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[Clinical features and prognosis of hepatosplenic candidiasis in patients with hematopathy]. [血液病患者肝脾念珠菌病的临床特征和预后]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20231230-00347
D P Zhu, R Ma, Y He, X Y Luo, W Han, C Li, J R Zhou, Y Liao, B R Tang, Q L T Longka, X J Huang, Y Q Sun

Hepatosplenic candidiasis (HSC) is a rare type of candidiasis that can occur in patients with hematologic malignancies, hematopoietic stem cell transplantation. At present, there is still a lack of studies on HSC in patients with hematologic disorders. Based on The Chinese Guidelines for the Diagnosis and Treatment of Invasive Fungal Disease in Patients with Hematological Disorders and Cancers (the 6th revision), We retrospectively analyzed the clinical characteristics and prognosis of patients with HSC treated in Peking University Institute of Hematology from 2008 to 2022. Finally, eighteen patients were included, with 1 (5.6%) proven, 2 (11.1%) probable, and 15 (83.3%) possible HSC. Among them, 3 (16.7%) patients occurred after haploid hematopoietic stem cell transplantation and 15 (83.3%) patients occurred after chemotherapy. 6 (33.3%) patients had positive blood cultures, including 4 cases of Candida tropicalis and 2 cases of Candida albicans. At 4 weeks of antifungal therapy, 10 (58.8%) patients achieved partial response (PR), At 8 weeks, 1 (6.3%) patients achieved complete response and 10 (62.5%) patients achieved PR. At 6 months after diagnosis, 3 (16.7%) patients died of hematopoietic recurrence, and none of them died of HSC. As a rare fungal infection disease, HSC has a low positive rate of microbiological and histological examinations, a persistent treat cycle, and has difficulty in remission, reminding us of the need for vigilance in patients with hematopoietic disorders and persistent fever.

肝脾念珠菌病(HSC)是一种罕见的念珠菌病,可发生在血液系统恶性肿瘤和造血干细胞移植患者身上。目前,有关血液系统疾病患者 HSC 的研究还很缺乏。根据《中国血液病和肿瘤患者侵袭性真菌病诊治指南》(第 6 版),我们回顾性分析了 2008 年至 2022 年在北京大学血液病研究所接受治疗的 HSC 患者的临床特征和预后。最后,我们纳入了 18 例患者,其中 1 例(5.6%)已证实为造血干细胞,2 例(11.1%)可能为造血干细胞,15 例(83.3%)可能为造血干细胞。其中,3 例(16.7%)患者发生在单倍体造血干细胞移植后,15 例(83.3%)患者发生在化疗后。6例(33.3%)患者血液培养呈阳性,其中4例为热带念珠菌,2例为白色念珠菌。抗真菌治疗 4 周后,10 例(58.8%)患者获得部分应答,8 周后,1 例(6.3%)患者获得完全应答,10 例(62.5%)患者获得应答。确诊后 6 个月,3 名患者(16.7%)死于造血功能复发,其中没有人死于造血干细胞。作为一种罕见的真菌感染性疾病,HSC 的微生物学和组织学检查阳性率低,治疗周期长,缓解困难,提醒我们需要警惕造血障碍和持续发热患者。
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引用次数: 0
[Clinical characteristics and prognosis of patients with myelodysplastic syndrome with a bone marrow nucleated erythroid cell proportion of greater than or equal to 50]. [骨髓有核红细胞比例大于或等于 50 的骨髓增生异常综合征患者的临床特征和预后]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20240517-00183
Y P Zeng, B Li, T J Qin, Z F Xu, S J Qu, L J Pan, Q Y Gao, M Jiao, J Y Wu, H J Wang, C W Li, Y J Ja, Q Sun, Z J Xiao
<p><p><b>Objective:</b> To analyze the clinical characteristics and prognosis of patients with myelodysplastic syndrome (MDS) with a bone marrow nucleated erythroid cell proportion of greater than or equal to 50% (MDS-E) . <b>Methods:</b> The clinical characteristics and prognostic factors of patients with MDS-E were retrospectively analyzed by collecting the case data of 1 436 newly treated patients with MDS diagnosed in the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences from May 2014 to June 2023. <b>Results:</b> A total of 1 436 newly diagnosed patients with complete data were included in the study, of which 337 (23.5%) patients with MDS-E had a younger age of onset and lower neutrophil and platelet counts compared with those in patients with an erythroid cell proportion of less than 50% (MDS-NE) (all <i>P</i><0.05). The proportion of MDS cases with ring sideroblasts (MDS-RS) was higher in the MDS-E group than in the MDS-NE group, and multi-hit TP53 mutations were more enriched in the MDS-E group than in the MDS-NE group (all <i>P</i><0.05). Among patients with MDS-RS, the frequency of complex karyotypes and the TP53 mutation rate were significantly lower in the MDS-E group than in the MDS-NE group (0 <i>vs</i> 11.9%, <i>P</i>=0.048 and 2.4% <i>vs</i> 15.1%, <i>P</i>=0.053, respectively). Among patients with TP53 mutations, the frequencies of complex karyotypes and multi-hit TP53 mutations were significantly higher in the MDS-E group than in the MDS-NE group (87.5% <i>vs</i> 64.6%, <i>P</i>=0.003 and 84.0% <i>vs</i> 54.2%, <i>P</i><0.001, respectively). Survival analysis of patients with MDS-RS found that the overall survival (OS) in the MDS-E group was better than that in the MDS-NE group [not reached <i>vs</i> 63 (95% <i>CI</i> 53.3-72.7) months, <i>P</i>=0.029]. Among patients with TP53 mutations and excess blasts, the OS in the MDS-E group was worse than that in the MDS-NE group [6 (95% <i>CI</i> 2.2-9.8) months <i>vs</i> 12 (95% <i>CI</i> 8.9-15.1) months, <i>P</i>=0.022]. Multivariate analysis showed that age of ≥65 years (<i>HR</i>=2.47, 95% <i>CI</i> 1.43-4.26, <i>P</i>=0.001), mean corpuscular volume (MCV) of ≤100 fl (<i>HR</i>=2.62, 95% <i>CI</i> 1.54-4.47, <i>P</i><0.001), and TP53 mutation (<i>HR</i>=2.31, 95% <i>CI</i> 1.29-4.12, <i>P</i>=0.005) were poor prognostic factors independent of the Revised International Prognostic Scoring System (IPSS-R) prognosis stratification in patients with MDS-E. <b>Conclusion:</b> Among patients with MDS-RS, MDS-E was strongly associated with a lower proportion of complex karyotypes and TP53 mutations, and the OS in the MDS-E group was longer than that in the MDS-NE group. Among patients with TP53 mutations, MDS-E was strongly associated with complex karyotypes and multi-hit TP53 mutations, and among TP53-mutated patients with excess blasts, the OS in the MDS-E group was shorter than that in the MDS-NE group. Age of ≥65 years, MCV of ≤100 fl, and TP53 mut
目的分析骨髓有核红细胞比例大于或等于 50%(MDS-E)的骨髓增生异常综合征(MDS)患者的临床特征和预后。方法:通过收集2014年5月至2023年6月中国医学科学院血液病研究所医院新收治的1 436例MDS患者的病例资料,对MDS-E患者的临床特征和预后因素进行回顾性分析。结果研究共纳入了1 436例资料完整的新诊断患者,其中337例(23.5%)MDS-E患者与红细胞比例低于50%(MDS-NE)的患者相比,发病年龄更小、中性粒细胞和血小板计数更低(全部PPvs 11.9%,P=0.048;2.4% vs 15.1%,P=0.053)。在TP53突变患者中,MDS-E组复杂核型和多基因TP53突变的频率明显高于MDS-NE组(87.5% vs 64.6%,P=0.003和84.0% vs 54.2%,Pvs 63 (95% CI 53.3-72.7)个月,P=0.029]。在TP53突变和胚泡过多的患者中,MDS-E组的OS比MDS-NE组差[6(95% CI 2.2-9.8)个月 vs 12(95% CI 8.9-15.1)个月,P=0.022]。多变量分析显示,年龄≥65岁(HR=2.47,95% CI 1.43-4.26,P=0.001)、平均血球容积(MCV)≤100 fl(HR=2.62,95% CI 1.54-4.47,PHR=2.31,95% CI 1.29-4.12,P=0.005)是MDS-E患者的不良预后因素,与修订版国际预后评分系统(IPSS-R)预后分层无关。结论在MDS-RS患者中,MDS-E与较低比例的复杂核型和TP53突变密切相关,MDS-E组的OS长于MDS-NE组。在TP53突变的患者中,MDS-E与复杂核型和TP53多基因突变密切相关,在TP53突变且胚泡过多的患者中,MDS-E组的OS短于MDS-NE组。年龄≥65岁、MCV≤100 fl和TP53突变是影响MDS-E患者OS的独立不良预后因素。
{"title":"[Clinical characteristics and prognosis of patients with myelodysplastic syndrome with a bone marrow nucleated erythroid cell proportion of greater than or equal to 50].","authors":"Y P Zeng, B Li, T J Qin, Z F Xu, S J Qu, L J Pan, Q Y Gao, M Jiao, J Y Wu, H J Wang, C W Li, Y J Ja, Q Sun, Z J Xiao","doi":"10.3760/cma.j.cn121090-20240517-00183","DOIUrl":"10.3760/cma.j.cn121090-20240517-00183","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To analyze the clinical characteristics and prognosis of patients with myelodysplastic syndrome (MDS) with a bone marrow nucleated erythroid cell proportion of greater than or equal to 50% (MDS-E) . &lt;b&gt;Methods:&lt;/b&gt; The clinical characteristics and prognostic factors of patients with MDS-E were retrospectively analyzed by collecting the case data of 1 436 newly treated patients with MDS diagnosed in the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences from May 2014 to June 2023. &lt;b&gt;Results:&lt;/b&gt; A total of 1 436 newly diagnosed patients with complete data were included in the study, of which 337 (23.5%) patients with MDS-E had a younger age of onset and lower neutrophil and platelet counts compared with those in patients with an erythroid cell proportion of less than 50% (MDS-NE) (all &lt;i&gt;P&lt;/i&gt;&lt;0.05). The proportion of MDS cases with ring sideroblasts (MDS-RS) was higher in the MDS-E group than in the MDS-NE group, and multi-hit TP53 mutations were more enriched in the MDS-E group than in the MDS-NE group (all &lt;i&gt;P&lt;/i&gt;&lt;0.05). Among patients with MDS-RS, the frequency of complex karyotypes and the TP53 mutation rate were significantly lower in the MDS-E group than in the MDS-NE group (0 &lt;i&gt;vs&lt;/i&gt; 11.9%, &lt;i&gt;P&lt;/i&gt;=0.048 and 2.4% &lt;i&gt;vs&lt;/i&gt; 15.1%, &lt;i&gt;P&lt;/i&gt;=0.053, respectively). Among patients with TP53 mutations, the frequencies of complex karyotypes and multi-hit TP53 mutations were significantly higher in the MDS-E group than in the MDS-NE group (87.5% &lt;i&gt;vs&lt;/i&gt; 64.6%, &lt;i&gt;P&lt;/i&gt;=0.003 and 84.0% &lt;i&gt;vs&lt;/i&gt; 54.2%, &lt;i&gt;P&lt;/i&gt;&lt;0.001, respectively). Survival analysis of patients with MDS-RS found that the overall survival (OS) in the MDS-E group was better than that in the MDS-NE group [not reached &lt;i&gt;vs&lt;/i&gt; 63 (95% &lt;i&gt;CI&lt;/i&gt; 53.3-72.7) months, &lt;i&gt;P&lt;/i&gt;=0.029]. Among patients with TP53 mutations and excess blasts, the OS in the MDS-E group was worse than that in the MDS-NE group [6 (95% &lt;i&gt;CI&lt;/i&gt; 2.2-9.8) months &lt;i&gt;vs&lt;/i&gt; 12 (95% &lt;i&gt;CI&lt;/i&gt; 8.9-15.1) months, &lt;i&gt;P&lt;/i&gt;=0.022]. Multivariate analysis showed that age of ≥65 years (&lt;i&gt;HR&lt;/i&gt;=2.47, 95% &lt;i&gt;CI&lt;/i&gt; 1.43-4.26, &lt;i&gt;P&lt;/i&gt;=0.001), mean corpuscular volume (MCV) of ≤100 fl (&lt;i&gt;HR&lt;/i&gt;=2.62, 95% &lt;i&gt;CI&lt;/i&gt; 1.54-4.47, &lt;i&gt;P&lt;/i&gt;&lt;0.001), and TP53 mutation (&lt;i&gt;HR&lt;/i&gt;=2.31, 95% &lt;i&gt;CI&lt;/i&gt; 1.29-4.12, &lt;i&gt;P&lt;/i&gt;=0.005) were poor prognostic factors independent of the Revised International Prognostic Scoring System (IPSS-R) prognosis stratification in patients with MDS-E. &lt;b&gt;Conclusion:&lt;/b&gt; Among patients with MDS-RS, MDS-E was strongly associated with a lower proportion of complex karyotypes and TP53 mutations, and the OS in the MDS-E group was longer than that in the MDS-NE group. Among patients with TP53 mutations, MDS-E was strongly associated with complex karyotypes and multi-hit TP53 mutations, and among TP53-mutated patients with excess blasts, the OS in the MDS-E group was shorter than that in the MDS-NE group. Age of ≥65 years, MCV of ≤100 fl, and TP53 mut","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 7","pages":"651-659"},"PeriodicalIF":0.0,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11388124/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142133977","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
[Daratumumab for the treatment of relapsed T-cell acute lymphoblastic leukemia after allogeneic hematopoietic stem cell transplantation: a case report]. [达拉单抗治疗异基因造血干细胞移植后复发的T细胞急性淋巴细胞白血病:病例报告]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20231130-00285
X Wang, M M Hu, J Y Xiao, Z H Lin, T T Shi, X F Li, H Y Qiu
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引用次数: 0
[Chinese consensus on autologous stem cell transplantation for adult acute leukemia (2024)]. [中国成人急性白血病自体干细胞移植共识(2024 年)]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20240611-00218

Autologous stem cell transplantation (ASCT) emerges as a therapeutic strategy following remission in adult acute leukemia (AL). It offers advantages over allogeneic hematopoietic stem cell transplantation (allo-HSCT), including independence from donor availability, absence of graft-versus-host disease (GVHD), and a reduced risk of transplant-related mortality. Furthermore, when juxtaposed with the extended regimens of consolidation chemotherapy, ASCT stands out by markedly abbreviating treatment duration, alleviating the economic strain on patients, and enhancing their overall quality of life. Despite these benefits, the adoption of ASCT among adult AL patients in China remains disproportionately low. To enhance clinical physicians' understanding of the role and position of ASCT in AL management and to improve the clinical efficacy of ASCT, it is urgent to establish a consensus among experts on ASCT for adult acute leukemia in our nation.

自体干细胞移植(ASCT)是成人急性白血病(AL)缓解后的一种治疗策略。与同种异体造血干细胞移植(allo-HSCT)相比,自体干细胞移植更具优势,包括不受供体限制、无移植物抗宿主疾病(GVHD)、降低移植相关死亡风险。此外,与延长疗程的巩固性化疗相比,体外受体移植明显缩短了治疗时间,减轻了患者的经济压力,提高了患者的整体生活质量。尽管有这些优势,但在中国,成人 AL 患者采用 ASCT 的比例仍然过低。为了加强临床医生对ASCT在AL治疗中的作用和地位的认识,提高ASCT的临床疗效,亟需在我国成人急性白血病ASCT治疗方面达成专家共识。
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引用次数: 0
[Expression and clinical significance of CCL17, CCL22, and CCR4 in newly diagnosed multiple myeloma]. [CCL17、CCL22和CCR4在新诊断多发性骨髓瘤中的表达及临床意义]
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20231230-00349
Z F Xiao, S S Zou, C F Yi, Y Zhao, L S Wu, Y H Feng

Objective: To study the expressions of C-C class chemokine 17 (CCL17), C-C class chemokine 22 (CCL22), and C-C chemokine receptor 4 (CCR4) in newly diagnosed multiple myeloma (NDMM) for analyzing their correlations with clinical features and to preliminarily explore their roles in the development of NDMM. Methods: The study included 40 patients with NDMM and 20 healthy volunteers from the Department of Hematology of the Affiliated Hospital of Zunyi Medical University from July 2020 to December 2022. Peripheral blood, bone marrow, and bone marrow biopsy tissue samples were collected from the two groups. The expression levels of CCL17, CCL22, and CCR4 in patients with NDMM were analyzed using real-time quantitative reverse transcriptase polymerase chain reaction (RQ-PCR), enzyme-linked immunosorbent assay (ELISA), and immunohistochemistry. The mRNA expression levels of CCL17, CCL22, and CCR4 in the bone marrow mononuclear cell (BMMNC) of patients with NDMM were analyzed to assess their correlations with clinical indicators. Results: The mRNA expression levels of CCL17, CCL22, and CCR4 in BMMNC were higher in patients with NDMM than in controls (all P<0.05). The protein expression levels of CCL17 and CCL22 in peripheral blood supernatants and bone marrow supernatants were higher in patients with NDMM than in controls (all P<0.05). The expression levels of CCL17, CCL22, and CCR4 in bone marrow biopsy tissues were higher in patients with NDMM than in controls (all P<0.05). The mRNA expression level of CCL17 was increased in NDMM patients with combined anemia, bone damage, renal damage, and M protein level ≥30 g/L (all P<0.05). The mRNA expression level of CCL22 was increased in NDMM patients with combined anemia, bone damage, and renal damage (all P<0.05). The mRNA expression level of CCR4 was increased in NDMM patients with combined anemia and renal damage (all P<0.05) . Conclusion: CCL17, CCL22, and CCR4 were highly expressed in clinical samples from patients with NDMM compared to those from controls, and they may be involved in the occurrence and development of NDMM.

研究目的研究新诊断多发性骨髓瘤(NDMM)中C-C类趋化因子17(CCL17)、C-C类趋化因子22(CCL22)和C-C趋化因子受体4(CCR4)的表达,分析它们与临床特征的相关性,并初步探讨它们在NDMM发病中的作用。研究方法研究纳入遵义医学院附属医院血液科2020年7月至2022年12月的40名NDMM患者和20名健康志愿者。收集两组患者的外周血、骨髓和骨髓活检组织样本。采用实时定量逆转录酶聚合酶链反应(RQ-PCR)、酶联免疫吸附试验(ELISA)和免疫组织化学方法分析CCL17、CCL22和CCR4在NDMM患者中的表达水平。分析NDMM患者骨髓单核细胞(BMMNC)中CCL17、CCL22和CCR4的mRNA表达水平,评估其与临床指标的相关性。结果显示NDMM患者骨髓单核细胞中CCL17、CCL22和CCR4的mRNA表达水平高于对照组(均为PPPPPPC结论:NDMM患者骨髓单核细胞中CCL17、CCL22和CCR4的mRNA表达水平高于对照组:与对照组相比,CCL17、CCL22和CCR4在NDMM患者的临床样本中高表达,它们可能与NDMM的发生和发展有关。
{"title":"[Expression and clinical significance of CCL17, CCL22, and CCR4 in newly diagnosed multiple myeloma].","authors":"Z F Xiao, S S Zou, C F Yi, Y Zhao, L S Wu, Y H Feng","doi":"10.3760/cma.j.cn121090-20231230-00349","DOIUrl":"10.3760/cma.j.cn121090-20231230-00349","url":null,"abstract":"<p><p><b>Objective:</b> To study the expressions of C-C class chemokine 17 (CCL17), C-C class chemokine 22 (CCL22), and C-C chemokine receptor 4 (CCR4) in newly diagnosed multiple myeloma (NDMM) for analyzing their correlations with clinical features and to preliminarily explore their roles in the development of NDMM. <b>Methods:</b> The study included 40 patients with NDMM and 20 healthy volunteers from the Department of Hematology of the Affiliated Hospital of Zunyi Medical University from July 2020 to December 2022. Peripheral blood, bone marrow, and bone marrow biopsy tissue samples were collected from the two groups. The expression levels of CCL17, CCL22, and CCR4 in patients with NDMM were analyzed using real-time quantitative reverse transcriptase polymerase chain reaction (RQ-PCR), enzyme-linked immunosorbent assay (ELISA), and immunohistochemistry. The mRNA expression levels of CCL17, CCL22, and CCR4 in the bone marrow mononuclear cell (BMMNC) of patients with NDMM were analyzed to assess their correlations with clinical indicators. <b>Results:</b> The mRNA expression levels of CCL17, CCL22, and CCR4 in BMMNC were higher in patients with NDMM than in controls (all <i>P</i><0.05). The protein expression levels of CCL17 and CCL22 in peripheral blood supernatants and bone marrow supernatants were higher in patients with NDMM than in controls (all <i>P</i><0.05). The expression levels of CCL17, CCL22, and CCR4 in bone marrow biopsy tissues were higher in patients with NDMM than in controls (all <i>P</i><0.05). The mRNA expression level of CCL17 was increased in NDMM patients with combined anemia, bone damage, renal damage, and M protein level ≥30 g/L (all <i>P</i><0.05). The mRNA expression level of CCL22 was increased in NDMM patients with combined anemia, bone damage, and renal damage (all <i>P</i><0.05). The mRNA expression level of CCR4 was increased in NDMM patients with combined anemia and renal damage (all <i>P</i><0.05) . <b>Conclusion:</b> CCL17, CCL22, and CCR4 were highly expressed in clinical samples from patients with NDMM compared to those from controls, and they may be involved in the occurrence and development of NDMM.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"45 7","pages":"672-677"},"PeriodicalIF":0.0,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11388129/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142133990","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 progress in classification and prognosis evaluation of BCR::ABL1 positive acute lymphoblastic leukemia]. [BCR::ABL1阳性急性淋巴细胞白血病的分类和预后评估进展]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20240315-00096
Y C Yan, C Wang, J Q Mi, J Wang

The application of tyrosine kinase inhibitors and targeted immunotherapy has revolutionized the therapeutic strategies and clinical outcome for BCR::ABL1-positive B-cell acute lymphoblastic leukemia (BCR::ABL1(+) B-ALL). The classification was updated successively by the World Health Organization and the International Consensus Classification in 2022. The risk stratification of this entity, for the first time, was modified by the National Comprehensive Cancer Network in 2023, both minimal residual disease assessment and IKZF1(plus) genotyping recognized as critical prognostic factors. These important updates would have significant implications for clinical management. Therefore, this review focused on the latest advances in the classification and prognostic evaluation of BCR::ABL1(+) B-ALL.

酪氨酸激酶抑制剂和靶向免疫疗法的应用彻底改变了BCR::ABL1阳性B细胞急性淋巴细胞白血病(BCR::ABL1(+) B-ALL)的治疗策略和临床疗效。世界卫生组织和国际共识分类法于 2022 年先后对该分类法进行了更新。2023 年,美国国家综合癌症网络(National Comprehensive Cancer Network)首次对该实体的风险分层进行了修改,最小残留病评估和 IKZF1(plus)基因分型被认为是关键的预后因素。这些重要的更新将对临床管理产生重大影响。因此,本综述重点关注 BCR::ABL1(+) B-ALL 分类和预后评估的最新进展。
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引用次数: 0
[Chinese consensus for the bispecific T cell engager in the treatment of acute lymphoblastic leukemia (2024)]. [治疗急性淋巴细胞白血病的双特异性 T 细胞诱导剂的中国共识(2024 年)]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20240528-00194

Acute lymphoblastic leukemia (ALL) is one of the most common acute leukemias, with rapid onset and progression. The standardized application of chemotherapy and transplantation have improved the prognosis of patients, while the unmet therapeutic needs still exist. Recently novel immunotherapies including Bispecific T cell Engager develop rapidly, offering more options for ALL treatment and also demanding higher requirements for clinical diagnosis and treatment management. Based on the evidence of domestic and international medical evidence and clinical experience, the expert panel updated Chinese consensus for the Bispeific T cell Engager in the treatment of B-cell acute lymphoblastic leukemia (2022) and formulated this edition of the Chinese expert consensus.

急性淋巴细胞白血病(ALL)是最常见的急性白血病之一,起病急、进展快。标准化化疗和移植的应用改善了患者的预后,但仍有治疗需求未得到满足。近来,包括双特异性T细胞Engager在内的新型免疫疗法发展迅速,为ALL的治疗提供了更多选择,同时也对临床诊断和治疗管理提出了更高的要求。基于国内外医学证据和临床经验,专家组更新了双特异性T细胞诱导治疗B细胞急性淋巴细胞白血病(2022年)的中国共识,并制定了本版中国专家共识。
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引用次数: 0
[Allogeneic hematopoietic stem cell transplantation for Shwachman-Diamond syndrome: a report of three cases and literature review]. [异基因造血干细胞移植治疗舒瓦赫曼-钻石综合征:三例病例报告及文献综述]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20240107-00009
A H Feng, J M Shi, H R Fu, J Yu, W Y Zheng, Y Y Zhu, H Huang, Y M Zhao

This study reports on three patients with Shwachman-Diamond syndrome (SDS) who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) at the First Affiliated Hospital of Zhejiang University School of Medicine. Based on relevant literature, the clinical manifestations and genetic mutation characteristics of SDS were summarized, and the efficacy and timing of allo HSCT for such patients were explored. Three SDS patients were all male, with transplant ages of 32, 33, and 32 years old, respectively. All three patients were diagnosed in childhood. Case 1 presented with anemia as the initial clinical manifestation, which gradually progressed to a decrease in whole blood cells; Case 2 and 3 both present with a decrease in whole blood cells as the initial clinical manifestation. Case 1 and 3 have intellectual disabilities, while case 3 presents with pancreatic steatosis and chronic pancreatitis. All three patients have short stature. Three patients all detected heterozygous mutations in the SBDS: c.258+2T>C splice site. The family members of the three patients have no clinical manifestations of SDS. All three patients were treated with a reduced dose pre-treatment regimen (Fludarabine+Busulfan+Me-CCNU+Rabbit Anti-human Thymocyte Globulin). Case 1 and case 2 underwent haploid hematopoietic stem cell transplantation, while case 3 underwent unrelated donor hematopoietic stem cell transplantation. Case 1 was diagnosed with myelodysplastic syndrome transforming into acute myeloid leukemia before transplantation, but experienced early recurrence and death after transplantation; Case 2 is secondary implantation failure, dependent on platelet transfusion; Case 3 was removed from medication maintenance treatment after transplantation, and blood routine monitoring was normal.

本研究报告了在浙江大学医学院附属第一医院接受异基因造血干细胞移植(allo-HSCT)的三名舒瓦赫曼-钻石综合征(SDS)患者。根据相关文献,总结了SDS的临床表现和基因突变特点,并探讨了异基因造血干细胞移植对此类患者的疗效和时机。三名SDS患者均为男性,移植年龄分别为32岁、33岁和32岁。三名患者均在儿童时期确诊。病例 1 最初的临床表现为贫血,后来逐渐发展为全血细胞减少;病例 2 和病例 3 最初的临床表现均为全血细胞减少。病例 1 和 3 有智力障碍,而病例 3 则伴有胰腺脂肪变性和慢性胰腺炎。三名患者均身材矮小。三名患者均检测到 SBDS:c.258+2T>C 剪接位点的杂合突变。三名患者的家庭成员均无 SDS 临床表现。三名患者均接受了减量预处理方案(氟达拉滨+布舒凡+Me-CNU+兔抗人胸腺细胞球蛋白)治疗。病例 1 和病例 2 接受了单倍体造血干细胞移植,而病例 3 则接受了非亲缘供体造血干细胞移植。病例1在移植前被诊断为骨髓增生异常综合征转变为急性髓性白血病,但移植后早期复发并死亡;病例2为继发性移植失败,依赖血小板输注;病例3在移植后停药维持治疗,血常规监测正常。
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引用次数: 0
[The impact of immune cells selection on the therapeutic efficacy of CAR-T cell therapy]. [免疫细胞的选择对 CAR-T 细胞疗法疗效的影响]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20240321-00104
W X Qi, W L Zhang, H M Jing

Here we summarized novel Chimeric antigen receptor T-cell immunotherapy (CAR-T) based on the immune material aspect. Young healthy donor T cells, stem cell-like memory T cells, human induced pluripotent stem cells and umbilical cord blood T cells are all potential candidates to enhance CAR-T cell therapy depending on their anti-tumor efficacy. Besides, due to less restricted major histocompatibility complex (MHC) mismatch effect, viral specific T cells, γδT cells, invariant natural killer T cells and macrophages also become idealized T cell sources in terms of Universal CAR-T (UCAR-T) cell therapeutics. In addition, studies demonstrated that more balanced CD4(+)/CD8(+) T cell ratio and eliminating monocytes during leukapheresis have a positive influence on CAR-T cell functioning, whereas T cells with higher exhaustion markers expression hampers anti-tumor ability of CAR-T cells after infusion. To avoid application of such T cells or mitigate the impact using immune checkpoint inhibitors is of great importance.

在此,我们总结了基于免疫物质方面的新型嵌合抗原受体T细胞免疫疗法(CAR-T)。年轻的健康供体T细胞、干细胞样记忆T细胞、人类诱导多能干细胞和脐带血T细胞都是增强CAR-T细胞疗法的潜在候选者,这取决于它们的抗肿瘤功效。此外,由于主要组织相容性复合体(MHC)错配效应限制较少,病毒特异性 T 细胞、γδT 细胞、不变性自然杀伤 T 细胞和巨噬细胞也成为通用 CAR-T (UCAR-T)细胞疗法的理想 T 细胞来源。此外,研究表明,CD4(+)/CD8(+) T 细胞比例更均衡以及在白细胞清除过程中消除单核细胞对 CAR-T 细胞的功能有积极影响,而表达较高衰竭标志物的 T 细胞则会阻碍 CAR-T 细胞输注后的抗肿瘤能力。避免使用这类T细胞或使用免疫检查点抑制剂减轻其影响非常重要。
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引用次数: 0
[Rituximab based treatment in pediatric Epsstain Bar Virus associated lymphocyte proliferative diseases after aplastic anemia with haplo-identical transplantation:a prospective single centre study]. [基于利妥昔单抗的再生障碍性贫血伴单倍体同种异体移植后小儿伊普斯坦巴病毒相关淋巴细胞增生性疾病治疗:一项前瞻性单中心研究]。
Q3 Medicine Pub Date : 2024-07-14 DOI: 10.3760/cma.j.cn121090-20231229-00345
F Zhang, G H Hu, P Suo, Z L Xu, L Bai, H F Wang, S Y M Huang, L P Xu, Y J Chang, X H Zhang, X J Huang, Y F Cheng

Epstein-Barr virus (EBV) associated post-transplant lymphoproliferative disorders (PTLD) are one of the most severe complications after hematopoietic stem cell transplantation (HSCT). This study includes 31 cases of aplastic anemia (AA) patients who developed PTLD after haploidentical transplantation, summarizing their clinical characteristics and categorizing them into either rituximab monotherapy group or combination therapy group based on whether their condition improved by 1 log after a single dose of rituximab. The incidence of PTLD after HSCT in children with AA was 10.16%, and the incidence of PTLD in patients with age >10 years was significantly increased (χ(2)=11.336, P=0.010). Of the 31 patients, 27 were clinically diagnosed and 4 were pathologically confirmed. Finally, 15 patients were classified into the rituximab treatment group and 15 patients into the combination treatment groups. Finally three patients died, and the 2-year overall survival rate was (89.7±5.6) %. Standard pre-treatment protocols and EBV reactivation are risk factors affecting the prognosis of PTLD. There was no statistically significant difference in the impact of the two treatment schemes on prognosis.

与爱泼斯坦-巴氏病毒(EBV)相关的移植后淋巴组织增生性疾病(PTLD)是造血干细胞移植(HSCT)后最严重的并发症之一。本研究纳入了31例在单倍体移植后出现PTLD的再生障碍性贫血(AA)患者,总结了他们的临床特征,并根据单剂量利妥昔单抗治疗后病情是否改善1个对数值,将他们分为利妥昔单抗单药治疗组和联合治疗组。AA患儿造血干细胞移植后PTLD的发生率为10.16%,年龄大于10岁的患者PTLD发生率显著增加(χ(2)=11.336,P=0.010)。在 31 例患者中,27 例经临床诊断,4 例经病理确诊。最后,15 名患者被分为利妥昔单抗治疗组,15 名患者被分为联合治疗组。最后有 3 名患者死亡,2 年总生存率为(89.7±5.6)%。标准治疗前方案和EB病毒再激活是影响PTLD预后的危险因素。两种治疗方案对预后的影响在统计学上没有明显差异。
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Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
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