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[Bendamustine combined with anti-CD20 monoclonal antibody in the first-line treatment of older patients with indolent B-cell non-Hodgkin lymphoma: a multicenter retrospective study]. [苯达莫司汀联合抗cd20单克隆抗体一线治疗老年惰性b细胞非霍奇金淋巴瘤:一项多中心回顾性研究]。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20241227-00597
S C Qin, Y Miao, Z L Zhang, J Zhang, Y Y Shi, Y Q Miao, W Y Gu, W C Zheng, Z X Jia, G Q Lin, H W Ni, X H Xu, M Xu, X Y Xie, L Wang, Y Zhuang, W Zhang, P Liu, J Y Li, W Y Shi

Objective: To investigate the efficacy and safety of bendamustine combined with anti-CD20 monoclonal antibody in the first-line treatment of older patients with indolent B-cell non-Hodgkin lymphoma (B-iNHL) . Methods: The clinical data of 159 patients with B-iNHL enrolled in 16 hospitals from Jiangsu Cooperative Lymphoma Group from December 1, 2019, to April 20, 2024, were analyzed for regimen efficacy and safety. Bendamustine plus rituximab (BR) and bendamustine plus obinutuzumab (BG) were administered to 139 (87.4% ) and 20 (12.6% ) patients, respectively. Results: Among the 159 patients, 101 (63.5% ) were male and 58 (36.5% ) were female, with a median age of 69 years (range: 60-84). Efficacy could be assessed in 138 (86.8% ) patients. The efficacy assessment demonstrated that the overall response rate was 92.0% with complete and partial remissions in 75 (54.3% ) and 52 (37.7% ) cases, respectively. With a median follow-up of 24 months (range: 4-64), the progression-free survival rate was (87.5 ± 3.0) % and the overall survival rate was (83.2 ± 3.3) %. Of the 27 patients who died, 6 (22.2% ) died due to disease progression. The mean applied dose of bendamustine per cycle was 73.0 (50.8-89.7) mg/m(2) per day, administered on days 1 and 2. Adverse events of grade 3 or higher were reported in 53 (33.3% ) patients, with infection (30 cases,18.9% ) and neutropenia (24 cases, 15.1% ) demonstrating the highest incidence. Conclusion: Bendamustine combined with anti-CD20 monoclonal antibody demonstrated good efficacy and is well-tolerated in the first-line treatment of elderly patients with B-iNHL.

目的:探讨苯达莫司汀联合抗cd20单克隆抗体一线治疗老年惰性b细胞非霍奇金淋巴瘤(B-iNHL)的疗效和安全性。方法:分析江苏省合作淋巴瘤组16家医院2019年12月1日至2024年4月20日159例B-iNHL患者的临床资料,分析方案的有效性和安全性。苯达莫司汀+利妥昔单抗(BR)和苯达莫司汀+ obinutuzumab (BG)分别用于139例(87.4%)和20例(12.6%)患者。结果:159例患者中,男性101例(63.5%),女性58例(36.5%),中位年龄69岁(60 ~ 84岁)。138例(86.8%)患者可评价疗效。疗效评估显示,总有效率为92.0%,完全缓解75例(54.3%),部分缓解52例(37.7%)。中位随访24个月(4-64个月),无进展生存率为(87.5±3.0)%,总生存率为(83.2±3.3)%。在27例死亡患者中,6例(22.2%)死于疾病进展。苯达莫司汀每周期的平均给药剂量为73.0 (50.8-89.7)mg/m(2) /天,第1天和第2天给药。53例(33.3%)患者报告了3级及以上不良事件,其中感染(30例,18.9%)和中性粒细胞减少(24例,15.1%)的发生率最高。结论:苯达莫司汀联合抗cd20单克隆抗体一线治疗老年B-iNHL疗效良好,耐受性良好。
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引用次数: 0
[Recurrent fever, persistent cytopenia]. [反复发热,持续性细胞减少]。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20241129-00492
Y Miao, J Zhang, H Zhang, C Y Ding, Z Wang, Z L Su, J Y Li, W Y Shi

Intravascular large B-cell lymphoma (IVLBCL) is a rare large B-cell lymphoma subtype. We report a patient who presented with "recurrent fever and pancytopenia." A 64-year-old female patient had previously been diagnosed with Waldenstrom's macroglobulinemia and had received zanubrutinib treatment. In February 2023, the patient revisited due to "recurrent fever and pancytopenia." A positron emission tomography/computed tomography scan demonstrated significant enlargement of the bilateral adrenal glands. After an adrenal biopsy, she was diagnosed with diffuse large B-cell lymphoma, not otherwise specified. The patient received chemotherapy with the R-CHOP regimen (rituximab + cyclophosphamide + doxorubicin + vincristine + prednisone). After three treatment courses, a cranial magnetic resonance imaging examination indicated central nervous system infiltration of the lymphoma. After reviewing the pathology of the adrenal biopsy, the final diagnosis was revised as IVLBCL. Despite aggressive treatment, the disease continued to progress, and the patient died two months later. According to a multidisciplinary level, this article discusses the case from the perspective of a multidisciplinary team collaboration, involving imaging, pathology, dermatology, and lymphoma, to provide reference opinions for the clinical diagnosis and treatment of IVLBCL.

血管内大b细胞淋巴瘤(IVLBCL)是一种罕见的大b细胞淋巴瘤。我们报告一位以“反复发热和全血细胞减少症”为表现的病人。一名64岁的女性患者先前被诊断为Waldenstrom巨球蛋白血症,并接受了扎鲁替尼治疗。2023年2月,患者因“反复发热和全血细胞减少症”复诊。正电子发射断层扫描/计算机断层扫描显示双侧肾上腺明显增大。肾上腺活检后,她被诊断为弥漫性大b细胞淋巴瘤,没有其他说明。患者接受R-CHOP方案(利妥昔单抗+环磷酰胺+阿霉素+长春新碱+强的松)化疗。经过三个疗程的治疗后,颅磁共振成像检查显示中枢神经系统浸润淋巴瘤。在回顾肾上腺活检病理后,最终诊断修改为IVLBCL。尽管进行了积极的治疗,但病情仍在继续恶化,患者在两个月后死亡。本文从多学科层面,从影像学、病理学、皮肤病学、淋巴瘤等多学科团队合作的角度对该病例进行探讨,为IVLBCL的临床诊断和治疗提供参考意见。
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引用次数: 0
[Allogeneic hematopoietic stem cell transplantation for the treatment of BCR::ABL-negative neutrophilic myeloid neoplasms: a clinical analysis of 12 cases]. 异基因造血干细胞移植治疗BCR: abl阴性中性粒细胞肿瘤12例临床分析
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20241211-00560
T T Han, Y He, J Liu, Y Chen, F R Wang, J Z Wang, Y Y Chen, H X Fu, L P Xu, X H Zhang, X J Huang, Y Wang

Objective: To investigate the efficacy and safety of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for the treatment of BCR::ABL-negative chronic neutrophilic leukemia (CNL) and MDS/MPN with neutrophilia. Methods: This study retrospectively analyzed 12 cases of CNL and MDS/MPN with neutrophilia that underwent allo-HSCT from March 2017 to June 2024, comprising 7 males and 5 females with a median age of 48 (IQR: 28, 59) years. The 2-year overall survival (OS), disease-free survival (DFS), cumulative incidence of relapse (CIR), and transplantation-related mortality (TRM) rates were analyzed. Complications were also assessed. Results: Of the 12 patients, 6 received matched sibling HSCT and 6 received haploidentical HSCT. All patients had successful engraftment, and the median times of neutrophil and platelet engraftment were 17 (IQR: 11, 24) days and 15 (IQR: 9, 28) days, respectively. Grade Ⅱ-Ⅳ acute graft versus host disease (GVHD) and chronic GVHD occurred in 2 and 4 cases, respectively. The 2-year OS, DFS, CIR, and TRM rates were (65.6 ± 16.4) %, (41.7 ± 16.6) %, (47.2 ±18.2) %, and (11.1 ± 11.4) %, respectively, after a median follow-up time of 637 (IQR: 330, 943) days. One patient died from treatment-related complications due to respiratory failure caused by coronavirus disease 2019. Two patients died due to relapse. Conclusion: Allo-HSCT can be applied as a safe and effective approach to treat CNL and MDS/MPN with neutrophilia.

目的:探讨同种异体造血干细胞移植治疗BCR:: abl阴性慢性中性粒细胞白血病(CNL)和MDS/MPN合并中性粒细胞的疗效和安全性。方法:本研究回顾性分析2017年3月至2024年6月12例CNL和MDS/MPN伴中性粒细胞增生患者行异体造血干细胞移植的病例,其中男性7例,女性5例,中位年龄48岁(IQR: 28,59)。分析2年总生存期(OS)、无病生存期(DFS)、累积复发率(CIR)和移植相关死亡率(TRM)。并发症也进行了评估。结果:在12例患者中,6例接受了匹配的兄弟姐妹HSCT, 6例接受了单倍相同的HSCT。所有患者均移植成功,中性粒细胞和血小板移植的中位时间分别为17 (IQR: 11、24)天和15 (IQR: 9、28)天。Ⅱ-Ⅳ级急性移植物抗宿主病(GVHD)和慢性移植物抗宿主病分别发生2例和4例。中位随访时间为637 (IQR: 330, 943)天,2年OS、DFS、CIR和TRM分别为(65.6±16.4)%、(41.7±16.6)%、(47.2±18.2)%和(11.1±11.4)%。一名患者因2019冠状病毒病引起的呼吸衰竭而死于与治疗相关的并发症。2例因复发死亡。结论:同种异体造血干细胞移植是治疗CNL和MDS/MPN合并中性粒细胞增多的一种安全有效的方法。
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引用次数: 0
[High-throughput sequencing in identifying somatic hypermutation in immunoglobulin heavy chain variable regions with complex clonal backgrounds]. [高通量测序鉴定具有复杂克隆背景的免疫球蛋白重链可变区体细胞超突变]。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20241016-00394
M G Gao, R Wei, Y Liu, X J Huang, S M Yang, X S Zhao

Objective: To compare the performance of next-generation sequencing (NGS) and Sanger sequencing in investigating somatic hypermutation (SHM) status of immunoglobulin heavy chain variable region (IGHV) genes. It specifically focuses on identifying key factors contributing to discrepancies between the two methods, particularly under complex clonal backgrounds, to inform optimized strategies for clinical application. Methods: This retrospective analysis included 53 samples, comprising 43 identified as non-monoclonal and 10 as monoclonal using Sanger sequencing. All samples were further analyzed using NGS to assess IGHV SHM. The two methods were used for systematic comparison. For discordant cases, in-depth attribution analysis was conducted, considering factors, including clonal abundance quantification, differences in primer design, and interpretation criteria. Results: Among the 53 patients who underwent both Sanger and NGS testing, 36 were male and 17 were female, with a median age of 64 years (range: 33-88). Diagnoses included chronic lymphocytic leukemia (CLL) in 35 (66.0% ), diffuse large B-cell lymphoma in 9 (17.0% ), follicular lymphoma in 3 (5.7% ), mantle cell lymphoma in 3 (5.7% ), and other types in 3 (5.7% ) cases. In the 43 cases with non-monoclonal profiles using Sanger sequencing, NGS revealed 23 cases as biclonal or polyclonal, 17 as monoclonal, and 3 with no detectable clonality. The primary discrepancies between the two methods involved variations in clonality assessment, IGHV gene rearrangement types, and mutation rates. Among the 10 cases identified as monoclonal using Sanger sequencing, NGS detected biclonality and markedly different IGHV rearrangement types in 2 and 4 cases, respectively. Minor differences were observed in SHM percentage between the two methods; however, these did not substantially affect the overall determination of mutational status. Conclusion: Compared with Sanger sequencing, NGS exhibits superior performance in assessing IGHV SHM status under complex clonal conditions. It provides greater sensitivity and accuracy in detecting subclonal components and quantifying clonal proportions, thereby providing a more precise molecular basis for diagnosing and prognostically assessing lymphoid malignancies, including CLL.

目的:比较新一代测序(NGS)和Sanger测序在研究免疫球蛋白重链可变区(IGHV)基因体细胞超突变(SHM)状态中的作用。它特别侧重于识别导致两种方法之间差异的关键因素,特别是在复杂的克隆背景下,为临床应用的优化策略提供信息。方法:回顾性分析53份样本,其中43份经Sanger测序鉴定为非单克隆,10份为单克隆。使用NGS进一步分析所有样品以评估IGHV SHM。采用两种方法进行系统比较。对于不一致的案例,考虑克隆丰度定量、引物设计差异和解释标准等因素,进行深入的归因分析。结果:在同时接受Sanger和NGS检测的53例患者中,男性36例,女性17例,中位年龄64岁(范围:33-88岁)。慢性淋巴细胞白血病35例(66.0%),弥漫性大b细胞淋巴瘤9例(17.0%),滤泡性淋巴瘤3例(5.7%),套细胞淋巴瘤3例(5.7%),其他类型3例(5.7%)。在Sanger测序的43例非单克隆病例中,NGS显示23例为双克隆或多克隆,17例为单克隆,3例未检测到克隆。两种方法之间的主要差异包括克隆性评估、IGHV基因重排类型和突变率的差异。在Sanger测序鉴定为单克隆的10例病例中,NGS分别在2例和4例中检测到双克隆性和显著不同的IGHV重排类型。两种方法的SHM百分比差异较小;然而,这些并没有实质性地影响突变状态的总体确定。结论:与Sanger测序相比,NGS在复杂克隆条件下评估IGHV SHM状态方面表现出更好的性能。它在检测亚克隆成分和定量克隆比例方面提供了更高的灵敏度和准确性,从而为包括CLL在内的淋巴细胞恶性肿瘤的诊断和预后评估提供了更精确的分子基础。
{"title":"[High-throughput sequencing in identifying somatic hypermutation in immunoglobulin heavy chain variable regions with complex clonal backgrounds].","authors":"M G Gao, R Wei, Y Liu, X J Huang, S M Yang, X S Zhao","doi":"10.3760/cma.j.cn121090-20241016-00394","DOIUrl":"10.3760/cma.j.cn121090-20241016-00394","url":null,"abstract":"<p><p><b>Objective:</b> To compare the performance of next-generation sequencing (NGS) and Sanger sequencing in investigating somatic hypermutation (SHM) status of immunoglobulin heavy chain variable region (IGHV) genes. It specifically focuses on identifying key factors contributing to discrepancies between the two methods, particularly under complex clonal backgrounds, to inform optimized strategies for clinical application. <b>Methods:</b> This retrospective analysis included 53 samples, comprising 43 identified as non-monoclonal and 10 as monoclonal using Sanger sequencing. All samples were further analyzed using NGS to assess IGHV SHM. The two methods were used for systematic comparison. For discordant cases, in-depth attribution analysis was conducted, considering factors, including clonal abundance quantification, differences in primer design, and interpretation criteria. <b>Results:</b> Among the 53 patients who underwent both Sanger and NGS testing, 36 were male and 17 were female, with a median age of 64 years (range: 33-88). Diagnoses included chronic lymphocytic leukemia (CLL) in 35 (66.0% ), diffuse large B-cell lymphoma in 9 (17.0% ), follicular lymphoma in 3 (5.7% ), mantle cell lymphoma in 3 (5.7% ), and other types in 3 (5.7% ) cases. In the 43 cases with non-monoclonal profiles using Sanger sequencing, NGS revealed 23 cases as biclonal or polyclonal, 17 as monoclonal, and 3 with no detectable clonality. The primary discrepancies between the two methods involved variations in clonality assessment, IGHV gene rearrangement types, and mutation rates. Among the 10 cases identified as monoclonal using Sanger sequencing, NGS detected biclonality and markedly different IGHV rearrangement types in 2 and 4 cases, respectively. Minor differences were observed in SHM percentage between the two methods; however, these did not substantially affect the overall determination of mutational status. <b>Conclusion:</b> Compared with Sanger sequencing, NGS exhibits superior performance in assessing IGHV SHM status under complex clonal conditions. It provides greater sensitivity and accuracy in detecting subclonal components and quantifying clonal proportions, thereby providing a more precise molecular basis for diagnosing and prognostically assessing lymphoid malignancies, including CLL.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 9","pages":"815-819"},"PeriodicalIF":0.0,"publicationDate":"2025-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12580819/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145379003","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
[Frontier advances in chimeric antigen receptor T cells and chimeric antigen receptor regulatory T cells therapies for autoimmune diseases]. 嵌合抗原受体T细胞和嵌合抗原受体调节性T细胞治疗自身免疫性疾病的前沿进展。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20250524-00242
S A Pan, J Shi

Autoimmune diseases (AID) are disorders in which the immune system mistakenly attacks the body's own tissues. However, current immunosuppressive therapies seldom achieve durable, drug-free remission, indicating the urgent need for therapeutic strategies that are both more precise and longer-lasting. Chimeric antigen receptor (CAR) -T cells are generated by genetically engineering T cells to specifically recognize and kill cells that express particular antigens, thereby providing a novel therapeutic approach for AID. This review summarizes the immunological mechanisms of CAR-T cells and -regulatory T cells (Treg) in treating AID, and systematically reviews the latest advances in applying these treatments to rheumatic AID, immune-mediated neurological AID, and refractory autoimmune hemolytic anemia, among others. Further, we discuss the safety-related limitations of CAR-T /CAR-Treg treatment for AID, and outline other CAR-based cellular therapies that can be used to treat AID beyond CAR-T and CAR-Treg cells.

自身免疫性疾病(AID)是免疫系统错误地攻击人体自身组织的疾病。然而,目前的免疫抑制疗法很少能实现持久的无药物缓解,这表明迫切需要更精确和更持久的治疗策略。嵌合抗原受体(CAR) -T细胞是由基因工程T细胞产生的,可以特异性识别和杀死表达特定抗原的细胞,从而为艾滋病提供了一种新的治疗方法。本文综述了CAR-T细胞和-调节性T细胞(Treg)治疗AID的免疫学机制,并系统综述了这些治疗方法在风湿性AID、免疫介导的神经性AID和难治性自身免疫性溶血性贫血等方面的最新进展。此外,我们讨论了CAR-T /CAR-Treg治疗艾滋病的安全性相关限制,并概述了除CAR-T和CAR-Treg细胞外,可用于治疗艾滋病的其他基于car的细胞疗法。
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引用次数: 0
[Acquired factor ⅩⅢ deficiency with FⅩⅢ inhibitor secondary to rheumatoid arthritis: a case report]. [继发于类风湿关节炎的获得性因子ⅩⅢ缺乏伴FⅩⅢ抑制剂1例报告]。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20250610-00269
B C Zhu, H C Cai, Y Zhang, L Wang, W Wang
{"title":"[Acquired factor ⅩⅢ deficiency with FⅩⅢ inhibitor secondary to rheumatoid arthritis: a case report].","authors":"B C Zhu, H C Cai, Y Zhang, L Wang, W Wang","doi":"10.3760/cma.j.cn121090-20250610-00269","DOIUrl":"10.3760/cma.j.cn121090-20250610-00269","url":null,"abstract":"","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 9","pages":"874"},"PeriodicalIF":0.0,"publicationDate":"2025-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12580798/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145379000","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
[Renal response and prognosis of newly diagnosed patients with multiple myeloma with renal impairment applying VRD and autologous hematopoietic stem cell transplantation]. [新诊断多发性骨髓瘤合并肾损害患者应用VRD和自体造血干细胞移植的肾反应及预后]。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20241211-00564
X Y Wu, Y Huang, H M Shen, H Y You, Z Yan, Y Xie, W Q Yao, S Yan, J Wang, Y Y Zhai, X L Shi, J J Shang, S Jin, L Z Yan, D P Wu, C C Fu

Objective: To investigate the feasibility of the bortezomib, lenalidomide, and dexamethasone (VRD) regimen combined with autologous hematopoietic stem cell transplantation (auto-HSCT) in patients with multiple myeloma (MM) and renal impairment, analyze treatment efficacy and renal responses stratified based on renal dysfunction severity, and explore the prognostic significance of early renal response and its affecting factors. Methods: This retrospective study, conducted at the First Affiliated Hospital of Soochow University, categorized 316 patients with newly diagnosed MM (NDMM) from August 2018 to October 2022 based on renal function for analysis of clinical characteristics, treatment response, and prognosis. Continuous variables were compared using t-tests or Mann-Whitney U tests, categorical variables utilizing Chi-square tests, survival outcomes employing Kaplan-Meier and Log-rank tests, and renal response predictors with logistic regression. Results: Patients were stratified based on baseline estimated glomerular filtration rate (eGFR) : normal [≥90 ml·min(-1)· (1.73 m(2)) (-1), n=160], mild [≥60 ml·min(-1)· (1.73 m(2)) (-1) to <90 ml·min(-1)· (1.73 m(2)) (-1), n=55], moderate [≥30 ml·min(-1)· (1.73 m(2)) (-1) to <60 ml·min(-1)· (1.73 m(2)) (-1), n=39], and severe impairment [<30 ml·min(-1)· (1.73 m(2)) (-1), n=62]. Moderate and severe renal impairment correlated with advanced International Staging System/Revised International Staging System classification, lower hemoglobin levels, frailty, and higher light-chain/IgD subtype prevalence (P<0.05). Despite younger age (P=0.001) and higher transplant rates (P=0.041) in severe cases, overall response rates (ORR: 93.7% ; ≥VGPR: 82.9% ) were comparable across groups (P>0.05). Among 24 dialysis-dependent patients at diagnosis, 11 (45.8% ) achieved dialysis independence after induction [median: 3.0 (0.5-4.0) months], including 10 undergoing auto-HSCT. In 89 evaluable patients [baseline eGFR <50 ml·min(-1)· (1.73 m(2)) (-1)], renal ORR (RORR) was 70.8% [rapid complete response: 31.5% ; rapid partial response: 11.2% ; rapid minimal response (RMR) : 28.1% ]. Renal response predicted better survival (overall survival: HR=0.36, 95% CI: 0.13-0.99, P=0.049). Moderate-to-severe renal impairment was associated with increased transplant-related adverse events and delayed engraftment (P<0.05) ; however, auto-HSCT significantly improved outcomes after 33.5-month median follow-up (range: 2-65 months). Multivariate analysis identified 1q21+ (OR=3.58, 95% CI: 1.17-11.02, P=0.026) and light-chain subtype (OR=2.86, 95% CI: 1.08-7.69, P=0.036) as independent predictors of poor renal response. Conclusion: VRD regimen plus auto-HSCT demonstrates robust efficacy in NDMM, including patients with renal impairment, with a 70.8% RORR and manageable toxicity.

目的:探讨硼替佐米、来那度胺、地塞米松(VRD)联合自体造血干细胞移植(auto-HSCT)治疗多发性骨髓瘤(MM)合并肾功能损害患者的可行性,分析根据肾功能不全程度分层的治疗效果和肾反应,探讨早期肾反应的预后意义及其影响因素。方法:回顾性研究于2018年8月至2022年10月在苏州大学第一附属医院对316例新诊断MM (NDMM)患者进行肾功能分类,分析其临床特征、治疗反应及预后。使用t检验或Mann-Whitney U检验对连续变量进行比较,使用卡方检验对分类变量进行比较,使用Kaplan-Meier检验和Log-rank检验对生存结果进行比较,使用逻辑回归对肾脏反应预测因子进行比较。结果:根据基线估计肾小球滤过率(eGFR)对患者进行分层:正常[≥90 ml·min(-1)·(1.73 m(2)) (-1), n=160],轻度[≥60 ml·min(-1)·(1.73 m(2))(-1)至n=55],中度[≥30 ml·min(-1)·(1.73 m(2))(-1)至n=39],重度[n=62]。中重度肾功能损害与高级国际分期系统/修订国际分期系统分类、较低的血红蛋白水平、虚弱、较高的轻链/IgD亚型患病率(PP=0.001)和严重病例较高的移植率(P=0.041)相关,总有效率(ORR: 93.7%;≥VGPR: 82.9%)组间具有可比性(P < 0.05)。在24例确诊时依赖透析的患者中,11例(45.8%)在诱导后实现了透析独立[中位数:3.0(0.5-4.0)个月],其中10例接受了自体造血干细胞移植。89例可评估患者[基线eGFR HR=0.36, 95% CI: 0.13-0.99, P=0.049]。中度至重度肾损害与移植相关不良事件和移植延迟增加(OR= 3.58, 95% CI: 1.17-11.02, P=0.026)和轻链亚型(OR=2.86, 95% CI: 1.08-7.69, P=0.036)相关,是肾不良反应的独立预测因子。结论:VRD方案加自体造血干细胞移植对NDMM(包括肾功能损害患者)显示出强大的疗效,RORR为70.8%,毒性可控。RMR≥与预后良好相关,而1q21+和轻链亚型独立预测肾反应较差。
{"title":"[Renal response and prognosis of newly diagnosed patients with multiple myeloma with renal impairment applying VRD and autologous hematopoietic stem cell transplantation].","authors":"X Y Wu, Y Huang, H M Shen, H Y You, Z Yan, Y Xie, W Q Yao, S Yan, J Wang, Y Y Zhai, X L Shi, J J Shang, S Jin, L Z Yan, D P Wu, C C Fu","doi":"10.3760/cma.j.cn121090-20241211-00564","DOIUrl":"10.3760/cma.j.cn121090-20241211-00564","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the feasibility of the bortezomib, lenalidomide, and dexamethasone (VRD) regimen combined with autologous hematopoietic stem cell transplantation (auto-HSCT) in patients with multiple myeloma (MM) and renal impairment, analyze treatment efficacy and renal responses stratified based on renal dysfunction severity, and explore the prognostic significance of early renal response and its affecting factors. <b>Methods:</b> This retrospective study, conducted at the First Affiliated Hospital of Soochow University, categorized 316 patients with newly diagnosed MM (NDMM) from August 2018 to October 2022 based on renal function for analysis of clinical characteristics, treatment response, and prognosis. Continuous variables were compared using t-tests or Mann-Whitney U tests, categorical variables utilizing Chi-square tests, survival outcomes employing Kaplan-Meier and Log-rank tests, and renal response predictors with logistic regression. <b>Results:</b> Patients were stratified based on baseline estimated glomerular filtration rate (eGFR) : normal [≥90 ml·min(-1)· (1.73 m(2)) (-1), <i>n</i>=160], mild [≥60 ml·min(-1)· (1.73 m(2)) (-1) to <90 ml·min(-1)· (1.73 m(2)) (-1), <i>n</i>=55], moderate [≥30 ml·min(-1)· (1.73 m(2)) (-1) to <60 ml·min(-1)· (1.73 m(2)) (-1), <i>n</i>=39], and severe impairment [<30 ml·min(-1)· (1.73 m(2)) (-1), <i>n</i>=62]. Moderate and severe renal impairment correlated with advanced International Staging System/Revised International Staging System classification, lower hemoglobin levels, frailty, and higher light-chain/IgD subtype prevalence (<i>P</i><0.05). Despite younger age (<i>P</i>=0.001) and higher transplant rates (<i>P</i>=0.041) in severe cases, overall response rates (<i>ORR</i>: 93.7% ; ≥VGPR: 82.9% ) were comparable across groups (<i>P</i>>0.05). Among 24 dialysis-dependent patients at diagnosis, 11 (45.8% ) achieved dialysis independence after induction [median: 3.0 (0.5-4.0) months], including 10 undergoing auto-HSCT. In 89 evaluable patients [baseline eGFR <50 ml·min(-1)· (1.73 m(2)) (-1)], renal ORR (RORR) was 70.8% [rapid complete response: 31.5% ; rapid partial response: 11.2% ; rapid minimal response (RMR) : 28.1% ]. Renal response predicted better survival (overall survival: <i>HR</i>=0.36, 95% <i>CI</i>: 0.13-0.99, <i>P</i>=0.049). Moderate-to-severe renal impairment was associated with increased transplant-related adverse events and delayed engraftment (<i>P</i><0.05) ; however, auto-HSCT significantly improved outcomes after 33.5-month median follow-up (range: 2-65 months). Multivariate analysis identified 1q21+ (<i>OR</i>=3.58, 95% <i>CI</i>: 1.17-11.02, <i>P</i>=0.026) and light-chain subtype (<i>OR</i>=2.86, 95% <i>CI</i>: 1.08-7.69, <i>P</i>=0.036) as independent predictors of poor renal response. <b>Conclusion:</b> VRD regimen plus auto-HSCT demonstrates robust efficacy in NDMM, including patients with renal impairment, with a 70.8% RORR and manageable toxicity. ","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 9","pages":"839-847"},"PeriodicalIF":0.0,"publicationDate":"2025-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12580808/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145378707","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
[Significance of the 50% hemolytic complement in hemolysis assessment and efficacy of eculizumab in patients with paroxysmal nocturnal hemoglobinuria]. 【50%溶血补体在突发性夜间血红蛋白尿患者溶血评估及eculizumab疗效中的意义】。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20250118-00034
L Li, X Y Huang, X Q Ding, Z W Liu, C Yang, M Chen, J Yin, B Han

This study retrospectively analyzed data from 25 patients with paroxysmal nocturnal hemoglobinuria (PNH) admitted to Peking Union Medical College Hospital and Dongfang Hospital of Beijing University of Chinese Medicine from January 2023 to June 2024. Patients receiving sufficient eculizumab treatment for at least 3 months and who completed hemolytic complex (CH50) level testing pre- and post-treatment for 3 and 6 months were selected. Blood routine, biochemistry, and the 50% CH50-related indicators were monitored pre- and post-treatment. Among these patients, 24 completed 6 months of treatment and CH50 testing. After 3 and 6 months of eculizumab treatment, all patients with PNH showed significant improvement in symptoms, with lactate dehydrogenase (LDH) levels decreasing from a baseline of (1 814.4 ± 924.8) U/L to (248.5 ± 61.0) U/L and (239.3 ± 44.8) U/L. Hemoglobin levels increased from a baseline of (73.9±14.4) g/L to (99.9 ± 21.3) g/L and (99.6 ± 19.8) g/L. The baseline CH50 level was (32.4±14.7) %, which decreased to 2.0% (1.0% -8.0% ) and 1.0% (1.0% -4.0% ) at 3 and 6 months posttreatment, respectively. At baseline, a linear correlation was found between CH50 and LDH levels (P<0.001), and the trend of CH50 changes was significantly lower than LDH at 3 and 6 months post-treatment with eculizumab, with similar trends. However, no linear correlation was observed between CH50 and LDH levels or other parameters at 3 and 6 months of medication. Our case demonstrates that eculizumab is effective for PNH hemolysis treatment. The serum CH50 level may be a biomarker for complement blockade induced by eculizumab, which can, to some extent, reflect the intravascular hemolysis of PNH and the efficacy of eculizumab.

本研究回顾性分析了2023年1月至2024年6月在北京协和医院和北京中医药大学东方医院收治的25例阵发性夜间血红蛋白尿(PNH)患者的资料。选择接受足量eculizumab治疗至少3个月,并在治疗前和治疗后3个月和6个月完成溶血复合物(CH50)水平检测的患者。治疗前后监测血常规、生化及50% ch50相关指标。在这些患者中,24例完成了6个月的治疗和CH50检测。在eculizumab治疗3个月和6个月后,所有PNH患者的症状均有显著改善,乳酸脱氢酶(LDH)水平从基线(1 814.4±924.8)U/L降至(248.5±61.0)U/L和(239.3±44.8)U/L。血红蛋白水平从基线(73.9±14.4)g/L上升到(99.9±21.3)g/L和(99.6±19.8)g/L。基线CH50水平为(32.4±14.7)%,治疗后3个月和6个月分别降至2.0%(1.0% -8.0%)和1.0%(1.0% -4.0%)。基线时,CH50和LDH水平呈线性相关(P
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引用次数: 0
[Chinese expert consensus on the diagnosis and management of Epstein-Barr virus positive diffuse large B cell lymphoma (2025)]. 【爱泼斯坦-巴尔病毒阳性弥漫性大B细胞淋巴瘤诊断与治疗中国专家共识(2025)】。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20250729-00354-1

Epstein-Barr virus positive diffuse large B cell lymphoma (EBV(+) DLBCL) is a rare subtype of diffuse large B cell lymphoma with a poor prognosis. Due to the low incidence of EBV(+) DLBCL, the understanding of the disease has not been unified, and the treatment remains non-standardized. In order to strengthen the understanding of EBV(+) DLBCL in our country, improve the diagnosis and treatment level, and help promote multi-center clinical research, the Lymphoid Disease Group, Chinese Society of Hematology, Chinese Medical Association and Lymphoma Expert Committee of Chinese Society of Clinical Oncology (CSCO) organized relevant experts to discuss and form this consensus on the diagnosis and treatment of EBV(+) DLBCL, combined with the latest research progress in China and abroad.

eb病毒阳性弥漫大B细胞淋巴瘤(EBV(+) DLBCL)是一种罕见的弥漫大B细胞淋巴瘤亚型,预后较差。由于EBV(+) DLBCL发病率较低,对该病的认识尚未统一,治疗仍不规范。为加强我国对EBV(+) DLBCL的认识,提高诊治水平,促进多中心临床研究,淋巴病学组、中国血液学学会、中华医学会、中国临床肿瘤学会淋巴瘤专家委员会组织相关专家共同探讨,形成EBV(+) DLBCL的诊治共识。结合国内外最新研究进展。
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引用次数: 0
[Prognostic value of the FS-15 frailty score in patients with myelodysplastic syndromes]. [FS-15衰弱评分在骨髓增生异常综合征患者中的预后价值]。
Q3 Medicine Pub Date : 2025-09-14 DOI: 10.3760/cma.j.cn121090-20250303-00107
X Wang, T J Qin, Z F Xu, S Q Qu, B Li, L J Pan, Q Y Gao, M Jiao, Y Zhong, B H Jiang, L L Liu, J Y Zhao, W J Xie, Z J Xiao

Objective: To identify the prognostic value of the Revised 15-item Myelodysplastic Syndrome-specific frailty scale (FS-15) in Chinese patients with myelodysplastic syndromes (MDS) . Methods: This retrospective study analyzed 812 patients with newly diagnosed MDS admitted to the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, and Peking Union Medical College from August 2016 to June 2023. Patients were assessed using the FS-15 and subsequently categorized into frail and non-frail groups. Clinical and laboratory characteristics, as well as overall survival (OS), were compared between these groups. Results: ① The median patient age was 55 years (IQR 45-64), with a median follow-up of 22.5 months (95% CI: 20.2-24.9) and a median OS of 43.3 months (95% CI: 36.8-49.8). The median FS-15 score was 0.42, with a cutoff value of 0.44. Male patients demonstrated higher median FS-15 scores than female patients (0.42 vs 0.38, P=0.006). In both the Revised International Prognostic Scoring System (IPSS-R; P=0.001) and Molecular International Prognostic Scoring System (IPSS-M; P=0.014) stratifications, FS-15 scores were significantly higher in the very high-risk group compared with the very low-risk group. ② The median OS was 54.7 months (95% CI: 47.5-NA) and 31.5 months (95% CI: 22.9-41.0) in the nonfrail (n=452) and frail groups (n=360), respectively (P<0.001). The 3-year OS rates were (63.2 ± 3.2) % and (46.4 ± 3.6) % for the non-frail and frail groups, with 5-year OS rates of (49.9 ± 4.7) % and (32.0 ± 4.3) %, respectively (P<0.001). ③Subgroup analysis revealed that nonfrail patients demonstrated significantly higher 3-year OS rates than frail patients in both the IPSS-M low-risk and very high-risk groups (all P<0.05). Similarly, nonfrail patients demonstrated superior 3-year OS rates compared with frail patients in the IPSS-R very low-risk, low-risk, and high-risk groups (all P<0.05). ④Among patients receiving hypomethylating agent therapy, the overall response rate was significantly higher in the non-frail group than in the frail group (86.7% vs 64.6%, P=0.007). Moreover, the frail group experienced higher rates of treatment-related adverse events, including febrile neutropenia (67.1% vs 47.4%, P=0.016) and liver function abnormalities (30.0% vs 14.5%, P=0.023), compared with the non-frail group. Conclusion: The FS-15 frailty score is a feasible and effective tool for assessing frailty in patients newly diagnosed with MDS in China and serves as a valuable prognostic indicator.

目的:探讨修订的15项骨髓增生异常综合征特异性衰弱量表(FS-15)在中国骨髓增生异常综合征(MDS)患者中的预后价值。方法:回顾性分析2016年8月至2023年6月在中国医学科学院血液学血液病研究所和北京协和医学院住院的812例新诊断MDS患者。使用FS-15对患者进行评估,随后将其分为虚弱组和非虚弱组。比较两组患者的临床和实验室特征以及总生存期(OS)。结果:①患者中位年龄55岁(IQR 45-64),中位随访22.5个月(95% CI: 20.2-24.9),中位OS为43.3个月(95% CI: 36.8-49.8)。FS-15评分中位数为0.42,截止值为0.44。男性患者的FS-15中位评分高于女性患者(0.42 vs 0.38, P=0.006)。在修订的国际预后评分系统(IPSS-R, P=0.001)和分子国际预后评分系统(IPSS-M, P=0.014)分层中,高危组的FS-15评分明显高于极低危组。②非虚弱组(n=452)和虚弱组(n=360)的中位OS分别为54.7个月(95% CI: 47.5-NA)和31.5个月(95% CI: 22.9-41.0) (PPPPvs 64.6%, P=0.007)。此外,与非虚弱组相比,虚弱组经历了更高的治疗相关不良事件发生率,包括发热性中性粒细胞减少症(67.1% vs 47.4%, P=0.016)和肝功能异常(30.0% vs 14.5%, P=0.023)。结论:FS-15衰弱评分是评估中国新诊断MDS患者衰弱程度的一种可行、有效的工具,可作为有价值的预后指标。
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Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
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