. The use of PT-Cy did not impact the ability of donor HLA-Bw4 to decrease TRM. Conclusion: In haploidentical HSCT from maternal or related donors without ex vivo T-cell depletion, the presence of donor HLA-Bw4 expression promotes rapid NK cell reconstitution and functional recovery and is significantly associated with lower TRM, especially infection-related mortality. These findings underscore the clinical significance of donor HLA-Bw4 expression in patients who underwent HSCT. Hence, the consideration of donor HLA-Bw4 in recipient selection and HSCT strategies holds important clinical implications.

目的研究供体人类白细胞抗原(HLA)-Bw4表达对自然杀伤细胞(NK)重建和接受母体或亲缘供体单倍体造血干细胞移植(HSCT)的受者移植结果的影响。研究方法这项研究前瞻性地招募了32名接受母体或旁系供者T完全性单倍体造血干细胞移植的患者(队列1),以评估供者HLA-Bw4表达对NK细胞重建的促进作用。此外,还对278名接受母体或旁系供体T完全性单倍体造血干细胞移植的患者(队列2)进行了回顾性分析,以分析供体HLA-Bw4表达对造血干细胞移植结果的影响。因此,在接受或不接受移植后环磷酰胺(PT-Cy)调理的患者中,比较了供者 HLA-Bw4 表达对造血干细胞移植结果的影响。研究结果表达HLA-Bw4等位基因的供体有助于NK细胞重建和功能恢复,而PT-Cy对其没有影响。表达HLA-Bw4的供体可降低移植相关死亡率(TRM),尤其是与感染相关的死亡率。使用 PT-Cy 并不影响供体 HLA-Bw4 降低 TRM 的能力。结论在无体内T细胞耗竭的母体或亲缘供体单倍体造血干细胞移植中,供体HLA-Bw4的表达可促进NK细胞的快速重建和功能恢复,并与较低的TRM(尤其是感染相关死亡率)显著相关。这些发现强调了供体 HLA-Bw4 表达在造血干细胞移植患者中的临床意义。因此,在受体选择和造血干细胞移植策略中考虑供体 HLA-Bw4 具有重要的临床意义。
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引用次数: 0
[The prognostic value of colonoscopy grading for acute graft-versus-host disease in patients with malignant hematological disorders after unrelated cord blood transplantation]. [非亲缘脐带血移植后恶性血液病患者急性移植物抗宿主病的结肠镜检查分级的预后价值]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn121090-20231206-00293
S L Wang, G Y Sun, X Y Zhu, X M Xu, F Ye, S L Li, S Chen

Objective: To investigate the prognostic value of enteroscopic grading for the prognostic assessment of patients with malignant hematological diseases who developed intestinal acute graft-versus-host disease (IT-aGVHD) after unrelated cord blood transplantation (UCBT) . Methods: Fifty patients with IT-aGVHD who developed hormone resistance after UCBT from June 2016 to June 2023 at Anhui Provincial Hospital were collected to compare the effective and survival rates of IT-aGVHD treatment in the group with milder enteroscopic mucosal injury (27 cases, enteroscopic grading of Ⅰ and Ⅱ) and the group with more severe injury (23 cases, enteroscopic grading of Ⅲ and Ⅳ) and to retrospectively analyze the factors affecting patients' prognosis. Results: Patients in the mild and severe groups had an effective rate of 92.6% and 47.8% at 28 days after colonoscopy (P<0.001), 81.5% and 39.1% at 56 days after colonoscopy (P=0.002), with optimal effective rate of 92.6% and 65.2% (P=0.040), respectively, and the differences were statistically significant. The multifactorial analysis found that enteroscopic grading was an independent risk factor affecting the effective rate of IT-aGVHD treatment. The overall survival rate at 2 years after colonoscopy was 70.4% (95% CI 52.0% -88.8% ) and 34.8% (95% CI 14.8% -54.8% ) for patients in the mild and severe groups, respectively, and the difference was statistically significant (P=0.003). Multifactorial analysis revealed that enteroscopic grading, cytomegalovirus infection status, second-line treatment regimen, and patients' age were independent risk factors for survival. Conclusion: The treatment efficacy and prognosis of patients in the group with less severe enteroscopic injury (grades Ⅰ and Ⅱ) were better than those in the group with more severe injury (grades Ⅲ and Ⅳ) .

目的研究肠镜分级对非亲缘脐带血移植(UCBT)后发生肠道急性移植物抗宿主病(IT-aGVHD)的恶性血液病患者预后评估的价值。方法:收集2016年6月至2023年6月安徽省立医院50例UCBT后出现激素抵抗的IT-aGVHD患者,比较肠镜下黏膜损伤较轻组(27例、肠镜分级为Ⅰ级和Ⅱ级的27例)与损伤较重的23例(肠镜分级为Ⅲ级和Ⅳ级的23例)进行比较,并回顾性分析影响患者预后的因素。结果轻度组和重度组患者在结肠镜检查后28天的有效率分别为92.6%和47.8%(PP=0.002),最佳有效率分别为92.6%和65.2%(P=0.040),差异有统计学意义。多因素分析发现,肠镜分级是影响 IT-aGVHD 治疗有效率的独立危险因素。轻度组和重度组患者结肠镜检查后2年的总生存率分别为70.4%(95% CI 52.0% -88.8%)和34.8%(95% CI 14.8% -54.8%),差异有统计学意义(P=0.003)。多因素分析显示,肠镜分级、巨细胞病毒感染状况、二线治疗方案和患者年龄是影响生存率的独立风险因素。结论肠镜损伤较轻组(Ⅰ级和Ⅱ级)患者的疗效和预后均优于损伤较重组(Ⅲ级和Ⅳ级)患者。
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引用次数: 0
[Chinese guideline for diagnosis and treatment of adult acute lymphoblastic leukemia (2024)]. [中国成人急性淋巴细胞白血病诊断和治疗指南(2024 年)]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn121090-20240319-00102

Adult acute lymphoblastic leukemia (ALL) is one of the most common acute leukemia in adults. There are relatively unified diagnostic criteria and systematic treatment regimens reported by different research groups in the world. Since 2013, three versions of expert consensus/guidelines on the diagnosis and treatment of adult ALL in China have been published, which are of great significance for improving the level of diagnosis and treatment of this disease. In 2022, the classification of ALL (precursor lymphocyte neoplasms-lymphoblastic leukemias/lymphomas) had been updated in the WHO classification of haematolymphoid tumors, and some new concepts had been proposed. In recent years, the rapid development of immunotherapy has improved the efficacy of adult ALL, and commercial antibodies and CAR-T cell products have been available in China, the clinical practice is increasing. In order to promote the standardization of clinical diagnosis and treatment of adult ALL, by referring to the latest guidelines and literatures all over the world, this guideline may contribute to the better understanding of diagnosis, treatment and efficacy monitoring for adult ALL.

成人急性淋巴细胞白血病(ALL)是成人中最常见的急性白血病之一。目前,世界上不同研究组报道了相对统一的诊断标准和系统的治疗方案。自2013年以来,我国成人ALL诊治专家共识/指南已发布了三个版本,对提高该病的诊治水平具有重要意义。2022年,WHO血液淋巴肿瘤分类中更新了ALL(前体淋巴细胞瘤-淋巴细胞白血病/淋巴瘤)的分类,并提出了一些新的概念。近年来,免疫治疗的快速发展提高了成人 ALL 的疗效,商业抗体和 CAR-T 细胞产品已在中国上市,临床实践也在不断增加。为了促进成人ALL临床诊治的规范化,本指南参考了国际上最新的指南和文献,以期对成人ALL的诊断、治疗和疗效监测有更好的认识。
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引用次数: 0
[Clinicopathologic characteristics and survival analysis of primary large B-cell lymphoma of the central nervous system]. [中枢神经系统原发性大 B 细胞淋巴瘤的临床病理特征和存活率分析]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn121090-20231126-00278
Q F Xu, R Shen, Y G Shen, Y W Cao, Y Qian, P P Xu, S Cheng, L Wang, W L Zhao

Objective: To retrospectively analyze the clinical and pathologic characteristics, response to treatment, survival, and prognosis of patients with primary large B-cell lymphoma of the central nervous system (PCNSLBCL) . Methods: Clinical and pathologic data of 70 patients with PCNSLBCL admitted to Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from December 2010 to November 2022 were collected for retrospective analysis. Survival analysis was performed using the Kaplan-Meier method and log-rank test, and prognosis analysis was conducted using the Cox proportional hazards model. Results: Among 70 patients with PCNSLBCL, complete remission (CRs) were achieved in 49 (70.0% ) and partial remission in 4 (5.7% ) after the first-line induction therapy; the overall remission rate was 75.7%. The 2-year progression-free survival (PFS) rate was 55.8% and the median progression-free survival (mPFS) time was 35.9 months, whereas the 2-year overall survival (OS) rate was 79.1% with a median OS time not reached. After CR induced by first-line therapy, cumulative incidence of relapse (CIR) was lower in patients who had received auto-HSCT than in those who had not received consolidation therapy (P=0.032), whose 2-year PFS rate was 54.4% and mPFS time was 35.9 months; comparatively, the 2-year PFS rate in patients having received oral maintenance of small molecule drugs reached 84.4% with a mPFS time of 79.5 months (P=0.038). Multivariant analysis demonstrated that Class 3 in the Memorial Sloan-Kettering Cancer Center (MSKCC) prognostic model is an independent adverse prognostic factor of OS in patients with PCNSLBCL (HR=3.127, 95% CI 1.057-9.253, P=0.039) . Conclusions: In patients with PCNSLBCL achieving CR after the first-line induction therapy, auto-HSCT as consolidation therapy would lead to a decreased CIR, and PFS time could be prolonged by oral maintenance of small molecule drugs. Class 3 MSKCC prognostic model is independently associated with poorer OS.

目的回顾性分析中枢神经系统原发性大B细胞淋巴瘤(PCNSLBCL)患者的临床和病理特征、对治疗的反应、生存率和预后。研究方法收集2010年12月至2022年11月期间上海交通大学医学院附属瑞金医院收治的70例中枢神经系统原发性大B细胞淋巴瘤(PCNSLBCL)患者的临床和病理资料,进行回顾性分析。采用Kaplan-Meier法和log-rank检验进行生存期分析,采用Cox比例危险度模型进行预后分析。结果70名PCNSLBCL患者中,49人(70.0%)在一线诱导治疗后获得完全缓解(CR),4人(5.7%)获得部分缓解;总缓解率为75.7%。2年无进展生存期(PFS)率为55.8%,中位无进展生存期(mPFS)时间为35.9个月,而2年总生存期(OS)率为79.1%,中位OS时间尚未达到。一线治疗诱发CR后,接受过自体HSCT的患者的累积复发率(CIR)低于未接受巩固治疗的患者(P=0.032),后者的2年PFS率为54.4%,mPFS时间为35.9个月;相比之下,接受过小分子药物口服维持治疗的患者的2年PFS率达到84.4%,mPFS时间为79.5个月(P=0.038)。多变量分析表明,纪念斯隆-凯特琳癌症中心(MSKCC)预后模型中的3级是影响PCNSLBCL患者OS的独立不良预后因素(HR=3.127,95% CI 1.057-9.253, P=0.039)。结论对于一线诱导治疗后达到CR的PCNSLBCL患者,自体HSCT作为巩固治疗将导致CIR下降,口服小分子药物可延长PFS时间。MSKCC 3级预后模型与较差的OS独立相关。
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引用次数: 0
[High-risk acute myeloid leukemia with DEK-NUP214 rearrangement and eyelid infiltration: a case report]. [伴有 DEK-NUP214 重排和眼睑浸润的高危急性髓性白血病:病例报告]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn121090-20231207-00297
X Z Li, R Huang, T Q Gao, X W Xie, Y Q Zhou, Q L Zeng, J D Liu, X K Gu, Z H Liu
{"title":"[High-risk acute myeloid leukemia with DEK-NUP214 rearrangement and eyelid infiltration: a case report].","authors":"X Z Li, R Huang, T Q Gao, X W Xie, Y Q Zhou, Q L Zeng, J D Liu, X K Gu, Z H Liu","doi":"10.3760/cma.j.cn121090-20231207-00297","DOIUrl":"10.3760/cma.j.cn121090-20231207-00297","url":null,"abstract":"","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 5","pages":"512"},"PeriodicalIF":0.0,"publicationDate":"2024-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11270490/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141535497","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Analysis of the efficacy and prognostic factors of allogeneic hematopoietic stem cell transplantation in patients with myelodysplastic syndrome with blastomycosis and survival comparison of different subtypes after the WHO 2022 reclassification]. [骨髓增生异常综合征伴泡霉菌病患者异基因造血干细胞移植的疗效和预后因素分析,以及世卫组织 2022 年重新分类后不同亚型的存活率比较]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn121090-20231109-00258
H Wang, R Z Ma, A M Pang, D L Yang, X Chen, R L Zhang, J L Wei, Q L Ma, W H Zhai, Y He, E L Jiang, M Z Han, S Z Feng
<p><p><b>Objective:</b> To evaluate the efficacy and prognostic factors of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with myelodysplastic syndrome accompanied by myelodysplasia (MDS-EB) and to compare the prognosis of different subtypes of patients classified by World Health Organization (WHO) 2022. <b>Methods:</b> A total of 282 patients with MDS-EB who underwent allo-HSCT at the Hematology Hospital of the Chinese Academy of Medical Sciences from October 2006 to December 2022 were included in the study. The WHO 2022 diagnostic criteria reclassified MDS into three groups: myelodysplastic tumors with type 1/2 of primitive cell proliferation (MDS-IB1/IB2, 222 cases), MDS with fibrosis (MDS-f, 41 cases), and MDS with biallelic TP53 mutation (MDS-biTP53, 19 cases). Their clinical data were retrospectively analyzed. <b>Results:</b> ① The median age of 282 patients was 46 (15-66) years, with 191 males and 91 females. Among them, 118 (42% ) and 164 (58% ) had MDS-EB1 and MDS-EB2, respectively. ②Among the 282 patients, 256 (90.8% ) achieved hematopoietic reconstruction after transplantation, with 11 (3.9% ) and 15 (5.3% ) having primary and secondary implantation dysfunctions, respectively. The cumulative incidence of acute graft-versus-host disease (GVHD) 100 days post-transplantation was (42.6±3.0) %, and the cumulative incidence of grade Ⅱ-Ⅳ acute GVHD was (33.0±2.8) %. The cumulative incidence of chronic GVHD 1 year post-transplantation was (31.0±2.9) %. Post-transplantation, 128 (45.4% ), 63 (22.3% ), 35 (12.4% ), and 17 patients (6.0% ) developed cytomegalovirus infection, bacteremia, pulmonary fungal infection, and Epstein-Barr virus infection. ③The median follow-up time post-transplantation was 22.1 (19.2-24.7) months, and the 3-year overall survival (OS) and disease-free survival (DFS) rates were 71.9% (95% <i>CI</i> 65.7% -78.6% ) and 63.6% (95% <i>CI</i> 57.2% -70.7% ), respectively. The 3-year non-recurrent mortality rate (NRM) is 17.9% (95% <i>CI</i> 13.9% -22.9% ), and the 3-year cumulative recurrence rate (CIR) is 9.8% (95% <i>CI</i> 6.7% -13.7% ). The independent risk factors affecting OS post-transplantation include monocyte karyotype (<i>P</i>=0.004, <i>HR</i>=3.26, 95% <i>CI</i> 1.46-7.29), hematopoietic stem cell transplantation complication index (HCI-CI) of ≥3 points (<i>P</i><0.001, <i>HR</i>=2.86, 95% <i>CI</i> 1.72-4.75), and the occurrence of acute gastrointestinal GVHD of grade Ⅱ-Ⅳ (<i>P</i><0.001, <i>HR</i>=5.94, 95% <i>CI</i> 3.50-10.10). ④The 3-year OS and DFS rates in the MDS-IB1/IB2 group post-transplantation were better than those in the MDS-biTP53 group [OS: 72.0% (95% <i>CI</i> 63.4% -80.7% ) <i>vs</i> 46.4% (95% <i>CI</i> 26.9% -80.1% ), <i>P</i>=0.020; DFS: 67.4% (95% <i>CI</i> 60.3% -75.3% ) <i>vs</i> 39.7% (95% <i>CI</i> 22.3% -70.8% ), <i>P</i>=0.015]. The 3-year CIR was lower than that of the MDS-biTP53 group [7.3% (95% <i>CI</i> 4.3% -11.4% ) <i>vs</i> 26.9% (95% <i>CI</i> 9.2% -4
目的评估伴骨髓增生异常综合征(MDS-EB)患者异基因造血干细胞移植(allo-HSCT)的疗效和预后因素,并比较世界卫生组织(WHO)2022年划分的不同亚型患者的预后。研究方法研究共纳入2006年10月至2022年12月期间在中国医学科学院血液病医院接受allo-HSCT的282例MDS-EB患者。根据WHO 2022年的诊断标准,MDS被重新分为三组:原始细胞增生1/2型骨髓增生异常肿瘤(MDS-IB1/IB2,222例)、伴纤维化的MDS(MDS-f,41例)和伴TP53双倍突变的MDS(MDS-biTP53,19例)。对他们的临床数据进行了回顾性分析。结果282 例患者的中位年龄为 46(15-66)岁,男性 191 例,女性 91 例。其中,MDS-EB1 和 MDS-EB2 患者分别为 118 人(42%)和 164 人(58%)。282名患者中,256人(90.8%)在移植后实现了造血重建,分别有11人(3.9%)和15人(5.3%)出现原发性和继发性植入功能障碍。移植后100天急性移植物抗宿主病(GVHD)的累计发生率为(42.6±3.0)%,Ⅱ-Ⅳ级急性GVHD的累计发生率为(33.0±2.8)%。移植后1年的慢性GVHD累积发生率为(31.0±2.9)%。移植后分别有 128 例(45.4%)、63 例(22.3%)、35 例(12.4%)和 17 例(6.0%)患者出现巨细胞病毒感染、菌血症、肺部真菌感染和 Epstein-Barr 病毒感染。移植后的中位随访时间为 22.1(19.2-24.7)个月,3 年总生存率(OS)和无病生存率(DFS)分别为 71.9%(95% CI 65.7% -78.6% )和 63.6%(95% CI 57.2% -70.7% )。3年非复发死亡率(NRM)为17.9%(95% CI 13.9% -22.9%),3年累积复发率(CIR)为9.8%(95% CI 6.7% -13.7%)。影响移植后 OS 的独立危险因素包括单核细胞核型(P=0.004,HR=3.26,95% CI 1.46-7.29)、造血干细胞移植并发症指数(HCI-CI)≥3分(PHR=2.86,95% CI 1.72-4.75)和发生Ⅱ-Ⅳ级急性胃肠道GVHD(PHR=5.94,95% CI 3.50-10.10)。MDS-IB1/IB2组移植后的3年OS和DFS率优于MDS-biTP53组[OS:72.0% (95% CI 63.4% -80.7% ) vs 46.4% (95% CI 26.9% -80.1% ), P=0.020;DFS:67.4% (95% CI 60.3% -75.3% ) vs 39.7% (95% CI 22.3% -70.8% ), P=0.015]。3年CIR低于MDS-biTP53组[7.3% (95% CI 4.3% -11.4% ) vs 26.9% (95% CI 9.2% -48.5%),P=0.004]。MDS-IB1/IB2组、MDS-f组和MDS-biTP53组移植后3年的NRM分别为16.7%(95% CI 12.1% -22.1%)、20.5%(95% CI 9.4% -34.6%)和26.3%(95% CI 9.1% -47.5%)(P=0.690)。结论单体核型、HCI-CI和Ⅱ-Ⅳ级急性胃肠道GVHD是影响患者OS的独立危险因素。WHO 2022分级有助于区分allo-HSCT在不同亚组患者中的疗效。allo-HSCT可以改善MDS-f患者的不良预后,但MDS-biTP53患者移植后复发的风险更高。
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引用次数: 0
[Progress of allogeneic hematopoietic stem cell transplantation in KMT2A-rearranged acute leukemia]. [异基因造血干细胞移植治疗 KMT2A 重排急性白血病的进展]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn121090-20231026-00230
J Liu, X J Huang

KMT2A (lysine methyltransferase 2A) -rearranged acute leukemia is a class of leukemia with unique biological characteristics with moderate or poor prognosis. In recent years, allogeneic hematopoietic stem cell transplantation (allo-HSCT) has been increasingly indicated for patients with KMT2A-rearranged acute leukemia. By reviewing the clinical studies of allo-HSCT in KMT2A-rearranged acute leukemia, the efficacy of allo-HSCT in children and adults with KMT2A-rearranged acute myeloid leukemia and acute lymphoblastic leukemia was assessed, the factors affecting the prognosis of allo-HSCT were summarized, and the methods that may improve the outcomes of allo-HSCT were explored.

KMT2A(赖氨酸甲基转移酶2A)重排急性白血病是一类具有独特生物学特征的白血病,预后中等或较差。近年来,异基因造血干细胞移植(allo-HSCT)越来越多地用于 KMT2A 重排急性白血病患者。通过回顾KMT2A重排急性白血病异基因造血干细胞移植的临床研究,评估了异基因造血干细胞移植在儿童和成人KMT2A重排急性髓性白血病和急性淋巴细胞白血病患者中的疗效,总结了影响异基因造血干细胞移植预后的因素,并探讨了可改善异基因造血干细胞移植预后的方法。
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引用次数: 0
[Relapsed/refractory idiopathic multicentric Castleman disease successfully treated with sirolimus: a case report]. [西罗莫司成功治疗复发/难治性特发性多中心卡斯特曼病:病例报告]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn121090-20240119-00034
Y H Gao, L Zhang, J Li
{"title":"[Relapsed/refractory idiopathic multicentric Castleman disease successfully treated with sirolimus: a case report].","authors":"Y H Gao, L Zhang, J Li","doi":"10.3760/cma.j.cn121090-20240119-00034","DOIUrl":"10.3760/cma.j.cn121090-20240119-00034","url":null,"abstract":"","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 5","pages":"513"},"PeriodicalIF":0.0,"publicationDate":"2024-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11270497/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141535499","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[The efficacy and safety of protein A immunoadsorption combined with rituximab treatment for highly sensitized patients undergoing haplo-hematopoietic stem cell transplantation]. [蛋白 A 免疫吸附联合利妥昔单抗治疗接受单倍体造血干细胞移植的高度致敏患者的有效性和安全性]。
Q3 Medicine Pub Date : 2024-05-14 DOI: 10.3760/cma.j.cn121090-20231125-00277
L Li, W J Zhu, Q Zhu, S Y Zhou, C Ma, J Wang, X H Hu, Y Han, Y Wang, X W Tang, X Ma, S N Chen, H Y Qiu, L Y Chen, J He, D P Wu, X J Wu

Objective: To investigate the efficacy and safety of protein A immunoadsorption (PAIA) combined with rituximab (RTX) in highly sensitized patients who underwent haplo-hematopoietic stem cell transplantation (haplo-HSCT) . Methods: The clinical data of 56 highly sensitized patients treated with PAIA and RTX before haplo-HSCT at the First Affiliated Hospital of Soochow University and Soochow Hopes Hematonosis Hospital between March 2021 and June 2023 were retrospectively analyzed. The number of human leukocyte antigen (HLA) antibody types and the mean fluorescence intensity (MFI), humoral immunity, adverse reactions during adsorption, and survival within 100 days before and after adsorption were measured. Results: After receiving the PAIA treatment, the median MFI of patients containing only HLA Ⅰ antibodies decreased from 7 859 (3 209-12 444) to 3 719 (0-8 275) (P<0.001), and the median MFI of HLA Ⅰ+Ⅱ antibodies decreased from 5 476 (1 977-12 382) to 3 714 (0-11 074) (P=0.035). The median MFI of patients with positive anti-donor-specific antibodies decreased from 8 779 (2 697-18 659) to 4 524 (0-15 989) (P<0.001). The number of HLA-A, B, C, DR, and DQ antibodies in all patients decreased after the PAIA treatment, and the differences were statistically significant (A, B, C, DR: P<0.001, DQ: P<0.01). The humoral immune monitoring before and after the PAIA treatment showed a significant decrease in the number of IgG and complement C3 (P<0.001 and P=0.002, respectively). Forty-four patients underwent HLA antibody monitoring after transplantation, and the overall MFI and number of antibody types decreased. However, five patients developed new antibodies with low MFI, and nine patients continued to have high MFI. The overall survival, disease-free survival, non-recurrent mortality, and cumulative recurrence rates at 100 days post-transplantation were 83.8%, 80.2%, 16.1%, and 4.5%, respectively. Conclusions: The combination of PAIA and RTX has a certain therapeutic effect and good safety in the desensitization treatment of highly sensitive patients before haplo-HSCT.

目的研究蛋白 A 免疫吸附(PAIA)联合利妥昔单抗(RTX)对接受单倍体造血干细胞移植(haplo-HSCT)的高度致敏患者的有效性和安全性。方法:回顾性分析2021年3月至2023年6月期间在苏州大学附属第一医院和苏州霍普斯血液病医院接受PAIA和RTX治疗的56例高度致敏患者的临床数据。测量人类白细胞抗原(HLA)抗体类型数量和平均荧光强度(MFI)、体液免疫、吸附过程中的不良反应以及吸附前后100天内的存活率。结果显示接受 PAIA 治疗后,仅含有 HLA Ⅰ 型抗体的患者的中位 MFI 从 7 859(3 209-12 444)降至 3 719(0-8 275)(PP=0.035)。抗供体特异性抗体阳性患者的中位 MFI 从 8 779(2 697-18 659)降至 4 524(0-15 989)(PPPPP分别=0.002)。44 名患者在移植后接受了 HLA 抗体监测,总体 MFI 和抗体类型数量均有所下降。然而,有五名患者出现了新的低MFI抗体,九名患者的MFI仍然很高。移植后100天的总生存率、无病生存率、非复发死亡率和累积复发率分别为83.8%、80.2%、16.1%和4.5%。结论PAIA和RTX联合应用于单倍体造血干细胞移植前高度敏感患者的脱敏治疗具有一定的疗效和良好的安全性。
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引用次数: 0
[Analysis of the efficacy and safety of bone disease treatment in patients with newly diagnosed multiple myeloma treated with denosumab or zoledronic acid]. [使用地诺单抗或唑来膦酸治疗新确诊多发性骨髓瘤患者骨病的疗效和安全性分析]。
Q3 Medicine Pub Date : 2024-04-14 DOI: 10.3760/cma.j.cn121090-20231203-00289
Y Ma, X B Xiao, Y S Liu, X L Chen, S Z Yuan, S H Zhao, Y Lu, H Yin, J L Chen, Y Q Wang, N N Cheng, P Feng, W R Huang

Objective: This study investigated the efficacy and safety of denosumab (DENOS) versus zoledronic acid (ZOL) in the bone disease treatment of newly diagnosed multiple myeloma. Methods: The clinical data of 80 patients with myeloma bone disease (MBD) at the Fifth Medical Center of PLA General Hospital between March 1, 2021 and June 30, 2023 were retrospectively reviewed. Eighteen patients with severe renal impairment (SRI, endogenous creatinine clearance rate<30 ml/min) were treated with DENOS, and 62 non-SRI patients were divided into DENOS (30 patients) and ZOL group (32 patients) . Results: Hypocalcemia was observed in 26 (33%) patients, and 22 patients developed hypocalcemia during the first treatment course. The incidence of hypocalcemia in the non-SRI patients of DENOS group was higher than that in the ZOL group [20% (6/30) vs 13% (4/32), P=0.028]. The incidence of hypocalcemia in SRI was 89% (16/18). Multivariate logistic regression analysis revealed that endogenous creatinine clearance rate<30 ml/min was significantly associated with hypocalcemia after DENOS administration (P<0.001). After 1 month of antiresorptive (AR) drug application, the decrease in the serum β-C-terminal cross-linked carboxy-telopeptide of collagen type I concentrations of SRI and non-SRI patients in the DENOS group were significantly higher than that in the ZOL group (68% vs 59% vs 27%, P<0.001). The increase in serum procollagen type Ⅰ N-terminal propeptide concentrations of patients with or without SRI in the DENOS group were significantly higher than that in the ZOL group (34% vs 20% vs 11%, P<0.05). The level of intact parathyroid hormone in each group increased after AR drug treatment. None of the patients developed osteonecrosis of the jaw and renal adverse events, and no statistically significant differences in the overall response rate, complete remission and stringent complete remission rates were found among the groups (P>0.05), and the median PFS and OS time were not reached (P>0.05) . Conclusions: In the treatment of MBD, DENOS minimizes nephrotoxicity and has strong AR effect. Hypocalcemia is a common adverse event but is usually mild or moderate and manageable.

研究目的本研究探讨了地诺单抗(DENOS)与唑来膦酸(ZOL)在新诊断多发性骨髓瘤骨病治疗中的有效性和安全性。研究方法回顾性分析解放军总医院第五医学中心2021年3月1日至2023年6月30日期间80例骨髓瘤骨病(MBD)患者的临床资料。结果显示,18 例患者存在严重肾功能损害(SRI,内生肌酐清除率):26例(33%)患者出现低钙血症,22例患者在首个疗程中出现低钙血症。DENOS组非SRI患者的低钙血症发生率高于ZOL组[20%(6/30) vs 13%(4/32),P=0.028]。SRI患者的低钙血症发生率为89%(16/18)。多变量逻辑回归分析显示,内源性肌酐清除率Pvs为59% vs 27%,Pvs为20% vs 11%,PP>0.05),中位PFS和OS时间均未达到(P>0.05)。结论在MBD的治疗中,DENOS可将肾毒性降至最低,并具有较强的AR效应。低钙血症是一种常见的不良反应,但通常为轻度或中度,且可控。
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