L Yan, H Xiong, F Long, Z Chen, Z Wang, L Yang, F Tao, Y Chen, N Song, M Wu
{"title":"[Clinical analysis on the impact of pretransplant iron overload on allogeneic hematopoietic stem cell transplantation for patients with acquired severe aplastic anemia in pediatric].","authors":"L Yan, H Xiong, F Long, Z Chen, Z Wang, L Yang, F Tao, Y Chen, N Song, M Wu","doi":"10.3760/cma.j.cn121090-20231214-00310","DOIUrl":"10.3760/cma.j.cn121090-20231214-00310","url":null,"abstract":"<p><p>This study aimed to investigate the effect of iron overload on the transplant outcomes of pediatric patients with severe aplastic anemia (SAA) undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). A retrospective analysis was conducted on the clinical data of 74 children with SAA who received allo-HSCT at the Hematology Department of Wuhan Children's Hospital between January 2018 and August 2022. Children with iron overload (serum ferritin >1 000 μg/L) before transplantation had a longer disease course, received more red blood cell transfusions, and had a higher number of CD34(+) cells infused. Moreover, iron overload significantly delayed the reconstitution of regulatory T cells after transplantation, increasing the incidence of hemorrhagic cystitis and grade Ⅲ-Ⅳ acute graft-versus-host disease after transplantation. However, iron overload did not significantly affect the overall survival and failure-free survival rates of the children.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"586-590"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310813/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971928","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}
{"title":"[Chinese expert consensus on the diagnosis and treatment of acute graft-versus-host disease after hematopoietic stem cell transplantation (2024)].","authors":"","doi":"10.3760/cma.j.cn121090-20240608-00214","DOIUrl":"10.3760/cma.j.cn121090-20240608-00214","url":null,"abstract":"<p><p>Despite the continuous improvement in the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT), acute graft-versus-host disease (GVHD) remains a major complication and cause of death. In recent years, with the emergence of new drugs for the prevention and treatment of acute GVHD and the update of a series of clinical studies, there have been varying degrees of changes in the routine prevention and treatment regimens for acute GVHD. Based on the main research achievements and the accumulation of clinical experience in this field in recent years, this consensus further updates the \"The Consensus on Allogeneic Hematopoietic Stem Cell Transplantation for Hematological Diseases in China-Acute Graft-Versus-Host Disease (2020) .</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"525-533"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310805/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971927","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}
{"title":"[Effect and molecular mechanism of hesperadin-induced ferroptosis in chronic myeloid leukemia K562 cells].","authors":"J Y Wei, L Li, H M Liu","doi":"10.3760/cma.j.cn121090-20231218-00323","DOIUrl":"10.3760/cma.j.cn121090-20231218-00323","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the effect and molecular mechanism of hesperadin in inducing ferroptosis in chronic myeloid leukemia cell line K562 cells. <b>Methods:</b> The effects of hesperadin on the viability, proliferation, and migration of K562 cells were detected though CCK8, EDU-594, and Transwell assays, and the apoptotic rate of K562 cells was detected by flow cytometry. In addition, C11-BODIPY and FerroOrange were utilized to detect intracellular lipid peroxidation and Fe(2+) levels. Meanwhile, the expression levels of ferroptosis-associated protein solute carrier family 7 member 11 (SLC7A11) and glutathione peroxidase 4 (GPX4) in cells were detected through Western blot. Lipid peroxidation and Fe(2+) levels were also detected after transfection of cells with SLC7A11 overexpression plasmid. <b>Results:</b> Hesperadin decreased cell viability in a dose-dependent manner with IC(50) of 0.544 μmol/L. Hesperadin concentrations of 0.4 and 0.8 μmol/L were selected for follow-up experiments. EDU-594, Transwell, and flow cytometry showed significantly decreased proliferation and migration rate of K562 cells after 0.4 and 0.8 μmol/L hesperadin treatment for 24 h, and the apoptosis rate was significantly increased compared with the control group (<i>P</i><0.05). Western blot indicated a downregulated expression of the antiapoptotic protein Bcl-2 and an elevated expression of proapoptotic proteins Bax and Caspase-3. Moreover, hesperadin increased intracellular lipid peroxidation and Fe(2+) levels compared with the control treatment (<i>P</i><0.05). The combination of ferroptosis inhibitor (Fer-1) and hesperadin could reverse the effect of hesperadin on K562 cells. The mRNA and protein levels of ferroptosis-related genes SLC7A11 and GPX4 were significantly decreased in the 0.8 μmol/L hesperadin-treated group (<i>P</i><0.05). SLC7A11 overexpression can inhibit hesperadin effect and alleviate ferroptosis. <b>Conclusion:</b> Hesperadin can promote ferroptosis in K562 cells by regulating the SLC7A11/GPX4 axis.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"577-585"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310802/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971933","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}
{"title":"[Chinese expert consensus on minimal residual disease detection in multiple myeloma based on bone marrow samples (2024)].","authors":"","doi":"10.3760/cma.j.cn121090-20240430-00167","DOIUrl":"10.3760/cma.j.cn121090-20240430-00167","url":null,"abstract":"<p><p>Multiple myeloma (MM) is the second most common hematologic malignant tumor. Standard holistic treatment model and new drug-based regimens have greatly improved the survival of patients with MM; however, minimal residual disease (MRD) causes relapse in most patients. Therefore, combining MRD testing on the basis of traditional serological efficacy evaluation is necessary to achieve a more accurate assessment of the patient's disease status. At present, next-generation flow cytometry (NGF) and next-generation sequencing (NGS) are the mainstream technologies for detecting MRD based on bone marrow samples. To standardize and normalize MRD detection, the expert group discussed and formulated Chinese expert consensus on the application of NGF and NGS technology for bone marrow MRD detection in patients with MM.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"534-541"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310804/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971926","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}
{"title":"[Research progress of tumor-infiltrating lymphocytes in malignant hematological diseases].","authors":"R B Zheng, Y Xiao","doi":"10.3760/cma.j.cn121090-20230912-00113","DOIUrl":"10.3760/cma.j.cn121090-20230912-00113","url":null,"abstract":"<p><p>In recent years, immunotherapy has been progressing rapidly in tumor treatment, among which, adoptive immunotherapy of immunologically active cells has also gained increasing attention in the treatment of malignant hematological diseases. Tumor-infiltrating lymphocytes are a heterogeneous class of T-cell-based lymphocytes with high heterogeneity. As an important component of the tumor microenvironment, TILs are crucial in the development of malignant tumors. TILs are a new type of immunoreactive cells discovered after lymphokine-activated killer cells, which can show high specificity and efficacy without the need for large amounts of interleukin-2. Tumor immunotherapy with TILs has shown encouraging results and is valuable in determining patient prognosis. In this paper, we review the composition and characteristics of TILs and their progress in malignant hematologic diseases.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"610-614"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310799/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971995","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}
{"title":"[Dasatinib monotherapy for intramedullary T-lymphocyte blast crisis in chronic myeloid leukemia: a case report].","authors":"Y Zhang, J B Yang, Y M Ma","doi":"10.3760/cma.j.cn121090-20240110-00016","DOIUrl":"10.3760/cma.j.cn121090-20240110-00016","url":null,"abstract":"","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"608-609"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310809/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971931","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}
Y Y Mao, H Cai, X X Cao, J Feng, L Zhang, D B Zhou, J Li
{"title":"[Type of CEBPA mutations in acute myeloid leukemia and their effect on prognosis].","authors":"Y Y Mao, H Cai, X X Cao, J Feng, L Zhang, D B Zhou, J Li","doi":"10.3760/cma.j.cn121090-20240316-00098","DOIUrl":"10.3760/cma.j.cn121090-20240316-00098","url":null,"abstract":"<p><p><b>Objective:</b> To demonstrate the type of CEBPA gene mutations among patients with acute myeloid leukemia (AML), clinical characteristics, and prognostic effect on patient outcomes. <b>Methods:</b> Demographic data, clinical features, laboratory characteristics, and data about treatment and follow-up of 57 patients with CEBPA mutated AML diagnosed at Peking Union Medical College Hospital between April 2016 and November 2022 were collected and analyzed. <b>Results:</b> In total, 57 patients with CEBPA mutation accounted for 16.1% of all the 353 patients with AML, among which 28 patients had CEBPA-bZIPinf and 29 had CEBPA-other. Compared with the CEBPA-other group, the CEBPA-bZIPinf group was younger (54 <i>vs</i> 64 years, <i>P</i>=0.010), de novo AML was more common (<i>P</i>=0.001), and the level of bone marrow blast was higher (68.0% <i>vs</i> 36.3%, <i>P</i>=0.001). Moreover, 24 patients from the CEBPA-bZIPinf group and 19 from the CEBPA-other group received chemotherapy. The one-course complete remission (CR) rate of the CEBPA-bZIPinf group was significantly higher than that of the CEBPA-other (87.5% <i>vs</i> 47.4%, <i>P</i>=0.010) and CEBPA-wt (87.5% <i>vs</i> 50.3%, <i>P</i>=0.002) groups. After a median follow-up of 11 months, the median OS of the CEBPA-bZIPinf group was significantly longer than that of the CEBPA-wt group (not reached <i>vs</i> 22.1 months, <i>P</i>=0.012) . <b>Conclusion:</b> CEBPA-bZIPinf mutated AML is a unique clinical entity, with a younger age of diagnosis, better response to chemotherapy, and better prognosis.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"556-560"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310808/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971998","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}
S Q Ma, X Bai, L J Cao, Z N Ma, Z X Ding, Z J Yu, M Jiang
{"title":"[Rare VPS33B gene mutation combined with GP1BA mutation causes severe decrease in plasma VWF levels: a case report and literature review].","authors":"S Q Ma, X Bai, L J Cao, Z N Ma, Z X Ding, Z J Yu, M Jiang","doi":"10.3760/cma.j.cn121090-20231216-00317","DOIUrl":"10.3760/cma.j.cn121090-20231216-00317","url":null,"abstract":"<p><p>A 28-year-old woman was found to have coagulation factor Ⅷ activity (FⅧ∶C) <1% and von Willebrand factor antigen (VWF∶Ag) <1% during routine prenatal examinations. No pathogenic variation was found in the exon region of the VWF gene using next-generation sequencing. The clinical presentation of this patient does not match the clinical characteristics of type Ⅲ hemophilia [von Willebrand disease (VWD) ]; therefore, third-generation sequencing technology was used to perform whole-genome sequencing on the patient and her family members. Multiple members of the patient's paternal family carried a heterozygous variant of VPS33B, c.869G>C. The family members carrying this variant all had varying degrees of reduced VWF levels (39% -56% ). Moreover, the proband was detected with the heterozygous variant c.1474dupA in GP1BA. The ACMG and Clinvar databases determined that this variation was associated with platelet-type pseudo VWD. The decrease in VWF levels caused by heterozygous variations in VPS33B in families is the first international report, and no previous studies have reported cases of severe decrease in plasma VWF levels caused by double heterozygous variations in VPS33B and GP1BA.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"602-605"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310816/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971937","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}
G X Xu, W M Jin, B D Ye, S F Jiang, C Hu, X Huang, B S Xie, H F Jiang, L L Chen, R X Yao, Y Lu, L J Li, J Zhang, G F Ouyang, Y W Hong, H W Kong, Z J Qiu, W J Luo, B B Chu, H Q Zhang, H Zeng, X J Zhou, P F Shi, Y Xu, J Jin, H Y Tong
{"title":"[Analysis and summary of clinical characteristics of 289 patients with paroxysmal nocturnal hemoglobinuria in Zhejiang Province].","authors":"G X Xu, W M Jin, B D Ye, S F Jiang, C Hu, X Huang, B S Xie, H F Jiang, L L Chen, R X Yao, Y Lu, L J Li, J Zhang, G F Ouyang, Y W Hong, H W Kong, Z J Qiu, W J Luo, B B Chu, H Q Zhang, H Zeng, X J Zhou, P F Shi, Y Xu, J Jin, H Y Tong","doi":"10.3760/cma.j.cn121090-20240127-00041","DOIUrl":"10.3760/cma.j.cn121090-20240127-00041","url":null,"abstract":"<p><p><b>Objective:</b> To further improve the understanding of paroxysmal nocturnal hemoglobinuria (PNH), we retrospectively analyzed and summarized the clinical characteristics, treatment status, and survival status of patients with PNH in Zhejiang Province. <b>Methods:</b> This study included 289 patients with PNH who visited 20 hospitals in Zhejiang Province. Their clinical characteristics, comorbidity, laboratory test results, and medications were analyzed and summarized. <b>Results:</b> Among the 289 patients with PNH, 148 males and 141 females, with a median onset age of 45 (16-87) years and a peak onset age of 20-49 years (57.8% ). The median lactic dehydrogenase (LDH) level was 1 142 (604-1 925) U/L. Classified by type, 70.9% (166/234) were classical, 24.4% (57/234) were PNH/bone marrow failure (BMF), and 4.7% (11/234) were subclinical. The main clinical manifestations included fatigue or weakness (80.8%, 235/289), dizziness (73.4%, 212/289), darkened urine color (66.2%, 179/272), and jaundice (46.2%, 126/270). Common comorbidities were hemoglobinuria (58.7% ), renal dysfunction (17.6% ), and thrombosis (15.0% ). Moreover, 82.3% of the patients received glucocorticoid therapy, 70.9% required blood transfusion, 30.7% used immunosuppressive agents, 13.8% received anticoagulant therapy, and 6.3% received allogeneic hematopoietic stem cell transplantation. The 10-year overall survival (OS) rate was 84.4% (95% <i>CI</i> 78.0% -91.3% ) . <b>Conclusion:</b> Patients with PNH are more common in young and middle-aged people, with a similar incidence rate between men and women. Common clinical manifestations include fatigue, hemoglobinuria, jaundice, renal dysfunction, and recurrent thrombosis. The 10-year OS of this group is similar to reports from other centers in China.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"549-555"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310803/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971924","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}
J Zhang, R Ma, X Y Luo, X H Zhang, L P Xu, Y Wang, X D Mo, M Lyu, K Y Liu, X J Huang, Y Q Sun
{"title":"[Clinical characteristics of human parvovirus B19 infection after allogeneic stem cell transplantation].","authors":"J Zhang, R Ma, X Y Luo, X H Zhang, L P Xu, Y Wang, X D Mo, M Lyu, K Y Liu, X J Huang, Y Q Sun","doi":"10.3760/cma.j.cn121090-20231128-00284","DOIUrl":"10.3760/cma.j.cn121090-20231128-00284","url":null,"abstract":"<p><p>Human parvovirus B19 (HPVB19) belongs to Parvoviridae, a genus of erythrovirus, and has been associated with various human diseases, and HPVB19 infection is one of the most important causes of refractory anemia after allogeneic hematopoietic stem cell transplantation (allo-HSCT). This study retrospectively analyzed 24 patients with HSCT combined with HPVB19 infection to collate and summarize the clinical presentation, treatment, and regression of patients with combined HPVB19 infection after allo-HSCT and provide experience in the management of HPVB19 infection after allo-HSCT. The median age of the patients with HPVB19 infection was 25 years, and the median time of infection occurrence was +107 days after transplantation, and 22 (91.7% ) had anemia with a median hemoglobin (HGB) level of 77.5 (46-149) g/L, and 13 (54.2% ) had new-onset anemia or persistent decline in HGB. The median length of hospital stay was 19 days. Among patients with new-onset anemia or persistent decline in HGB, the mean increase in HGB after treatment with intravenous immunoglobulin and/or antiviral therapy was 15.69 g/L, and treatment was effective in 10 (76.92% ) patients. HPVB19 infection should be alerted to the development of refractory anemia after HSCT; despite the lack of specific treatment, the overall prognosis of HPVB19-infected patients is good.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 6","pages":"591-593"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11310815/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971929","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}