中国实验血液学杂志最新文献

{"title":"[The Clinical Phenotype and Molecular Pathogenic Mechanism of a Family with Hereditary Coagulation Factor V Deficiency].","authors":"Li-Ping Guo, Chun-Xia Dong, Gang Wang, Mei-Fang Wang, Lin-Hua Yang","doi":"10.19746/j.cnki.issn.1009-2137.2024.06.028","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.06.028","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the clinical phenotype and molecular pathogenic mechanism of a hereditary coagulation factor V deficiency (FⅤD) family.</p><p><strong>Methods: </strong>A phase I assay was used to measure coagulation factors II, V, VII, VIII, IX, X, Ⅺ, Ⅻ (FⅡ∶C, FⅤ∶C, FⅦ∶C, FⅧ∶C, FⅨ∶C, FⅩ∶C, FⅪ∶C, FⅫ∶C), activated partial thromboplastin time (APTT) and prothrombin time (PT) to determine the clinical phenotype and molecular pathogenesis of F VD. Prothrombin time (PT) were used for phenotypic identification; high-throughput exome sequencing was applied to screen the whole gene variants, and Sanger sequencing was used to verify the suspected variants in <i>F5</i> gene; MutationTaster, PolyPhen-2 bioinformatics software was used to predict the pathogenicity of the variants, ClustalX software was used to analyze the amino acid conservatism, and PyMol software was used to simulate the model of the mutant protein.</p><p><strong>Results: </strong>The pre-documented patient had significantly prolonged PT and APTT, FⅤ∶C was only 5.45%, and there was no significant abnormality in TT, FIB and the rest of the coagulation factors. The mother, father and sister of the proband had prolonged PT and APTT, and FⅤ∶C was reduced to different degrees. Genetic testing revealed the presence of a c.286G>C (p.Asp96His) pure missense variant in exon 3 of <i>F5</i> in the prior witness, and a c.286G>C (p.Asp96His) heterozygous missense variant in father, mother, and sister of the proband. Bioinformatics analysis suggested that p.Asp96His was a pathogenic variant, and the associated amino acid site was highly conserved among 10 species. Protein simulation showed that the mutation of Asp96 to His96 could lead to the disappearance of the original hydrogen bond and the change of the distance, destroying the original hydrogen bond interaction force and affecting the stability of the protein structure.</p><p><strong>Conclusion: </strong>The <i>F5</i> exon 3 c.286G>C (p.Asp96His) missense variant may have contributed to the reduction of FⅤ∶C in the preexisting individual and family members, as well as being the genetic etiology of coagulation factor V deficiency.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 6","pages":"1822-1828"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Clinical Features and Prognosis of Patients with Primary Extranodal Diffuse Large B-Cell Lymphoma].","authors":"Si Yi, Xia Li, Huan Tao, Hong-Bing Ma, Jie Ji, Yu Wu, Ting Niu, Yong-Qian Jia","doi":"10.19746/j.cnki.issn.1009-2137.2024.06.012","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.06.012","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the clinical features, gene mutation profile, efficacy and prognostic factors of primary extranodal diffuse large B-cell lymphoma(EN-DLBCL).</p><p><strong>Methods: </strong>A retrospective analysis was performed for 382 patients with primary EN-DLBCL with complete clinical data who were treated in West China Hospital from January 2013 to January 2023, and their clinical characteristics,gene mutation profile, efficacy and prognostic factors were analyzed.</p><p><strong>Results: </strong>The median age of the 382 patients with EN-DLBCL was 56(18-89) years old. The male-to-female ratio was 1.12∶1, and the most common primary sites were gastrointestinal tract (31.7%), Wechsler ring (19.1%) and breast gland (7.1%). A total of 51 gene mutations were fund, and the most common frequencies of gene mutations were <i>TP53</i> (32.5%), <i>MYD88</i> (32.5%), and <i>CD79B</i> (30.0%). The median follow-up was 63 months, and the 5-year progression-free survival (PFS) rate was 74.5% and the 5-year overall survival (OS) rate was 89.6%. The adverse factors on PFS were as follows: >1 extranodal sites involvement ( <i>P</i> <0.001), P53≥50% ( <i>P</i> <0.001), hyper double expression(hDEL) of C-myc >50%/Bcl-2>70% ( <i>P</i> <0.001). The adverse factors affecting the OS of patients were as follows: >1 extranodal sites involvement ( <i>P</i> <0.001), P53≥50% (<i>P</i> < 0.001), hDEL( <i>P</i> <0.001). Chemotherapy combined with local radiotherapy could improve PFS (<i>P</i> =0.041) and OS (<i>P</i> =0.003), while R-CHOP+X (molecule agents as BTKi、HDACi、Lenalidomide) failed to show a significant difference in PFS (<i>P</i> =0.075) and OS (<i>P</i> =0.767). Among the 40 patients who underwent next-generation sequencing at high risk, there was no significant in PFS (<i>P</i> =0.849) and OS (<i>P</i> =0.500) of patients with positive MYD88 and/or CD79B mutations (MCD subtype) treated with BTKi and patients with negative <i>MYD88</i> and <i>CD79B</i> mutations.</p><p><strong>Conclusion: </strong>Primary EN-DLBCL can involve multiple organs or tissue sites. <i>TP53</i> , <i>MYD88</i> , and <i>CD79B</i> are the most common gene mutations. The efficacy of BTKi in patients with positive MCD subtypes at intermediate and high risk is not inferior to that in MCD-negative control patients.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 6","pages":"1711-1718"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Expression and Function of MAIT Cells in Patients with Newly Diagnosed Acute Myeloid Leukemia].","authors":"Qian Peng, Zhi-Tao Wang, Ren-Hua Huang, Hui-Ping Wang, Hao Xiao, Zhi-Min Zhai","doi":"10.19746/j.cnki.issn.1009-2137.2024.06.003","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.06.003","url":null,"abstract":"<p><strong>Objective: </strong>To explore the changes in number and immune function of mucosal-associated invariant T (MAIT) cells in peripheral blood of patients with newly diagnosed acute myeloid leukemia (AML), and its correlation with the occurrence and development of AML.</p><p><strong>Methods: </strong>Seventy-five clinical samples of patients with newly diagnosed AML and 48 healthy control samples in our hospital from January 2022 to February 2023 were included. Multiparametric flow cytometry was used to detect the number of MAIT cells, membrane surface markers, effector phenotypes and functional indicators in the samples.</p><p><strong>Results: </strong>Compared with healthy controls, the percentage of MAIT cells in CD3⁺ T cells in peripheral blood of newly diagnosed AML patients was significantly reduced ( <i>P</i> < 0.001). The percentage of MAIT cells in all CD3⁺ T cells in bone marrow of AML patients was similar to that in peripheral blood (<i>P</i> >0.05). Most of MAIT cells in peripheral blood of AML patients were effector memory T cells. Compared with healthy controls, the proportion of effector memory MAIT cells decreased ( <i>P</i> < 0.05), while the proportion of terminally differentiated effector memory MAIT cells and PD-1⁺MAIT cells increased significantly (both <i>P</i> < 0.05). AML patients' peripheral blood MAIT cells expressed significantly higher levels of granzyme B and perforin than healthy controls (both <i>P</i> < 0.05), and secreted significantly lower levels of cytokines such as gamma interferon and tumor necrosis factor α than healthy controls (both <i>P</i> < 0.001).</p><p><strong>Conclusion: </strong>Compared with healthy controls, the proportion of MAIT cells in AML patients is reduced and the expression of functional markers is abnormal, suggesting that their function is impaired and may be involved in the occurrence and development of AML.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 6","pages":"1644-1650"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915859","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Genetic Mutation Profile and Risk Stratification of Cytogenetically Normal Acute Myeloid Leukemia with <i><i>CEBPA-bZIP</i></i> Mutations Based on Multi-Gene Sequencing].","authors":"Lei-Ming Cao, Ming-Yue Liao, Ya-Lan Zhou, Hao Jiang, Qian Jiang, Ying-Jun Chang, Lan-Ping Xu, Xiao-Hui Zhang, Xiao-Jun Huang, Guo-Rui Ruan","doi":"10.19746/j.cnki.issn.1009-2137.2024.06.001","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.06.001","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the gene mutation profile and prognostic significance of adult cytogenetically normal acute myeloid leukemia (CN-AML) with <i>CEBPA-bZIP</i> mutation.</p><p><strong>Methods: </strong>Targeted sequencing was implemented on the diagnostic bone marrow DNA samples of 141 adult CN-AML subjects with <i>CEBPA-bZIP</i> mutation. The nomogram model for leukemia-free survival (LFS) rate was generated by combining genetic abnormalities and clinical data. Risk stratification was conducted based on prognostic variables and the effect of risk-adjusted consolidation therapy was investigated by Kaplan-Meier method.</p><p><strong>Results: </strong>Four variables were finally included in our nomogram model after multivariate Cox analysis, and an equation for risk score calculation was obtained, risk score=1.300 2×white blood cell (WBC) (≥18.77×10⁹/L)+1.406 5×<i>CSF3R</i> mutation positive+2.648 9× <i>KMT2A</i> mutation positive+1.012 8×DNA methylation-related genes mutation positive. According to the nomogram model, patients were further divided into low-risk group (score=0, <i>n</i>=46) and high-risk group (score>0, <i>n</i>=95). Prognostic analysis showed that the 5-year LFS rate, 5-year overall survival (OS) rate, and 5-year cumulative incidence of relapse (CIR) of patients who received allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the high-risk group were 93.5%, 97.1%, and 3.5%, while those in patients who received maintenance chemotherapy were 32.9%, 70.5%, and 63.4%, respectively. The differences were statistically significant (all <i>P</i> < 0.05). Allo-HSCT could significantly improve the prognosis of patients in high-risk group. However, no corresponding benefit was observed in the low-risk group.</p><p><strong>Conclusion: </strong>Adult CN-AML with <i>CEBPA-bZIP</i> mutation has a complex co-mutation pattern. The nomogram model based on mutations of <i>CFS3R, KMT2A</i> and DNA methylation-related genes together with WBC count can further divide this subset of patients into a relatively low-risk group and a relatively high-risk group. For individuals in the high-risk group, allo-HSCT is proposed as post-remission therapy. The above data will benefit the prognosis estimation and treatment decision for adult CN-AML with <i>CEBPA-bZIP</i> mutation.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 6","pages":"1631-1637"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915870","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Effect of JMJD3-IRF4 Signaling Pathway-Mediated Macrophage Polarization on the Malignant Biological Behavior of Multiple Myeloma Cells].","authors":"Yu-Jie Zhang, Qi-Hui Cao, Yong-Hong Yang","doi":"10.19746/j.cnki.issn.1009-2137.2024.05.024","DOIUrl":"10.19746/j.cnki.issn.1009-2137.2024.05.024","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;To investigate the effect of macrophage polarization mediated by Jumonji domain containing-3 (JMJD3)-interferon regulatory factor 4 (IRF4) signaling pathway on the malignant biological behavior of multiple myeloma (MM) cells.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;THP-1 monocytes were induced to differentiate into macrophages by phorbol myristate acetate (PMA). THP-1 macrophages were divided into control group (normal culture), M2 induction group ［added recombinant human interleukin (IL) -4, IL-13 proteins］, M2+JMJD3 protein group (added recombinant human IL-4, IL-13 and JMJD3 proteins) and M2+JMJD3 inhibitor group (added recombinant human IL-4, IL-13 proteins and JMJD3 inhibitor), the proportion of CD206&lt;sup&gt;+&lt;/sup&gt; cells was detected by flow cytometry, the levels of IL-10 and transforming growth factor-β (TGF-β) in the culture supernatant were detected by ELISA assay, the expression levels of arginase-1 &lt;i&gt;(Arg-1), JMJD3&lt;/i&gt; and &lt;i&gt;IRF4&lt;/i&gt; mRNA were detected by real-time quantitative PCR (qRT-PCR), and the expression levels of Arg-1, JMJD3 and IRF4 proteins were detected by Western blot. Correspondingly, human MM cells U266 were cultured with THP-1 macrophage culture supernatant of each group, Methyl thiazolyl tetrazolium (MTT) method and plate colony formation assay were used to detect cell proliferation, cell apoptosis was detected by flow cytometry, Western blot was used to detect the expression levels of apoptosis-promoting protein Bcl-2-associated X protein (Bax) and cleaved caspase-3 in cells, and Transwell assay was used to detect cell migration and invasion.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Compared with the control group, the proportion of CD206&lt;sup&gt;+&lt;/sup&gt; cells in THP-1 macrophages, the mRNA and protein expression levels of Arg-1, JMJD3 and IRF4, and the levels of IL-10 and TGF-β in the cell culture supernatant in M2 induction group were significantly increased (&lt;i&gt;P&lt;/i&gt; &lt;0.001), meanwhile, the proliferation activity and the number of clones of U266 cells were significantly increased (&lt;i&gt;P&lt;/i&gt; &lt;0.01), the apoptosis rate and the expression levels of apoptosis-promoting protein Bax and cleaved caspase-3 were significantly decreased (&lt;i&gt;P&lt;/i&gt; &lt;0.001), the numbers of migrated cells and invasive cells were increased (&lt;i&gt;P&lt;/i&gt; &lt;0.001). Compared with M2 induction group, the proportion of CD206&lt;sup&gt;+&lt;/sup&gt; cells in THP-1 macrophages, the mRNA and protein expression levels of Arg-1, JMJD3 and IRF4, and the levels of IL-10 and TGF-β in the cell culture supernatant in M2+JMJD3 protein group were further increased (&lt;i&gt;P&lt;/i&gt; &lt;0.01), meanwhile, the proliferation activity and the number of clones of U266 cells were further increased (&lt;i&gt;P&lt;/i&gt; &lt;0.05), the apoptosis rate and the expression levels of apoptosis-promoting protein Bax and cleaved caspase-3 were further decreased (&lt;i&gt;P&lt;/i&gt; &lt;0.01), the numbers of migrated cells and invasive cells were further increased (&lt;i&gt;P&lt;/i&gt; &lt;0.001); However, the change trends of the above index","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 5","pages":"1455-1462"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142547940","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Expression and Clinical Significance of Serum HDAC2 in Patients with Acute Myeloid Leukemia].","authors":"Jing Huang, Jing-Jing Yang, Yan-Chao Xu, Hui-Qi Zhang","doi":"10.19746/j.cnki.issn.1009-2137.2024.05.003","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.05.003","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the expression of serum histone deacetylase 2 (HDAC2) in patients with acute myeloid leukemia (AML) and its clinical significance.</p><p><strong>Methods: </strong>The 150 AML patients who received treatment in our hospital from April 2017 to April 2019 were included as AML group, and further divided into survival group (108 cases) and death group (42 cases) according to their survival status. In addition, 100 health individuals undergoing health examination in the same period were included as control group. The expression of HDAC2 in serum was detected by real-time quantitative PCR. Cox regression was used to analyze the influencing factors of adverse prognosis. The predictive effect of serum HDAC2 for the adverse prognosis of AML patients was evaluated by the receiver operating characteristic (ROC) curve.</p><p><strong>Results: </strong>Compared with the control group, the expression of serum HDAC2, interleukin (IL)-6, tumor necrosis factor (TNF)-α, IL-1β and C-reactive protein (CRP) in AML group was obviously increased (all <i>P</i> <0.05). Compared with the survival group, the expression of serum HDAC2 in the death group was also increased (<i>P</i> <0.05). Serum HDAC2 was positively correlated with IL-6, TNF-α, IL-1β and CRP in AML patients (<i>r</i> =0.567, 0.559, 0.623, 0.537). According to Cox regression analysis, the IL-6, TNF-α, IL-1β and HDAC2 were independent risk factors affecting the prognosis of AML patients (all <i>P</i> <0.05). ROC analysis showed that the AUC of serum HDAC2 level in predicting the adverse prognosis of AML was 0.862.</p><p><strong>Conclusion: </strong>The expression level of serum HDAC2 in AML patients is increased, which has positive correlations with IL-6, TNF-α, IL-1β and CRP. HDAC2 is an independent risk factor for the poor prognosis of AML patients, and has a high predictive value.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 5","pages":"1317-1322"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142547964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Clinical Characteristics and Prognosis of Children with Hypodiploid B-cell Precursor Acute Lymphoblastic Leukemia].","authors":"Cheng-Xuan Chen, Kai-Zhi Weng, Hong Wen, Shu-Quan Zhuang, Xing-Guo Wu, Yong-Zhi Zheng","doi":"10.19746/j.cnki.issn.1009-2137.2024.05.008","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.05.008","url":null,"abstract":"<p><strong>Objective: </strong>To analyze the clinical characteristics and prognosis of children with hypodiploid B-cell precursor acute lymphoblastic leukemia (BCP-ALL).</p><p><strong>Methods: </strong>The clinical data of 1 287 children with BCP-ALL admitted to five hospital in Fujian province from April 2011 to December 2020 were retrospectively analyzed. According to the results of chromosome karyotype, all the patients were grouped into hypodiploid subgroup and non-hypodiploid subgroup. The clinical characteristics, early treatment response ［minimal residual disease (MRD) on middle stage of induction chemotherapy and end of induction chemotherapy］ and long-term efficacy ［overall survival (OS) and event-free survival (EFS)］ were compared. The prognostic factors of hypodiploid BCP-ALL were further explored.</p><p><strong>Results: </strong>Among 1 287 BCP-ALL patients， 28 patients (2.2%) were hypodiploid BCP-ALL. The proportion of patients with white blood cell count （WBC）≥50×10⁹/L in the hypodiploid subgroup was significantly higher than that in the non-hypodiploid subgroup (<i>P</i> =0.004), while there was no statistically significant difference in gender ratio, age group at initial diagnosis, and early treatment response between the two groups (all <i>P</i> >0.05). The 5-year EFS and OS rate of the hypodiploid subgroup were 75.0%(95%<i>CI</i> ：66.8%-83.2%) and 77.8%(95%<i>CI</i> ：69.8%-85.8%), respectively, which were lower than those of non-hypodiploid subgroup ［EFS: 79.6%(95%<i>CI</i> ：78.4%-80.8%); OS: 86.4%(95%<i>CI</i> ：85.4%-87.5%)］, but the difference was not statistically significant (all <i>P</i> >0.05). Further subgroup analysis by risk stratification showed that the 5-year EFS and OS rates of the hypodiploid subgroup were significantly lower than those in the low-risk (LR) group ［LR group EFS: 91.4% (95%<i>CI</i> ：88.4%-93.6% ), <i>P</i> < 0.001; OS: 94.7% (95%<i>CI</i> ：92.1%-96.4%), <i>P</i> < 0.001］ ; it was similar to that of BCP-ALL children stratified into intermediate-risk (IR) excluding hypodiploid ［IR group EFS: 79.4%(95%<i>CI</i> ：74.9%-83.2%), <i>P</i> =0.343; OS: 87.3%(95%<i>CI</i> ：83.6%-90.2%), <i>P</i> =0.111］; while was higher than that of EFS in HR group, but the difference was not statistically significant ［HR group EFS: 58.7%(95%<i>CI</i> ：52.6%-64.8%), <i>P</i> =0.178. OS: 69.9%(95%<i>CI</i> ：63.5%-75.4%), <i>P</i> =0.417］. Univariate analysis showed that gender, age, white blood cell count, and MRD on middle stage of induction chemotherapy had no significant impact on OS and EFS; chromosome count< 40 was a risk factor for lower OS (<i>P</i> =0.026), but has no significant effect on EFS; MRD≥0.01% after induction therapy was a risk factor for lower OS and EFS (<i>P</i> =0.002, and 0.001, respectively).</p><p><strong>Conclusion: </strong>Children with hypodiploid BCP-ALL have an intermediate prognosis, and MRD ≥0.01% after induction chemotherapy may be a risk factors for poor prognosis.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 5","pages":"1356-1364"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142548029","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Effect of Endothelial Activation and Stress Index(EASIX) on Prognosis of Peripheral T-Cell Lymphoma Patient].","authors":"Hui-Min Chen, Rui-Xue Ma, Qian-Qian Zhang, Feng-Yi Lu, Jin Hu, Qian-Nan Han, Zhen-Yu Li, Kai-Lin Xu, Wei Chen","doi":"10.19746/j.cnki.issn.1009-2137.2024.05.014","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.05.014","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the effect of endothelial activation and stress index (EASIX) on the prognosis of patients with angioimmunoblastic T-cell lymphoma (AITL) and peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), and to compare the clinical characteristics of patients in the low EASIX and high EASIX groups.</p><p><strong>Methods: </strong>The clinical data of 59 newly diagnosed AITL and PTCL-NOS patients admitted to the Affiliated Hospital of Xuzhou Medical University from January 2010 to September 2021 were retrospectively analyzed. The optimal cut-off value of EASIX was determined by receiver operating characteristic (ROC) curve; The chi-square test was used to analyze the correlation between EASIX and clinical features of patients with AITL and PTCL-NOS; The Kaplan-Meier survival curve was used to analyze the overall survival (OS) and progression-free survival (PFS) of the patients; Univariate and multivariate analyses were performed by using Cox proportional hazards model.</p><p><strong>Results: </strong>The optimal cut-off value of EASIX was 0.95, based on which the patients were divided into a low EASIX (<0.95) group and a high EASIX (≥0.95) group. Compared with the low EASIX group, the high EASIX group had a higher proportion of patients with advanced Ann Arbor stage, higher risk according to IPI, elevated LDH, hypoproteinemia, anemia, B symptoms,extranodal involvement, and bone marrow involvement. Survival analysis showed that the OS and PFS of patients in the high EASIX group were significantly shorter than those in the lower EASIX group(<i>P</i> <0.001). The multivariate analysis showed that EASIX was an independent risk factor for OS ［<i>HR</i>=7.217 (95%<i>CI</i> : 1.959-26.587), <i>P</i> =0.003］ and PFS ［<i>HR</i>=2.718(95%<i>CI</i> : 1.032-7.161), <i>P</i> =0.043］ of PTCL patients.</p><p><strong>Conclusion: </strong>High EASIX in newly diagnosed patients with AITL and PTCL-NOS suggests a poor prognosis, and high EASIX is a risk factor affecting prognosis of the patients.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 5","pages":"1394-1400"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142547939","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Effect and Influencing Factors of Peripheral Blood Hematopoietic Stem Cells Collection from Unrelated Donors].","authors":"Huan Yang, Yu Tang, Hua Sun, Hong-Xia Xiang","doi":"10.19746/j.cnki.issn.1009-2137.2024.05.039","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.05.039","url":null,"abstract":"<p><strong>Objective: </strong>To analyze the effect of peripheral blood hematopoietic stem cells (PBSC) collection from unrelated donors and its influencing factors.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on the mobilization and collection of PBSC from 113 unrelated donors at Yueyang Central Hospital from January 2021 to December 2023.</p><p><strong>Results: </strong>113 donors were successfully mobilized. The average count of PBSC mononuclear cells (MNC) and CD34⁺ cells were (12.40±7.41)×10⁸/kg and (10.64±8.07)×10⁶/kg, respectively. Univariate analysis showed that the PBSC CD34⁺ cells ratio of male donors was significantly higher than that in female donors (<i>P</i> =0.015). The peripheral blood (PB) white blood cell (WBC) count before collection was positively correlated with the PBSC nucleated cells count (<i>r</i> =0.388), and the donor's body weight, the PB CD34⁺ cell ratio before collection were positively correlated with the PBSC CD34⁺ cell ratio (<i>r</i> =0.259, <i>r</i> =0.780). The daily dose of rhG-CSF was negatively correlated with the PBSC CD34⁺ cell ratio (<i>r</i> =-0.285). Both rhG-CSF agents achieved successful mobilization. Multivariate analysis showed that PB WBC count before collection was a factor affecting the count of PBSC nucleated cells (<i>P</i> <0.001), while the PB CD34⁺cell ratio before collection was a factor affecting the PBSC CD34⁺ cell ratio (<i>P</i> <0.001).</p><p><strong>Conclusion: </strong>The mobilization and collection of PBSC from unrelated donors are good, and the PB WBC count and CD34⁺ cell ratio before collection are reliable indicators for predicting the collection effect.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 5","pages":"1555-1559"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142547938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Different Prophylaxis Strategies for Central Nervous System Recurrence of Diffuse Large B-Cell Lymphoma].","authors":"Shuang Qu, Li-Sheng Liao, Yan-Bin Zheng, Jie-Song Wang, Hong-Ming He, Bi-Yun Chen, Hong Sun","doi":"10.19746/j.cnki.issn.1009-2137.2024.05.015","DOIUrl":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2024.05.015","url":null,"abstract":"<p><strong>Objective: </strong>To analyze the effects of highdose methotrexate (HD-MTX) and lenalidomide as central nervous system (CNS) prophylaxis strategies in patients with diffuse large B-cell lymphoma (DLBCL).</p><p><strong>Methods: </strong>The data of DLBCL patients with high risk of CNS recurrence who were initially treated in Fujian Provincial Hospital and Fujian Cancer Hospital from January 2012 to June 2022 were analyzed retrospectively. The patients were divided into HD-MTX group and lenalidomide group according to different prophylaxis strategies. Each group was further divided into high-risk group and medium-risk group based on CNS-IPI score and/or testicular involvement. The CNS relapse-free survival (CRFS) rate, adverse effects, and the effects of different prophylaxis strategies on overall survival (OS) rate and progression-free survival (PFS) rate were evaluated in different groups and subgroups.</p><p><strong>Results: </strong>There were 200 patients enrolled in this study, 80 cases in lenalidomide group and 120 cases in HD-MTX group. According to the delivery timing of prophylactic HD-MTX, the patients in HD-MTX group were further divided into two groups: 80 cases at the end of induction chemotherapy and 40 cases during chemotherapy interval. At a median follow-up of 48(14-133) months, the 4-year CRFS rate, 4-year PFS rate, and 4-year OS rate of the HD-MTX group was 93.6%, 57.2%, and 68.8%, respectively, while that of the lenalidomide group was 90.4%, 69.4% and 75.6%. There were no significant differences in 4-year CRFS rate, 4-year PFS rate, and 4-year OS rate between HD-MTX group and lenalidomide group (all <i>P</i> >0.05), but lenalidomide group showed a trend of improvement in PFS. Further subgroup analysis showed that there was no significant difference in 4-year CRFS rate between high-risk patients of the two groups (91.7% <i>vs</i> 83.4%, <i>P</i> >0.05), while 4-year PFS rate showed difference (49.5% <i>vs</i> 64.2%, <i>P</i> <0.05). A total of 248 cycles were collected for adverse reaction analysis in the HD-MTX group, and 25 cycles occurred neutropenia accompanied with infection (10.1%), while in lenalidomide group 240 cycles were collected in which 20 cycles occurred neutropenia accompanied with infection (8.3%). Both the two groups had no treatment-related deaths.</p><p><strong>Conclusion: </strong>Compared with HD-MTX, lenalidomide combined with immunochemotherapy can prevent CNS relapse, at the same time, improve prognosis, which is a safe and well tolerated central prophylaxis strategy.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 5","pages":"1407-1413"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142548037","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}