Annals of Hematology最新文献_第8页

First clinical and pedigree study of rare HBB: c.316-90 A > G variant in β-globin gene in Chinese population using third-generation sequencing. 中国人群β-珠蛋白基因c.316-90 A . > G变异的首次临床和家系研究

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-31 DOI: 10.1007/s00277-024-06168-y

Jianlong Zhuang, Nan Huang, Yu Zheng, Na Zhang, Chunnuan Chen

{"title":"First clinical and pedigree study of rare HBB: c.316-90 A > G variant in β-globin gene in Chinese population using third-generation sequencing.","authors":"Jianlong Zhuang, Nan Huang, Yu Zheng, Na Zhang, Chunnuan Chen","doi":"10.1007/s00277-024-06168-y","DOIUrl":"https://doi.org/10.1007/s00277-024-06168-y","url":null,"abstract":"Introduction: β-thalassemia is a common genetic disease mainly caused by point mutations in the β-globin gene, eliciting a high prevalence in South China. The aim of the present study is to identify a rare HBB: c.316-90 A > G variant and provide the clinical and hematological features in two unrelated Chinese families.Methods: In this study, we collected eight subjects from two unrelated Chinese families. Conventional thalassemia gene testing was performed to investigate common α and β-thalassemia variants based on the PCR reverse dot hybridization technique. Third-generation sequencing (TGS) was utilized to examine the rare or novel HBA1, HBA2 and HBB gene variants, which will be further verified using Sanger sequencing.Results: A rare HBB: c.316-90 A > G variant was identified in the proband of Family 1 using TGS, and exhibited remarkably low levels of hemoglobin (Hb), Hb A2, MCV and MCH. The other members in Family 1 did not have the HBB: c.316-90 A > G variant and elicited normal hematological screening results. In Family 2, the proband also carried the HBB: c.316-90 A > G variant and exhibited low levels of MCV, MCH and Hb A2, but with normal Hb value. However, pedigree analysis results revealed that the proband's mother and nephew also carried the HBB: c.316-90 A > G variant, but with normal hematological screening results.Conclusion: This study first conducts clinical and hematological analysis of the HBB: c.316-90 A > G variant in two unrelated Chinese families, which provides valuable data for genetic counseling of the corresponding individuals.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909112","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Severe aplastic anemia with acquired X chromosome clonality as a sole abnormality. 以获得性X染色体克隆为唯一异常的严重再生障碍性贫血。

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-31 DOI: 10.1007/s00277-024-06166-0

Tim Jang, Rachel D Burnside, Joanna Chaffin, Robert Seifert, John W Hiemenz

引用次数: 0

Long-term remission following CAR-T therapy in a patient with transformed follicular lymphoma relapse after allogeneic stem cell transplantation. 同种异体干细胞移植后转化性滤泡淋巴瘤复发患者CAR-T治疗后的长期缓解。

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-30 DOI: 10.1007/s00277-024-06150-8

Ryuta Kubo, Koichi Onodera, Yasushi Onishi, Noriko Fukuhara, Hideo Harigae

{"title":"Long-term remission following CAR-T therapy in a patient with transformed follicular lymphoma relapse after allogeneic stem cell transplantation.","authors":"Ryuta Kubo, Koichi Onodera, Yasushi Onishi, Noriko Fukuhara, Hideo Harigae","doi":"10.1007/s00277-024-06150-8","DOIUrl":"https://doi.org/10.1007/s00277-024-06150-8","url":null,"abstract":"Follicular lymphoma (FL) may undergo histological transformation (HT) into a more aggressive lymphoma. Although rituximab for B-cell non-Hodgkin lymphomas (B-NHL) has greatly improved the overall survival (OS) of patients with transformed FL (tFL), relapse after anthracycline-based chemoimmunotherapy has a poor prognosis. CD19-targeting chimeric antigen receptor-modified T-cell (CAR-T) therapy is a promising treatment for relapsed or refractory (r/r) large B-cell lymphoma (LBCL), including tFL. However, lymphopenia and reduced T-cell fitness caused by bendamustine exposure for treatment of underlying FL may impair the feasibility and reduce the efficacy of CAR-T therapy. Herein, we report the case of a 44-year-old woman with tFL who relapsed following allogeneic hematopoietic stem cell transplantation (allo-HSCT) and received CAR-T therapy. The patient could not initially undergo CAR-T therapy due to lymphopenia caused by bendamustine exposure, but CAR-T therapy became feasible following allo-HSCT. Although CAR-T therapy using T cells harvested from an allo-HSCT recipient may theoretically cause alloreactivity, the patient did not experience graft versus host disease (GVHD) or serious complications specific to CAR-T therapy, such as cytokine release syndrome (CRS) or immune effector cell-associated neurotoxicity syndrome (ICANS), and she has remained in complete response (CR) for >18 months. CAR-T therapy following allo-HSCT for patients with r/r tFL may be a safe and effective treatment option. Allo-HSCT may enhance the efficacy of CAR-T therapy.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909115","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Validation and improvement of the molecular international prognostic scoring system in Chinese patients with myelodysplastic syndromes. 在中国骨髓增生异常综合征患者中验证和改进国际分子预后评分系统。

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-30 DOI: 10.1007/s00277-024-06162-4

Nanfang Huang, Yang Song, Lingyun Wu, Qi He, Zheng Zhang, Juan Guo, Feng Xu, Chunkang Chang, Xiao Li

{"title":"Validation and improvement of the molecular international prognostic scoring system in Chinese patients with myelodysplastic syndromes.","authors":"Nanfang Huang, Yang Song, Lingyun Wu, Qi He, Zheng Zhang, Juan Guo, Feng Xu, Chunkang Chang, Xiao Li","doi":"10.1007/s00277-024-06162-4","DOIUrl":"https://doi.org/10.1007/s00277-024-06162-4","url":null,"abstract":"Various prognostic models have been proposed to improve the accuracy of prognostic assessment for Myelodysplastic syndromes (MDS). Recently, the Molecular International Prognostic Scoring System (IPSS-M) has been developed. Here, we validated the accuracy of IPSS-M in Chinese MDS patients, and proposed a prognostic model more suitable for Chinese patients. We analyzed the clinical, molecular and cytogenetic data of 798 primary MDS patients, and compared the accuracy of IPSS-R and IPSS-M in predicting overall survival (OS) and acute myeloid leukemia (AML) transformation. Using Cox proportional hazards model, we screened out 14 genes that had significant impacts on OS and AML progression. In our study, 44.86% of individuals were reclassified from IPSS-R to IPSS-M, of whom 64.80% were upstaged and 35.2% were downstaged. IPSS-M showed better performance than IPSS-R in predicting AML transformation (C-index: 0.84 vs. 0.81), but it was similar to IPSS-R in OS (C-index: 0.77 vs. 0.76). By combining age, mutational data and IPSS-R, we developed a new prognostic model more suitable for the Chinese patients (c-index was 0.81 for OS and 0.89 for AML transformation, respectively).","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909123","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Comparative safety of different first-line treatments for chronic lymphocytic leukemia/small lymphocytic lymphoma: A systematic review and network meta-analysis. 慢性淋巴细胞白血病/小淋巴细胞淋巴瘤不同一线治疗的安全性比较：系统综述和网络荟萃分析

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-28 DOI: 10.1007/s00277-024-06136-6

Qingyun Liu, Jiaxing Zhao, Yumiao Li, Youchao Jia

{"title":"Comparative safety of different first-line treatments for chronic lymphocytic leukemia/small lymphocytic lymphoma: A systematic review and network meta-analysis.","authors":"Qingyun Liu, Jiaxing Zhao, Yumiao Li, Youchao Jia","doi":"10.1007/s00277-024-06136-6","DOIUrl":"https://doi.org/10.1007/s00277-024-06136-6","url":null,"abstract":"The first-line treatment for chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) has recently undergone major changes, and targeted therapies have ushered in a new era of CLL/SLL treatment. Scientists in different countries have successively analyzed the efficacy of various drugs, but safety studies are relatively insufficient. Therefore, this systematic evaluation and retrospective meta-analysis was conducted to compare the differences in adverse effects and their incidence among first-line treatment regimens for CLL/SLL. We searched the Cochrane Library, PubMed, Web of Science, and Embase databases, with a cutoff date of December 2023. Frequency-based network meta-analysis was performed using STATA 16.0, and the risk of bias was assessed using the Cochrane Risk of Bias Assessment Tool (RoB2.0). Thirty-seven randomized controlled trials involving 15,557 patients were included, and the results showed that, compared with other regimens, zanubrutinib had a lower probability of causing adverse hematologic effects and a lower probability of causing severe anemia (SUCRAs: 79. 6%), all-grade anemia (SUCRAs: 87.2%), severe thrombocytopenia (SUCRAs: 97.0%), all-grade thrombocytopenia (SUCRAs: 90.6%), severe neutropenia (SUCRAs: 91.8%) and all-grade neutropenia (SUCRAs: 86.6%) than the other regimens. The higher rates of adverse reactions seen with each of the other first-line regimens were not concentrated in any single regimen. The second-generation BTK inhibitors may have a lower probability of causing hematologic adverse reactions. However, its adverse effects in other systems are still noteworthy. The cardiovascular toxicity of venetoclax combination regimens should not be overlooked.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142891482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Study on clinical features of early death in older patients with acute promyelocytic leukemia. 老年急性早幼粒细胞白血病患者早期死亡的临床特征研究。

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-27 DOI: 10.1007/s00277-024-06165-1

Yanqiu Zhao, Xinru Yu, Liru Yan, Deli Shi, Jinqiao Li, Wenyi Hou, Qisheng Zhang, Yuxin Zhu, Yingmei Zhang

{"title":"Study on clinical features of early death in older patients with acute promyelocytic leukemia.","authors":"Yanqiu Zhao, Xinru Yu, Liru Yan, Deli Shi, Jinqiao Li, Wenyi Hou, Qisheng Zhang, Yuxin Zhu, Yingmei Zhang","doi":"10.1007/s00277-024-06165-1","DOIUrl":"https://doi.org/10.1007/s00277-024-06165-1","url":null,"abstract":"This study aimed to analyze the baseline clinical characteristics, as well as the clinical features and risk factors of early death (ED) in older patients with acute promyelocytic leukemia (APL). A retrospective analysis was conducted on 198 consecutive older patients (age ≥ 50 years) with newly diagnosed APL who received arsenic trioxide alone as induction therapy, with 354 younger patients (age < 50 years) as controls. Ten easily obtainable clinical parameters were selected. Compared with the younger group, the older group had less fever, lower white blood cell (WBC) count, lower levels of albumin, and higher levels of creatinine upon admission. While the younger group had higher levels of aspartate aminotransferase and lower levels of fibrinogen (all P < 0.05). The ED rate was significantly higher in the older group (21.7% vs. 12.4%; P = 0.004). The causes of ED in the older group were bleeding, infection, embolism, differentiation syndrome, and others, in order of incidence. The cumulative incidence of ED due to infection (P = 0.0003) and embolism (P = 0.034) was significantly higher in the older group. For the older patients, independent risk factors for ED were WBC count > 5.9 × 109 /L, albumin < 35.85 g/L, creatinine > 72.9 µmol/L and fibrinogen < 1.137 g/L; independent risk factor for bleeding ED was WBC count > 6.2 × 109 /L; while independent risk factors for infection ED were albumin < 36.55 g/L and fibrinogen < 1.035 g/L. The baseline clinical characteristics, the clinical features and risk factors of ED in older APL patients were all quite different from those in younger patients, so research specifically targeting older patients with APL is very necessary.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142891540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Risk stratification based on changes in the standard maximal uptake value on PET/CT and the plasma Epstein‒Barr virus (EBV) DNA status after two cycles of chemotherapy for extranodal NK-/T-cell lymphoma. 结外NK / t细胞淋巴瘤化疗两周期后，基于PET/CT标准最大摄取值和血浆eb病毒（EBV） DNA状态变化的风险分层

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-27 DOI: 10.1007/s00277-024-06149-1

Quanguang Ren, Yue Cui, Zhao Wang, Xiaojie Fang, Meiting Chen, Zegeng Chen, Tongyu Lin, Yongsheng Jiang, He Huang

{"title":"Risk stratification based on changes in the standard maximal uptake value on PET/CT and the plasma Epstein‒Barr virus (EBV) DNA status after two cycles of chemotherapy for extranodal NK-/T-cell lymphoma.","authors":"Quanguang Ren, Yue Cui, Zhao Wang, Xiaojie Fang, Meiting Chen, Zegeng Chen, Tongyu Lin, Yongsheng Jiang, He Huang","doi":"10.1007/s00277-024-06149-1","DOIUrl":"https://doi.org/10.1007/s00277-024-06149-1","url":null,"abstract":"Although different types of prognostic indices have been applied in extranodal NK-/T-cell lymphoma (ENKTL), they are based mainly on clinical characteristics before treatment. Moreover, these methods lack early assessment and tumor metabolic parameters. It remains unclear whether changes in the plasma Epstein-Barr virus DNA (EBVDNA) status and SUVmax after two cycles of chemotherapy may predict disease prognosis. We retrospectively analyzed the clinical records of 119 patients with ENKTL. According to the multivariate analysis, limited stage (LS), interim EBVDNA (I-EBVDNA) negativity and a ≥ 50% decrease in the sum of the SUVmax for the target lesion (DSSTL) were significantly associated with complete remission after two cycles of chemotherapy (p = 0.005, p = 0.016 and 0.026, respectively). LS disease, I-EBVDNA negativity and ≥ 50% DSSTL were strongly associated with prolonged PFS (HR = 2.953, 95% CI 1.433-6.009, p = 0.003; HR = 2.479, 95% CI 1.239-4.958, p = 0.01; and HR = 2.048, 95% CI 1.037-4.405, p = 0.039, respectively). Based on these predictors of PFS, a preliminary scoring system was developed. Patients with scores of 1 and 2/3 had poorer survival outcomes than those with a score of 0 (HR = 2.030, 95% CI 0.816-5.048, p = 0.044, and HR = 2.377, 95% CI 1.663-3.396, p = 0.000, respectively). This scoring system also applied well to overall survival (OS) and appeared to be superior to the revised Ann Arbor staging system (p < 0.001, vs. p = 0.205). By assessing the early response to chemotherapy, interim changes in the SUVmax and I-EBVDNA could be used to predict disease prognosis and better stratify patients into subgroups with different prognoses of ENKTL. Further prospective studies are needed to verify these findings.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142891491","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Investigation of a hemophilia family with one female hemophilia A patient and 12 male hemophilia A patients. 1例血友病a女1例，a男12例的血友病家庭调查。

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-26 DOI: 10.1007/s00277-024-06158-0

Jie Wang, Qiang Li, Yan Cheng, Aihui Wang, Cuicui Qiao, Jingru Shao, Tiantian Wang, Hehe Wang, Xueqin Zhang, Man-Chiu Poon, Xinsheng Zhang, Yunhai Fang

{"title":"Investigation of a hemophilia family with one female hemophilia A patient and 12 male hemophilia A patients.","authors":"Jie Wang, Qiang Li, Yan Cheng, Aihui Wang, Cuicui Qiao, Jingru Shao, Tiantian Wang, Hehe Wang, Xueqin Zhang, Man-Chiu Poon, Xinsheng Zhang, Yunhai Fang","doi":"10.1007/s00277-024-06158-0","DOIUrl":"https://doi.org/10.1007/s00277-024-06158-0","url":null,"abstract":"Hemophilia A (HA) is an X-chromosome-linked recessive genetic disorder. Female carriers may have bleeding symptoms, but rarely have moderate or severe disease. We identified a female patient with moderate HA by pedigree tracking and genetic testing in a HA family involving consanguineous marriage. To investigate the clinical and laboratory data, as well as F8 genetic variant affecting members in her family. We constructed a detailed pedigree diagram and performed coagulation analyses, including factor VIII activity (FVIII:C), FVIII inhibitor, and von Willebrand factor antigen (VWF: Ag) on 20 family members. The genomic DNA of 11 members was screened for intron 1 and intron 22 inversions using long-distance real-time polymerase chain reaction (RT-PCR). Their F8 coding genes were sequenced with an automatic next-generation sequencing. Thirteen HA persons with hemophilia (12 males, one female) and 18 female carriers were identified in the family. VWF: Ag level was normal in all 13 persons with hemophilia and 7 carriers tested. The female HA patient had FVIII:C 1.9 IU/dL and was homozygous for F8:c.1918G > T:p.V640F. Genetic testing is conducive to the diagnosis of hemophilia carriers and persons with hemophilia. F8: c.1918G > T:p.V640F is the pathogenic HA variant in this family. In any hemophilia family, we need to pay more attention to female carriers and patients.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142891485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Molecular and clinical characterization of two independent Chinese families with protein C deficiency. 两个独立的中国蛋白C缺乏家族的分子和临床特征。

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-26 DOI: 10.1007/s00277-024-06156-2

Mengzhen Wen, Yifan Lu, Haixiao Xie, Langyi Qin, Longying Ye, Ke Zhang, Mingshan Wang, Lihong Yang

{"title":"Molecular and clinical characterization of two independent Chinese families with protein C deficiency.","authors":"Mengzhen Wen, Yifan Lu, Haixiao Xie, Langyi Qin, Longying Ye, Ke Zhang, Mingshan Wang, Lihong Yang","doi":"10.1007/s00277-024-06156-2","DOIUrl":"https://doi.org/10.1007/s00277-024-06156-2","url":null,"abstract":"This study aims to investigate the clinical characterization and molecular pathogenic basis of hereditary protein C (PC) deficiency in two independent Chinese families, and conduct in vitro expression studies on the newly discovered p.Trp444Arg mutation. The PC activity (PC: A) was tested using the chromogenic substrate, and PC antigen (PC: Ag) was detected via enzyme-linked immunosorbent assay (ELISA). To identify the mutation sites, nine exons of the PROC gene were amplified by PCR, and the products were directly sequenced. The conservation and pathogenicity of the mutations, as well as changes in the spatial structure of PC proteins before and after mutations, were analyzed using ClustalX-2.1-win, online bioinformatics software, and PyMOL. The function of the mutant proteins was detected using the thrombin generation assay (TGA). Recombinant PC was ectopically expressed in HEK293T cells, with mRNA levels quantified by RT-qPCR. The recombinant protein was further characterized using Western blotting, ELISA, and immunofluorescence microscopy. Proband A and B, aged 39 and 63 respectively, are both diagnosed with deep vein thrombosis (DVT) in both lower limbs and pulmonary embolism (PE). Two missense mutations, p.Arg440Cys and p.Trp444Arg, were identified in the probands. Bioinformatics and protein modeling analyses revealed that the two mutations probably affected the normal function of PC. The thrombin generation assay revealed impaired thrombin generation capacity in both probands, with proband B showing more severe impairment. In vitro expression experiments demonstrated that p.Trp444Arg do not significantly affect mRNA expression levels of PC protein compared to wild-type, but result in lower PC: Ag content and protein expression in the supernatant and higher levels in the lysate. These two mutations may be the causes of reduced PC in two independent Chinese families. Notably, this is the first reported instance of the p.Trp444Arg mutation.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142891488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Epstein-Barr virus-associated lymphoproliferative disease during remission after induction therapy with dasatinib in Philadelphia chromosome-positive acute lymphoblastic leukemia: a case report. 费城染色体阳性急性淋巴母细胞白血病患者在达沙替尼诱导治疗后缓解期间的爱泼斯坦-巴尔病毒相关淋巴细胞增生性疾病：1例报告

IF 3 3区医学

Annals of Hematology Pub Date : 2024-12-21 DOI: 10.1007/s00277-024-06153-5

Ayano Sugihara, Yasushi Kubota, Toshiaki Nagaie, Mai Fujita, Keitaro Ishii, Sho Okamoto, Hiroshi Ureshino, Hiroo Katsuya, Mai Takeuchi, Keita Kai, Toshihiko Ando, Kouichi Ohshima, Shinya Kimura

{"title":"Epstein-Barr virus-associated lymphoproliferative disease during remission after induction therapy with dasatinib in Philadelphia chromosome-positive acute lymphoblastic leukemia: a case report.","authors":"Ayano Sugihara, Yasushi Kubota, Toshiaki Nagaie, Mai Fujita, Keitaro Ishii, Sho Okamoto, Hiroshi Ureshino, Hiroo Katsuya, Mai Takeuchi, Keita Kai, Toshihiko Ando, Kouichi Ohshima, Shinya Kimura","doi":"10.1007/s00277-024-06153-5","DOIUrl":"https://doi.org/10.1007/s00277-024-06153-5","url":null,"abstract":"Dasatinib, a second-generation tyrosine kinase inhibitor, has been reported to have immunomodulatory effects. Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (EBV-LPD) occur in immunocompromised patients, such as those receiving methotrexate or other immunosuppressive drugs or after allogenic transplantation. EBV-LPD is also reported to be a rare side effect in patients receiving long-term dasatinib or imatinib. The present report describes a 60-year-old woman with Philadelphia chromosome-positive acute lymphoblastic leukemia who was treated with dasatinib and prednisolone for induction of remission. Fever, enlargement of the tonsils, multiple cervical lymphadenopathies and a splenic mass emerged after 1 month of treatment. Histopathological analysis of tonsil biopsy specimens showed diffuse proliferation of CD20-positive atypical cells with large, irregular nuclei. Some of these cells were positive for EBV-encoded small RNA, and her peripheral blood was positive for EBV-DNA (4.9 Log IU/mL), leading to a diagnosis of EBV-LPD. After discontinuation of dasatinib, her high fever and cervical lymphadenopathies disappeared without recurrence. The subsequently removed splenic mass was largely composed of non-neoplastic cytotoxic T cells resulting from a reaction to EBV-infected B cells. EBV-LPD should be included in the differential diagnosis of patients who develop lymphadenopathy during dasatinib treatment, regardless of its duration.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142871076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0