Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi最新文献

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[A multicenter retrospective clinical study of a simplified comprehensive geriatric assessment system in elderly patients with diffuse large B-cell lymphoma]. [一种简化的老年弥漫性大b细胞淋巴瘤患者老年综合评估系统的多中心回顾性临床研究]。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-02-14 DOI: 10.3760/cma.j.cn121090-20241121-00467
J Y Leng, Y H Cai, X P Ge, N P Zhao, Q Q Su, Z X Jia, J Qian, B Z Li, H Y Hua, X Z Lu, H Y Zhu, J Y Li, W Y Shi
{"title":"[A multicenter retrospective clinical study of a simplified comprehensive geriatric assessment system in elderly patients with diffuse large B-cell lymphoma].","authors":"J Y Leng, Y H Cai, X P Ge, N P Zhao, Q Q Su, Z X Jia, J Qian, B Z Li, H Y Hua, X Z Lu, H Y Zhu, J Y Li, W Y Shi","doi":"10.3760/cma.j.cn121090-20241121-00467","DOIUrl":"10.3760/cma.j.cn121090-20241121-00467","url":null,"abstract":"<p><p><b>Objective:</b> To evaluate the predictive value of simplified geriatric assessment (sGA) in elderly Chinese patients diagnosed with diffuse large B-cell lymphoma (DLBCL) . <b>Methods:</b> It retrospectively analyzed the relationships of sGA with the clinical characteristics, outcome, and prognosis of 219 patients aged ≥60 years who were newly diagnosed with DLBCL at six hospitals in Jiangsu province between January 2018 and December 2022. <b>Results:</b> The median age of 219 patients was 68 years (60-87 years). According to the sGA system criteria, 101 (46.1%), 103 (47.0%), and 15 (6.8%) elderly patients with DLBCL were categorized as fit, unfit, and frail, respectively. The most common adverse reactions after chemotherapy were hematologic, and the incidence of grade >2 hematologic adverse reactions was similar among the three groups (47.5% <i>vs</i> 41.7% <i>vs</i> 46.7%, respectively; <i>χ</i>(2)=0.712, <i>P</i>=0.700). Compared with the fit and unfit groups, the frail group showed tendencies toward for higher proportions of grade >2 gastrointestinal, pulmonary, and infectious adverse reactions (<i>P</i>>0.05 for all). The fit, unfit, and frail groups had respective remission rates of 74.3%, 46.6%, and 20.0% (<i>χ</i>(2)=25.249, <i>P</i><0.001) ; disease progression rates of 5.9%, 11.7%, and 26.7% (<i>χ</i>(2)=6.763, <i>P</i><0.05) ; 2-year overall survival rates of 92.1% (95% <i>CI</i> 86.6% to 97.9%), 77.6% (95% <i>CI</i> 69.5% to 86.6%), and 70.1% (95% <i>CI</i> 49.4% to 99.6%) (<i>P</i><0.05) ; and 2-year progression-free survival rates of 76.8% (95% <i>CI</i> 67.0% to 84.8%), 69.7% (95% <i>CI</i> 61.8% to 82.0%), and 65.7% (95% <i>CI</i> 53.3% to 100%) (<i>P</i>=0.399) . <b>Conclusion:</b> sGA can effectively predict treatment adverse effects and efficacy, disease progression, and long-term survival in elderly DLBCL.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 2","pages":"126-133"},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951222/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711370","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
[Efficacy and safety analysis of venetoclax in combination with multidrug chemotherapy in patients with newly diagnosed acute leukemia of ambiguous lineage]. 【venetoclax联合多药化疗治疗新诊断的不明谱系急性白血病的疗效及安全性分析】。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-02-14 DOI: 10.3760/cma.j.cn121090-20240419-00149
T Luo, Y R Fang, W J Liu, Q Sun, P Xu, M Hong, S X Qian
{"title":"[Efficacy and safety analysis of venetoclax in combination with multidrug chemotherapy in patients with newly diagnosed acute leukemia of ambiguous lineage].","authors":"T Luo, Y R Fang, W J Liu, Q Sun, P Xu, M Hong, S X Qian","doi":"10.3760/cma.j.cn121090-20240419-00149","DOIUrl":"10.3760/cma.j.cn121090-20240419-00149","url":null,"abstract":"<p><p><b>Objective:</b> To evaluate the efficacy and safety of venetoclax in combination with multidrug chemotherapy in patients with newly diagnosed acute leukemia of ambiguous lineage (ALAL) . <b>Methods:</b> A retrospective analysis of clinical data was performed on patients with newly diagnosed ALAL who were hospitalized at Jiangsu Provincial People's Hospital from June 2021 to July 2024. Of the 13 patients who received initial induction therapy with venetoclax combined with multidrug chemotherapy, 8 received VAA+P regimen, and 5 received V+IA regimen. Patients with FLT3 mutation were treated with FLT3 inhibitor, and Ph(+) patients received an additional tyrosine kinase inhibitor. Overall survival (OS), disease-free survival (DFS), and adverse events were analyzed. <b>Results:</b> According to the World Health Organization 5th edition of the classification of hematolymphoid tumors, the immunophenotypes were T/myeloid mixed-phenotype acute leukemia (MPAL) (<i>n</i>=4), B/myeloid MPAL (<i>n</i>=7), and ALAL- not otherwise specified (<i>n</i>=2). Of the seven patients with B/myeloid MPAL, four were Ph(+) and belonged to the group with specific gene abnormalities of ALAL. Three patients had FLT3 mutation (one with FLT3-TKD mutation and two with FLT3-ITD mutation). Prior to the second course of consolidation therapy, the efficacy of venetoclax induction therapy was evaluated, and a complete response rate of 100% was achieved in 13 patients. In the subsequent consolidation therapy phase, one patient discontinued treatment and was lost to follow-up; nine patients underwent allogeneic hematopoietic stem cell transplantation, four of whom died due to posttransplant complications and five achieved DFS. Of the three patients (≥70 years old) who received consolidation therapy as before, two achieved DFS and one died due to central nervous system leukemia. The median OS time was not reached in 13 patients; the 75th percentile survival time was 12.0 months, with a 12-month cumulative survival rate of 64.5%. The median DFS time was not reached in all patients; the 75th percentile DFS time was 8.2 months, with a 12-month cumulative DFS rate of 67.1%. All patients experienced grade 3 or 4 hematologic toxicity, including neutropenia and thrombocytopenia, during and after induction therapy. All patients recovered hematopoietic function after the initial induction therapy, with no fatal hemorrhage, tumor lysis syndrome, neurological adverse events, or grade 3 or higher organ toxicity, excluding preexisting conditions. <b>Conclusion:</b> Venetoclax in combination with multidrug chemotherapy was effective and associated with tolerable adverse reactions in patients with newly diagnosed ALAL.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 2","pages":"161-168"},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951214/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711397","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
[Mechanism of BIM-induced ibrutinib resistance in chronic lymphocytic leukemia]. [bim诱导的慢性淋巴细胞白血病依鲁替尼耐药机制]。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-02-14 DOI: 10.3760/cma.j.cn121090-20241121-00466
J L Zhang, B H Pan, J Z Wu, Y L Kong, L Wang, W Xu
{"title":"[Mechanism of BIM-induced ibrutinib resistance in chronic lymphocytic leukemia].","authors":"J L Zhang, B H Pan, J Z Wu, Y L Kong, L Wang, W Xu","doi":"10.3760/cma.j.cn121090-20241121-00466","DOIUrl":"10.3760/cma.j.cn121090-20241121-00466","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the relationship between the BCL2 family protein BIM and ibrutinib resistance in chronic lymphocytic leukemia (CLL) and to analyze its regulatory mechanisms on apoptosis and autophagy. <b>Methods:</b> RNA sequencing (RNA-seq) was used to examine changes in the expression of BCL2 family proteins in samples from patients with CLL, MEC1 cell lines, and ibrutinib-resistant cell lines (MR). Western blot was used to analyze changes in BIM protein expression during apoptosis in MR. shRNA knockdown was used to assess the effects of BIM on cell proliferation and apoptosis. RNA-seq and the autophagy inhibitor chloroquine treatment were used to study autophagy-related changes in MR. <b>Results:</b> BIM expression was significantly downregulated before and after drug resistance in CLL primary cells and MEC1 cell lines (<i>P</i><0.0001). Knockdown of BIM in CLL cells inhibited ibrutinib-induced apoptosis and promoted cell proliferation (<i>P</i><0.05 for both). In addition, protective autophagy was increased in MR and apoptosis was increased after administration of chloroquine and small interfering RNA. The increased expression of LC3-Ⅱ protein in BIM-knockdown cell lines (<i>P</i><0.01) suggested that reduction of BIM may mediate autophagy activation. <b>Conclusion:</b> Downregulation of BIM may be a key factor in promoting ibrutinib resistance in CLL by activating protective autophagy. These findings provided a potential target for improving CLL treatment.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 2","pages":"152-160"},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951217/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711390","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
[Rituximab combined with intensive immunochemotherapy for sporadic adult Burkitt lymphoma: efficacy and prognosis analyse]. [利妥昔单抗联合强化免疫化疗治疗散发性成人伯基特淋巴瘤:疗效及预后分析]。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-02-14 DOI: 10.3760/cma.j.cn121090-20241130-00505
C M Dong, H S Zou, W Zhang, W Liu, Y Wang, H M Liu, T Xie, H Li, Q Wang, W Y Huang, S H Yi, G An, L G Qiu, D H Zou
{"title":"[Rituximab combined with intensive immunochemotherapy for sporadic adult Burkitt lymphoma: efficacy and prognosis analyse].","authors":"C M Dong, H S Zou, W Zhang, W Liu, Y Wang, H M Liu, T Xie, H Li, Q Wang, W Y Huang, S H Yi, G An, L G Qiu, D H Zou","doi":"10.3760/cma.j.cn121090-20241130-00505","DOIUrl":"10.3760/cma.j.cn121090-20241130-00505","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To explore the therapeutic efficacy and prognostic factors of combined rituximab and intensive chemotherapy for sporadic adult Burkitt lymphoma (BL) . &lt;b&gt;Methods:&lt;/b&gt; This retrospective study examined the clinical and survival data of 30 patients newly diagnosed with BL between July 2011 and February 2023 at the Blood Diseases Hospital. Kaplan-Meier method was used for survival analysis, and the log-rank test was used for univariate analysis of prognostic factors. &lt;b&gt;Results:&lt;/b&gt; The median age of the 30 patients was 43 years (24 - 66 years), and the male to female ratio was 3: 2. Extranodal invasion was present in 80% of the patients, with involvement of the bone marrow in 53.3% and central nervous system in 10.0%. The Ann Arbor stage was Ⅲ and Ⅳ in 86.7%. According to the number of Burkitt Lymphoma International Prognostic Index (BL-IPI) risk factors, patients were classified as low risk (0) in 20.0%, intermediate risk (1) in 43.3%, and high risk (≥2) in 36.7%. All patients were treated with an induction regimen of rituximab combined with intensive chemotherapy, with objective and complete response rates of 80.0% and 76.7%, respectively. The median follow-up was 49 months (6-153 months), and the 5-year progression-free survival (PFS) and overall survival (OS) rates were both (76.7±7.7) %. All patients with limited stage (&lt;i&gt;n&lt;/i&gt;=4) achieved continuous complete remission (CCR). Patients who had high risk, advanced stage sensitive to induction therapy (&lt;i&gt;n&lt;/i&gt;=10) sequentially received first-line autologous hematopoietic stem cell transplantation (auto-HSCT) as consolidation therapy; 9 patients achieved CCR, whereas 1 patient with central nervous system invasion developed early disease progression and died. The BL-IPI low, intermediate, and high risk groups had respective 5-year PFS rates of (83.3±15.2) %, 100.0%, and (45.5±15.0) % (&lt;i&gt;P&lt;/i&gt;=0.0069) and OS rates of (83.3±15.2) %, 100.0%, and (45.5±15.0) % (&lt;i&gt;P&lt;/i&gt;=0.0075). The main adverse effects of induction therapy were myelosuppression and secondary infections, which were effectively managed by appropriate symptomatic treatment. Univariate analysis demonstrated that worse PFS was associated with BL-IPI score ≥2 (&lt;i&gt;HR&lt;/i&gt;=4.90, 95% &lt;i&gt;CI&lt;/i&gt; 1.02-23.45, &lt;i&gt;P&lt;/i&gt;=0.0329) ; extranodal invasion at ≥2 sites (&lt;i&gt;HR&lt;/i&gt;=12.62, 95% &lt;i&gt;CI&lt;/i&gt; 2.59-61.62, &lt;i&gt;P&lt;/i&gt;=0.0021) ; and failure to achieve first complete response (CR1) after induction therapy (&lt;i&gt;HR&lt;/i&gt;=31.86, 95% &lt;i&gt;CI&lt;/i&gt; 4.19-242.20, &lt;i&gt;P&lt;/i&gt;&lt;0.0001) . &lt;b&gt;Conclusions:&lt;/b&gt; Intensive immunochemotherapy regimens were effective and well-tolerated by adult patients with highly aggressive BL. Treatment efficacy was ideal in patients with limited-stage disease, whereas prognosis was unsatisfactory in patients with high-risk BL-IPI. Sequential first-line auto-HSCT consolidation therapy may further improve outcomes in patients with high-risk advanced-stage disease who are sensitive to induction therapy. BL-IPI score ≥2, ext","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 2","pages":"134-139"},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951218/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711471","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
[Progress in hematopoietic stem cell transplantation for acute myeloid leukemia]. [造血干细胞移植治疗急性髓系白血病进展]。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-02-14 DOI: 10.3760/cma.j.cn121090-20240422-00157
X L Zhu, X J Huang, Y Wang
{"title":"[Progress in hematopoietic stem cell transplantation for acute myeloid leukemia].","authors":"X L Zhu, X J Huang, Y Wang","doi":"10.3760/cma.j.cn121090-20240422-00157","DOIUrl":"10.3760/cma.j.cn121090-20240422-00157","url":null,"abstract":"<p><p>Acute myeloid leukemia (AML) is a malignant disease of myeloid hematopoietic stem/progenitor cells. Although new drugs have emerged in recent years, the status of hematopoietic stem cell transplantation (HSCT) in AML still remains irreplaceable. This article reviews the adjustment of indications for HSCT in AML, donor selection, and updates of conditioning regimens, new prevention strategies for post-transplant relapse, new targeted drugs, immunotherapy combined with HSCT to improve the prognosis of relapsed and refractory AML, and optimization of transplantation technology for elderly AML.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 2","pages":"179-185"},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951221/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711470","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
[Prognostic value of prolymphocyte percentage in chronic lymphocytic leukemia]. [慢性淋巴细胞白血病前淋巴细胞百分比的预后价值]。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-02-14 DOI: 10.3760/cma.j.cn121090-20241205-00537
Z L Zhang, J H Zhou, L X Xing, Y Wang, T L Qiu, R Wang, H Wang, L Fan, H Y Zhu, Y Miao, J Y Li
{"title":"[Prognostic value of prolymphocyte percentage in chronic lymphocytic leukemia].","authors":"Z L Zhang, J H Zhou, L X Xing, Y Wang, T L Qiu, R Wang, H Wang, L Fan, H Y Zhu, Y Miao, J Y Li","doi":"10.3760/cma.j.cn121090-20241205-00537","DOIUrl":"10.3760/cma.j.cn121090-20241205-00537","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the impact of peripheral blood prolymphocyte percentage on the prognosis of patients with chronic lymphocytic leukemia (CLL) . <b>Methods:</b> This study included 300 patients diagnosed with CLL at the Department of Hematology of Jiangsu Provincial People's Hospital from October 2011 to December 2020. The association between prolymphocyte percentage and other parameters was analyzed, and the optimal cutoff prolymphocyte percentage was determined by X-tile analysis. Further survival analysis and prognostic model construction were used to validate the predictive value of prolymphocyte percentage. <b>Results:</b> Of the 300 eligible patients with CLL who were enrolled, 50 received Bruton tyrosine kinase inhibitors (BTKi) as first-line treatment. The group with higher prolymphocyte percentage comprised more patients in the advanced stages (<i>P</i>=0.010) and had higher β(2)-microglobulin (<i>P</i><0.001), unmutated immunoglobulin heavy-chain variable region gene (<i>P</i><0.001), and TP53 aberration (<i>P</i>=0.004). The optimal cutoff percentage of prolymphocytes was 1%. Patients with a prolymphocyte percentage >1% had significantly shorter treatment-free survival (TFS) (<i>P</i><0.001) and overall survival time (<i>P</i>=0.007) than patients with a prolymphocyte percentage ≤1%. On multivariate analysis, prolymphocyte percentage >1% tended to have an independent prognostic value for TFS [<i>HR</i>=1.405 (95% <i>CI</i> 0.971~2.032), <i>P</i>=0.071]. Compared with the nomogram of CLL international prognostic index (CLL-IPI) alone, the nomogram of CLL-IPI combined with prolymphocyte percentage showed better discrimination (area under the curve: 0.778 <i>vs</i>. 0.637; <i>P</i>=0.006). In addition, patients with a prolymphocyte percentage >1% were more likely to progress after BTKi treatment (<i>P</i>=0.038) . <b>Conclusion:</b> Peripheral blood prolymphocyte percentage was associated with various clinical and biological parameters and prognosis among patients with treatment-naive CLL.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 2","pages":"140-146"},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951225/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711414","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
[Advancements in artificial intelligence for the precise diagnosis and treatment of hematological malignancies]. [人工智能在血液系统恶性肿瘤精准诊断和治疗中的进展]。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-02-14 DOI: 10.3760/cma.j.cn121090-20241022-00409
H X Yang, J Xiong, W L Zhao
{"title":"[Advancements in artificial intelligence for the precise diagnosis and treatment of hematological malignancies].","authors":"H X Yang, J Xiong, W L Zhao","doi":"10.3760/cma.j.cn121090-20241022-00409","DOIUrl":"10.3760/cma.j.cn121090-20241022-00409","url":null,"abstract":"<p><p>Hematological malignancy is a highly heterogeneous disease with complex biological characteristics and diverse clinical manifestations. Therefore, precise diagnosis and treatment are crucial and urgently needed. To further improve the accuracy of diagnosis and prognostication and to promote personalized therapy, artificial intelligence (AI) has been increasingly used. This study reviewed literature published in the last 5 years and summarized the application, benefits, and drawbacks of AI in the diagnosis, treatment, and prognosis of hematologic malignancies. Although AI can effectively improve the accuracy of diagnosis and therapy, low-quality data, poor interpretability of the model, and limited clinical transformation have impeded its popularization and application. In the future, the clinical application of AI in hematologic malignancy can be accelerated by establishing standards for clinical data processing, integrating multimodal information for accurate diagnosis and prognostication, and conducting systematic clinical verification of model algorithms.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 2","pages":"186-192"},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951223/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711375","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
[Familial erythrocytosis type 2 due to VHL germline mutations: a case report and literature review]. 【因VHL种系突变引起的家族性2型红细胞增多症1例报告及文献复习】。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-01-14 DOI: 10.3760/cma.j.cn121090-20241011-00390
N N Liu, L J Pan, Z J Xiao, Z F Xu
{"title":"[Familial erythrocytosis type 2 due to VHL germline mutations: a case report and literature review].","authors":"N N Liu, L J Pan, Z J Xiao, Z F Xu","doi":"10.3760/cma.j.cn121090-20241011-00390","DOIUrl":"10.3760/cma.j.cn121090-20241011-00390","url":null,"abstract":"<p><p>To enhance the understanding of familial erythrocytosis type 2 (ECYT2) resulting from compound heterozygous mutations in the VHL gene. <b>Methods:</b> We conducted a retrospective analysis of the case data from a patient with ECYT2 to investigate its pathogenesis, clinical features, diagnosis and treatment options, as well as prognosis, while also reviewing the relevant literature. <b>Results:</b> A 31-year-old man was admitted to the hospital due to facial and hand flushing that had persisted for 29 years. Whole exome sequencing revealed compound heterozygous mutations in VHL p.P81L and p.N90T. Both of his parents were found to carry only one of these heterozygous mutations, yet they exhibited normal phenotypes. Based on the patient's hematological tests, a clear diagnosis of ECYT2 was established. Following treatment with erythrocytapheresis and daily administration of aspirin at a dosage of 100 mg, the patient experienced relief from dizziness and headaches associated with blood hyperviscosity, without any thrombotic or bleeding complications during this period. <b>Conclusions:</b> ECYT2 is a rare group of autosomal recessive genetic disorders. This case of ECYT2, resulting from compound heterozygous mutations in the VHL gene, represents the first report in China. Clinically, it is characterized by elevated red cell mass, normal or increased serum erythropoietin levels, and normal hemoglobin oxygen affinity levels. These factors contribute to thrombotic and bleeding complications that can lead to early mortality.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 1","pages":"75-80"},"PeriodicalIF":0.0,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11886440/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143587437","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 features and prognosis of splenic marginal zone lymphoma with POD24]. 脾边缘区淋巴瘤伴POD24的临床特点及预后分析。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-01-14 DOI: 10.3760/cma.j.cn121090-20240729-00285
L Wang, Q Shi, W L Zhao, L Wang
{"title":"[Clinical features and prognosis of splenic marginal zone lymphoma with POD24].","authors":"L Wang, Q Shi, W L Zhao, L Wang","doi":"10.3760/cma.j.cn121090-20240729-00285","DOIUrl":"10.3760/cma.j.cn121090-20240729-00285","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the clinical characteristics, prognosis, and risk factors associated with disease progression within 24 months (POD24) after diagnosis in patients with splenic marginal zone lymphoma (SMZL) . <b>Methods:</b> Clinical data from 88 newly diagnosed SMZL patients treated at Ruijin Hospital, Shanghai Jiaotong University School of Medicine, between December 2009 and October 2022, were retrospectively analyzed. Patients were grouped based on the presence of POD24 for prognostic evaluation and comparison of clinical features. <b>Results:</b> There were 45 males (51.1% ) and 43 females (48.9% ), with a median age of 59 (24-82) years at the time of diagnosis. Ten (11.4% ) cases occurred POD24. The overall survival (OS) time and progression- free survival (PFS) time in the POD24 group were shorten than non-POD24 group [median OS time: 77 (11-159) months <i>vs</i> not reached, <i>P</i><0.001; 15 (4-24) months <i>vs</i> 121 (24-154) months, <i>P</i><0.001]. Univariate Cox analysis showed that Eastern Cooperative Oncology Group (ECOG) score ≥ 2 [<i>HR</i>=8.942 (95% <i>CI</i> 1.097-72.910), <i>P</i>=0.041], age-adjusted International Prognostic Index (aaIPI) score of high-risk [<i>HR</i>=5.070 (95% <i>CI</i> 1.256-20.461), <i>P</i>=0.023], POD24 [<i>HR</i>=14.049 (95% <i>CI</i> 3.339-59.107), <i>P</i><0.001], occurrence of tissue transformation [<i>HR</i>=7.819 (95% <i>CI</i> 1.952-31.316), <i>P</i>=0.004], and disease unremission status after initial treatment [<i>HR</i>=6.080 (95% <i>CI</i> 1.439-25.690), <i>P</i>=0.014] were the influencing factors for OS in SMZL patients. Multivariate analysis showed that POD24 [<i>HR</i>=5.859 (95% <i>CI</i> 1.249-27.475), <i>P</i>=0.025] and occurrence of tissue transformation [<i>HR</i>=5.520 (95% <i>CI</i> 1.050-29.009), <i>P</i>=0.044] were independent prognostic factors affecting OS. Univariate logistic analysis showed that ECOG ≥ 2 [<i>HR</i>=7.556 (95% <i>CI</i> 1.498-38.110), <i>P</i>=0.014], high risk of aaIPI score [<i>HR</i>=5.500 (95% <i>CI</i> 1.378- 21.945), <i>P</i>=0.016], occurrence of tissue transformation [<i>HR</i>=8.000 (95% <i>CI</i> 1.759-36.383), <i>P</i>=0.007], and disease unremission status after initial treatment [<i>HR</i>=9.136 (95% <i>CI</i> 2.216-37.675), <i>P</i>=0.002] were the influencing factors of POD24. Multifactorial analysis showed that disease unremission after initial treatment [<i>HR</i>=8.253 (95% <i>CI</i> 1.681- 40.518), <i>P</i>=0.009] was an independent risk factor affecting POD24. <b>Conclusions:</b> POD24 and tissue transformation are independent poor prognostic factors for OS in SMZL patients. Patients with POD24 are at a higher risk of developing tissue transformation. The failure to alleviate the disease after initial treatment is an independent risk factor affecting POD24 patients.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 1","pages":"81-87"},"PeriodicalIF":0.0,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11886443/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143587431","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 combined regimen based on obinutuzumab plus glucocorticoid for 4 cases of relapsed iTTP]. [单抗联合糖皮质激素治疗复发性iTTP 4例]。
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi Pub Date : 2025-01-14 DOI: 10.3760/cma.j.cn121090-20241107-00437
H J He, Y Li, H Tian, X Y Xu, J Su, X X Ge, D P Wu, Z Q Yu, J Yin
{"title":"[The combined regimen based on obinutuzumab plus glucocorticoid for 4 cases of relapsed iTTP].","authors":"H J He, Y Li, H Tian, X Y Xu, J Su, X X Ge, D P Wu, Z Q Yu, J Yin","doi":"10.3760/cma.j.cn121090-20241107-00437","DOIUrl":"10.3760/cma.j.cn121090-20241107-00437","url":null,"abstract":"<p><p><b>Objective:</b> To evaluate the efficacy and safety of obinutuzumab combined with glucocorticoid-based therapy in patients with relapsed immune thrombotic thrombocytopenic purpura (iTTP). <b>Methods:</b> This study analyzed the efficacy and adverse reactions of four patients with relapsed iTTP who were treated with a combination of obinutuzumab and glucocorticoids to assess the effectiveness and safety of the treatment. <b>Results:</b> All four patients had a history of multiple relapses and had previously undergone treatment with rituximab and bortezomib. Three patients exhibited additional autoantibodies. Following the combined therapy, all patients achieved clinical remission, with ADAMTS13 activity returning to normal levels and inhibitors testing negative. During a median follow-up period of 11 months (range: 3-17 months), all patients maintained sustained remission. No severe adverse events were reported during treatment or follow-up. <b>Conclusion:</b> The combination of obinutuzumab and glucocorticoid-based therapy is effective and safe for treating relapsed iTTP.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"46 1","pages":"70-74"},"PeriodicalIF":0.0,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11886434/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143587456","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
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