Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi最新文献_第8页

[Epstein-Barr virus-positive nodal T/NK-cell lymphoma presenting with hemophagocytic lymphohistiocytosis: a case report and literature review]. [eb病毒阳性淋巴结T/ nk细胞淋巴瘤伴噬血细胞性淋巴组织细胞增多症1例报告及文献复习]。

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20250922-00431

J Y Qiu, Y P Liu, H L Mi, Y L Yang, F Liang, H Z Fang, X T Jia, J Y Li, Y Miao

引用次数: 0

[Molecular and clinical characteristics and prognosis of myelodysplastic neoplasms with biallelic TET2 inactivation]. [双等位基因TET2失活骨髓增生异常肿瘤的分子、临床特征及预后]。

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20260115-00025

X Wang, L L Liu, B Li, Y J Jia, C W Li, T J Qin, Z F Xu, S Q Qu, L J Pan, Q Y Gao, M Jiao, Z J Xiao

{"title":"[Molecular and clinical characteristics and prognosis of myelodysplastic neoplasms with biallelic TET2 inactivation].","authors":"X Wang, L L Liu, B Li, Y J Jia, C W Li, T J Qin, Z F Xu, S Q Qu, L J Pan, Q Y Gao, M Jiao, Z J Xiao","doi":"10.3760/cma.j.cn121090-20260115-00025","DOIUrl":"10.3760/cma.j.cn121090-20260115-00025","url":null,"abstract":"Objective: To explore the clinical characteristics and prognostic significance of myelodysplastic neoplasms (MDS) with biallelic TET2 inactivation (bi-TET2) . Methods: Clinical data from 1 730 newly diagnosed de novo MDS patients treated at Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College between August 2016 and June 2025, with next-generation sequencing data, were retrospectively collected. The clinical characteristics and prognosis of patients with biallelic TET2 inactivation were analyzed. Results: Thirty-nine patients (2.3% ) harbored bi-TET2, including two with copy-neutral loss of heterozygosity on chromosome 4q24. Compared with patients with monoallelic TET2 mutations (n=147) and those without TET2 mutations (n=1 544), patients with bi-TET2 carried more gene mutations overall [4 (IQR: 3, 6) vs 4 (IQR: 2, 5) vs 2 (IQR: 1, 4), P<0.001] and had higher frequencies of mutations in ASXL1 (38.5% vs 29.9% vs 20.5%, P=0.001), SRSF2 (28.2% vs 12.9% vs 4.5%, P<0.001), SF3B1 (25.6% vs 18.4% vs 11.7%, P=0.004), EZH2 (18.0% vs 7.5% vs 4.5%, P=0.002), CUX1 (12.8% vs 3.4% vs 1.4%, P<0.001), KRAS (10.3% vs 4.3% vs 2.1%, P=0.006), CBL (10.3% vs 3.4% vs 2.6%, P=0.030), ZRSR2 (10.3% vs 6.1% vs 3.2%, P=0.017), and CEBPA (7.7% vs 1.4% vs 1.2%, P=0.019). Patients with bi-TET2 were older at diagnosis [65 (IQR: 58, 69) vs 61 (IQR: 50, 67) vs 55 (IQR: 44, 64) years, P<0.001] and had a higher proportion of normal karyotype (69.2% vs 46.3% vs 44.8%, P=0.024). Patients with bi-TET2 had a significantly shorter median overall survival (OS) time than patients with monoallelic TET2 mutations and those without TET2 mutations [18.5 (95% CI: 13.1-25.0) vs 40.6 (95% CI: 22.8-60.9) vs 54.7 (95% CI: 47.1-69.4) months, P=0.009]. Stratified analysis by IPSS-M prognostic risk categories showed that, in the high-risk group (moderate high-risk, high-risk, and very high-risk), patients with bi-TET2 had a significantly shorter median OS time[12.4 (95% CI: 8.4-NA) months] than patients with monoallelic TET2 mutations [19.7 (95% CI: 14.9-39.9) months] and those without TET2 mutations [32.0 (95% CI: 27.6-37.4) months, P<0.001]. In contrast, no significant difference in OS was observed in the low-risk group (very low-risk, low-risk, and moderate low-risk) . Conclusion: Patients with MDS harboring biallelic TET2 inactivation exhibit distinct clinical and molecular characteristics and in patients with relatively high IPSS-M risk, their prognosis is worse than that of patients with monoallelic TET2 mutations.","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 4","pages":"327-335"},"PeriodicalIF":0.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13195553/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147983375","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 of half-dose venetoclax combined with reduced-dose HA regimen in the induction therapy of acute myeloid leukemia]. [半剂量venetoclax联合低剂量HA方案诱导治疗急性髓系白血病的疗效分析]。

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20250516-00232

Z S Yan, X H Suo, G C Bai, B Zhang, J S He, J S Li, S N Wei, Q L Li, K Q Liu, Y C Mi

{"title":"[Analysis of the efficacy of half-dose venetoclax combined with reduced-dose HA regimen in the induction therapy of acute myeloid leukemia].","authors":"Z S Yan, X H Suo, G C Bai, B Zhang, J S He, J S Li, S N Wei, Q L Li, K Q Liu, Y C Mi","doi":"10.3760/cma.j.cn121090-20250516-00232","DOIUrl":"10.3760/cma.j.cn121090-20250516-00232","url":null,"abstract":"We retrospectively analyzed clinical data on newly diagnosed acute myeloid leukemia (AML) patients who received HAV treatment between June 2020 and April 2023. The HAV regimen includes venetoclax at 100 mg on day 0 and 200 mg from day 1 to 14, administered orally; homoharringtonine at 2 mg/m(2) from day 3 to 9, given by intravenous infusion; and cytarabine at 50 mg/m(2) from day 3 to 9, also administered by intravenous infusion. Among 33 patients, 36.4% (12/33) were male, with a median age of 60 years (range, 14-67 years). The overall response rate after one cycle of induction therapy was 90.9%, and the complete response (CR) rate was 87.9%. Among the patients, 79.3% achieved CR with measurable residual disease negativity. No patient died during induction therapy. The median recovery times of neutrophils (≥1.0×10(9)/L) and platelets (≥30×10(9)/L) were 10 (5-12) days and 10 (3-13) days, respectively. Until October 30, 2024, the estimated 3-year overall survival rate, event-free survival rate, and disease-free survival rate were 68.2% (95% CI: 59.7% -76.7% ), 66.7% (95% CI: 58.5% -74.9% ), and 73.6% (95% CI: 65.6% -81.6% ), respectively. HAV is a highly effective and safe induction therapy regimen for patients with newly diagnosed AML.","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 4","pages":"379-382"},"PeriodicalIF":0.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13195558/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147982862","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

[Characteristics and survival analysis of 60 cases of primary diffuse large B-cell lymphoma of the breast]. [60例原发性乳腺弥漫性大b细胞淋巴瘤的特点及生存分析]。

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20251110-00519

H Q Zhang, J Y He, S Y Chen, H L Zhang, L Wang, P P Xu, W L Zhao

{"title":"[Characteristics and survival analysis of 60 cases of primary diffuse large B-cell lymphoma of the breast].","authors":"H Q Zhang, J Y He, S Y Chen, H L Zhang, L Wang, P P Xu, W L Zhao","doi":"10.3760/cma.j.cn121090-20251110-00519","DOIUrl":"10.3760/cma.j.cn121090-20251110-00519","url":null,"abstract":"Objective: To investigate the characteristics and prognostic factors of primary breast diffuse large B-cell lymphoma (PB-DLBCL) . Methods: Clinical data on 60 patients with PB-DLBCL admitted to Ruijin Hospital, affiliated with Shanghai Jiao Tong University School of Medicine, from November 2013 to September 2022, were respectively collected, and survival and prognostic analyses were conducted. Among them, 44 patients underwent targeted sequencing (of 55 lymphoma-related genes) to assess the gene mutation status and its impact on survival outcomes. Results: All 60 patients with PB-DLBCL were female. The median age was 56.5 years (49.0, 61.0). The overall 5-year progression-free survival (PFS) rate was 66.5% (95% CI: 51.0% -86.7% ), and the 5-year overall survival (OS) rate was 90.6% (95% CI: 80.5% -100.0% ). After one-line treatment, 96.7% (58 patients) achieved complete remission (CR). The results of univariate analysis showed that elevated LDH (HR=3.380, 95% CI: 1.160-9.791, P=0.025) and nongerminal center B-cell (non-GCB) type (HR=3.560, 95% CI: 1.000-12.700, P=0.049) were adverse prognostic factors for PFS in patients with PB-DLBCL, whereas elevated LDH (HR=6.212, 95% CI: 1.150-38.941, P=0.034) was an adverse prognostic factor for OS. Multivariate analysis did not identify independent adverse prognostic factors for PFS in patients with PB-DLBCL. Targeted sequencing results showed that the mutation frequencies of PIM1, MYD88, KMT2D, CD79B, DTX1, and MPEG1 were >20%. Log-rank test revealed that patients with PIM1 mutations (n=27) had a significantly lower 5-year PFS rate compared to those without mutations (n=17) [59.8% (95% CI: 39.7% -90.0% ) vs 80.7% (95% CI: 58.3% -100% ), P=0.019]. Regarding MPEG1, patients without mutations (n=34) exhibited a significantly lower 5-year PFS rate than those with mutations (n=10) [59.4% (95% CI: 41.0% -86.1% ) vs 100%, P=0.048]. For DTX1, patients with mutations (n=10) had a significantly lower 5-year OS rate than those without mutations (n=34) [48.0% (95% CI: 18.8% -100% ) vs 100%, P=0.004]. Similarly, patients with MYD88 mutations (n=21) showed a significantly lower 5-year OS rate than those without mutations (n=23) [75.4% (95% CI: 52.9% -100% ) vs 100%, P=0.004]. Conclusion: The 5-year OS rate of PB-DLBCL is relatively high, whereas the PFS rate is relatively low. Elevated LDH levels and having the non-GCB type are adverse prognostic factors affecting patient survival. The mutation frequencies of PIM1, MYD88, KMT2D, CD79B, DTX1, and MPEG1 are higher in PB-DLBCL. Patients with mutations in PIM1, MYD88, and DTX1 have a poor prognosis, whereas those with MPEG1 mutations have a favorable prognosis.","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 4","pages":"372-378"},"PeriodicalIF":0.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13195559/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147983374","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 risk factors for early death in NPM1-mutated acute myeloid leukemia patients]. npm1突变急性髓系白血病患者早期死亡的危险因素分析

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20250929-00452

L L Wang, M K Shu, X Q Dou, Y N Ma, R Jiang, H H Chen, Y X Cheng, S N Chen

{"title":"[Analysis of risk factors for early death in NPM1-mutated acute myeloid leukemia patients].","authors":"L L Wang, M K Shu, X Q Dou, Y N Ma, R Jiang, H H Chen, Y X Cheng, S N Chen","doi":"10.3760/cma.j.cn121090-20250929-00452","DOIUrl":"10.3760/cma.j.cn121090-20250929-00452","url":null,"abstract":"Objective: To investigate the risk factors for early death (ED) in patients with NPM1-mutated acute myeloid leukemia (NPM1(mut) AML) . Methods: A retrospective analysis was conducted on 405 newly diagnosed primary NPM1(mut) AML patients admitted to the First Affiliated Hospital of Soochow University and the First People's Hospital of Yancheng between June 2018 and June 2023. Clinical and laboratory data at diagnosis were analyzed. Univariate and multivariate logistic regression analyses were used to explore the risk factors for early death in these patients. Results: The median age of the 405 NPM1(mut) AML patients was 53 (14-86) years, with 177 males and 228 females. The most common mutations at diagnosis were in DNMT3A (51.8% ), FLT3-ITD (51.3% ), FLT3-TKD (25.1% ), IDH2 (21.5% ), and TET2 (21.0% ). Thirty-five patients (8.6% ) experienced ED, with causes including 14 cases (40.0% ) of severe infection, 11 cases (31.4% ) of cerebral hemorrhage, 4 cases (11.4% ) of cerebral infarction, and 3 cases (8.6% ) of pulmonary hemorrhage. Compared with non-ED patients, ED patients had a significantly higher proportion of the CD34 and HLA-DR double-negative immunophenotype (CD34(-)/HLA-DR(-)) (71.4% vs 30.0%, P<0.001), a higher incidence of coagulopathy (80.0% vs 27.5%, P<0.001), and a higher incidence of TET2 mutations (40.0% vs 19.1%, P=0.004). Multivariate analysis showed that the Charlson comorbidity index (CCI) (OR=10.540, P=0.003), platelet count (OR=10.980, P=0.001), coagulopathy (OR=7.126, P=0.005), CD34(-)/HLA-DR(-) immunophenotype (OR=6.416, P=0.002), and infection (OR=59.080, P<0.001) were independent risk factors for ED in NPM1(mut) AML patients. Conclusion: Severe infection and bleeding were the main risk factors for ED in NPM1(mut) AML patients, and the CD34(-)/HLA-DR(-) immunophenotype should be considered in risk stratification and clinical management of patients with NPM1(mut) AML to guide early intervention.","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 4","pages":"358-363"},"PeriodicalIF":0.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13195547/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147983559","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

[Advances in ribosome biogenesis and targeted therapies for diffuse large B-cell lymphomas]. [核糖体生物发生和弥漫性大b细胞淋巴瘤的靶向治疗进展]。

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20250827-00403

M X Yue, W L Zhao, L Wang

{"title":"[Advances in ribosome biogenesis and targeted therapies for diffuse large B-cell lymphomas].","authors":"M X Yue, W L Zhao, L Wang","doi":"10.3760/cma.j.cn121090-20250827-00403","DOIUrl":"10.3760/cma.j.cn121090-20250827-00403","url":null,"abstract":"Ribosome biogenesis is an essential biological process for protein synthesis in eukaryotic cells and is also one of the most energy-consuming cellular processes. Recent multiomics studies have shown that ribosome biogenesis-related pathways are broadly upregulated in diffuse large B-cell lymphoma (DLBCL). These alterations are closely associated with molecular events, such as c-Myc rearrangement or overexpression, persistent activation of mTOR signaling, and TP53 pathway abnormalities, and are correlated with chemoresistance and poor prognosis. This review summarizes the structure of ribosomes, the major steps of ribosome biogenesis, and the regulatory networks governing them, with a particular focus on the role of dysregulated ribosome biogenesis in the pathogenesis and progression of DLBCL. Additionally, we systematically review the preclinical and clinical evidence for therapeutic strategies targeting ribosome biogenesis in DLBCL, including conventional chemotherapeutic agents like platinum compounds and anthracyclines, selective RNA polymerase I inhibitors, and XPO1 inhibitors. The potential clinical value of patient stratification, combination therapy, and resistance interventions based on ribosome biogenesis activity and TP53 status is also discussed. Current evidence suggests that dysregulated ribosome biogenesis is involved in DLBCL development and progression and may serve as an important basis for disease stratification and therapeutic intervention, providing a reference for future studies on precision treatment in DLBCL.","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 4","pages":"404-408"},"PeriodicalIF":0.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13195552/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147983650","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

[Long-term outcomes of first-line iFCR regimen in chronic lymphocytic leukemia/small lymphocytic lymphoma and the role of MRD]. [慢性淋巴细胞白血病/小淋巴细胞淋巴瘤一线iFCR方案的长期结局及MRD的作用]。

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20251125-00549

M Liu, Y Q Sha, S C Qin, Y Xia, Y Miao, H N Tang, Y J Wu, C Qiao, L Fan, J Y Li, H Y Zhu

{"title":"[Long-term outcomes of first-line iFCR regimen in chronic lymphocytic leukemia/small lymphocytic lymphoma and the role of MRD].","authors":"M Liu, Y Q Sha, S C Qin, Y Xia, Y Miao, H N Tang, Y J Wu, C Qiao, L Fan, J Y Li, H Y Zhu","doi":"10.3760/cma.j.cn121090-20251125-00549","DOIUrl":"10.3760/cma.j.cn121090-20251125-00549","url":null,"abstract":"Objective: This study aimed to investigate the clinical significance of measurable residual disease (MRD) in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) treated with ibrutinib plus fludarabine, cyclophosphamide, and rituximab (iFCR) regimen. Methods: Data of 34 patients with treatment-naive CLL/SLL treated with the iFCR regimen in the Department of Hematology of The First Affiliated Hospital of Nanjing Medical University from February 2019 to June 2025 were retrospectively collected and comprehensively analyzed. Data included MRD detected with flow cytometry (FCM) and NGS, efficacy evaluation, progression-free survival (PFS), and overall survival (OS). UMRD4 was defined as MRD of <10(-4) detected with FCM. According to peripheral blood MRD (PB-MRD) kinetics, patients were categorized into three groups: sustained uMRD4 (n=13), MRD relapse (n=4), and sustained MRD positive (n=7) . Results: Median follow-up was 60.2 (95% CI: 53.9-66.5) months, and the median PFS and median OS times were not reached. The 5-year PFS and OS rates were 58.6% (95% CI: 35.6% -96.6% ) and 94.4% (95% CI: 84.4% -100% ), respectively. Compared with patients with unmutated immunoglobulin heavy chain variable (IGHV) and TP53 aberration, patients demonstrating mutated IGHV (median PFS time: not reached vs 59.3 months, P= 0.019) and the absence of TP53 aberration (median PFS time: not reached vs 38.6 months, P=0.011) were associated with a longer PFS. After 2 months of combination therapy completion, patients with PB-uMRD4 achieved a higher rate of complete response (CR) compared with those with positive PB-MRD (73.3% vs 20.0%, P=0.015) and demonstrated a longer PB-uMRD4 duration during long-term follow-up (36.3±16.2 months vs 4.6±4.7 months, P<0.001). A good concordance was observed between PB-MRD and bone marrow MRD (BM-MRD) detected with FCM (P<0.001, r=0.739) and NGS (P<0.001, r=0.869). Further, the concordance of PB-MRD detection (P=0.003, r=0.439) between FCM and NGS was superior to that of BM-MRD (P=0.033, r=0.299). Treatment was either actively discontinued (those achieving uMRD4) or continued (those with detectable MRD) according to the BM-MRD level after 2 years of iFCR treatment. Furthermore, disease progression occurred in 1 (1/9, 11.1% ) patient who discontinued therapy and 7 (7/19, 36.8% ) patients who continued treatment. Among them, 7 patients who actively discontinued treatment and kept PB-uMRD4 and CR demonstrated no del (17p), del (11q), and TP53 mutations. The proportion of patients with complex karyotypes (7.7% vs 57.1%, P=0.031), del (17p) (0 vs 42.9%, P=0.031), and TP53 mutations (0 vs 42.9%, P=0.031) was lower in the sustained uMRD4 group compared with the sustained MRD-positive group. Conclusion:</","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 4","pages":"364-371"},"PeriodicalIF":0.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13195551/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147983434","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

[Clinical characteristics and prognostic analysis of adult KMT2A-associated acute myeloid leukemia]. 成人kmt2a相关性急性髓系白血病临床特点及预后分析

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20250526-00247

X Y Zhou, X E Wei, Y Liu, X Kong, J J Han, T Z Wang, Y F Shen, J Zhang, S L Xue, S N Chen, X W Tang, Z H Lin, J Chen, H Y Qiu

{"title":"[Clinical characteristics and prognostic analysis of adult KMT2A-associated acute myeloid leukemia].","authors":"X Y Zhou, X E Wei, Y Liu, X Kong, J J Han, T Z Wang, Y F Shen, J Zhang, S L Xue, S N Chen, X W Tang, Z H Lin, J Chen, H Y Qiu","doi":"10.3760/cma.j.cn121090-20250526-00247","DOIUrl":"10.3760/cma.j.cn121090-20250526-00247","url":null,"abstract":"Objective: To explore the clinicobiological characteristics and prognostic impact of different fusion partners of KMT2A in acute myeloid leukemia (AML) . Methods: The clinical features of 303 patients with KMT2A-associated AML who received treatment between January 2014 and August 2024 were retrospectively analyzed to investigate prognostic differences among fusion partner subgroups. Results: Among 303 patients with KMT2A-associated AML, the median age was 41 (16-82) years. KMT2A-PTD accounted for 18.2% (55/303), whereas KMT2A rearrangements (KMT2Ar) were observed in 81.8% (248/303). Among KMT2Ar AML patients, the complete remission (CR) rate after a course of induction treatment was 65.3% (162/248), whereas the 2-year overall survival (OS) rate and cumulative incidence of relapse (CIR) were 52.6% (95% CI: 46.3% -59.7% ) and 55.4% (95% CI: 44.8% -61.6% ), respectively. Prognoses varied across partner gene subtypes; the KMT2A::AFDN subgroup (n=69) had the poorest 2-year CIR of 68.5% (95% CI: 55.4% -78.5% ), whereas the uncommon KMT2Ar subgroup (n=31) had a 2-year CIR rate of 43.2% (95% CI: 24.8% -60.3% ). The group with KMT2A::MLLT3 and EVI1 overexpression (n=18) exhibited a significantly higher 2-year CIR, at 66.7% (95% CI: 38.5% -84.2% ) versus 44.0% (95% CI: 30.1% -57.0% ) for those with KMT2A::MLLT3 who did not show EVI1 overexpression (n=53) (P=0.018). Among patients with KMT2A-PTD, the CR rate after a course of treatment was 60.0% (33/55) ; venetoclax-based therapy yielded a significantly higher CR rate compared with nontargeted therapy: 86.7% (26/30) vs 28.0% (7/25) (P<0.001), with a 2-year OS rate of 68.7% (95% CI: 36.1% -87.1% ) versus 53.9% (95% CI: 32.2% -71.4% ) (P=0.035), and 2-year CIR rate of 34.2% (95% CI: 15.9% -53.5% ) versus 78.7% (95% CI: 54.2% -91.0% ) (P=0.002). Compared to low-intensity chemotherapy, intensive chemotherapy resulted in a significantly lower CR rate: 40.7% (11/27) versus 78.6% (22/28) (P=0.004), accompanied by a higher 2-year CIR[72.3% (95% CI: 47.6% -86.8% ) versus 41.7% (95% CI: 20.6% -61.6% ), P=0.017]. Among transplant patients, those undergoing hematopoietic stem cell transplantation (HSCT) in CR1 (n=156) had better outcomes than those transplanted in non-CR1 (n=62), with a 2-year OS rate of 74.1% (95% CI: 66.9% -82.0% ) versus 52.0% (95% CI: 40.6% -66.7% ) and 2-year CIR of 27.3% (95% CI: 20.0% -34.9% ) versus 91.9% (95% CI: 80.8% -96.7% ), respectively (both P<0.001). Multivariate Cox regression analysis showed that achieving CR after one induction cycle (HR=0.54, P<0.001; HR=0.67, P=0.010) and receiving allo-HSCT (HR=0.17, P<0.001; HR=0.41, P<0.001) were favorable prognostic factors for OS and CIR. KMT2A::AFDN was ","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 4","pages":"336-344"},"PeriodicalIF":0.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13195550/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147983446","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

[Aggressive NK-cell leukemia with CD5(+)CD10(-) monoclonal B cell lymphocytosis and hemophagocytic lymphohistiocytosis: a case report and literature review]. 侵袭性nk细胞白血病伴CD5(+)CD10（-）单克隆B细胞淋巴细胞增多症和噬血细胞淋巴组织细胞增多症1例报告并文献复习。

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20250920-00429

M Liu, X J Liu, Y Zhou, M N Zhang, D Hao, M Shi

{"title":"[Aggressive NK-cell leukemia with CD5(+)CD10(-) monoclonal B cell lymphocytosis and hemophagocytic lymphohistiocytosis: a case report and literature review].","authors":"M Liu, X J Liu, Y Zhou, M N Zhang, D Hao, M Shi","doi":"10.3760/cma.j.cn121090-20250920-00429","DOIUrl":"10.3760/cma.j.cn121090-20250920-00429","url":null,"abstract":"Aggressive natural killer (NK) -cell leukemia (ANKL) is a rare hematological malignancy characterized by the abnormal proliferation of NK cells. It progresses rapidly and has a high mortality rate. This article reports the diagnosis and treatment of a rare case of a patient with ANKL and concurrent CD5(+)CD10(-) monoclonal B-cell lymphocytosis and hemophagocytic lymphohistiocytosis (HLH), and the relevant literature was reviewed. The patient was a 73-year-old male who presented with fever and lymphadenopathy in multiple areas without any obvious cause for over 1 month. Laboratory examinations, including bone marrow cell morphology and biopsy, revealed two distinct populations of abnormal cells. Flow cytometry, immunohistochemical staining, and gene testing confirmed the diagnoses of ANKL and CD5(+)CD10(-) monoclonal B-cell lymphocytosis, with concurrent HLH. The patient was initially treated with etoposide and dexamethasone. After receiving three courses of chemotherapy based on pegaspargase and other combination regimens, the patient's condition deteriorated. Resuscitation treatment was ineffective; the patient died due to multiple organ failure.","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 4","pages":"390-393"},"PeriodicalIF":0.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13195554/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147983612","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

[Chinese guidelines for the clinical application of antibacterial drugs for febrile neutropenia (2026)]. [中国发热性中性粒细胞减少症抗菌药物临床应用指南（2026）]。

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2026-04-14 DOI: 10.3760/cma.j.cn121090-20260120-00041

引用次数: 0