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Real-world data analysis of survival outcomes of patients with primary mediastinal large B-cell lymphoma treated with immunochemotherapy: the role of consolidative radiation therapy. 免疫化疗治疗原发性纵隔大b细胞淋巴瘤患者生存结果的真实世界数据分析:巩固放疗的作用
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-04-22 DOI: 10.1007/s44313-025-00076-4
Yong-Pyo Lee, Junhun Cho, Young Hyeh Ko, Dongryul Oh, Seok Jin Kim, Won Seog Kim, Sang Eun Yoon

Purpose: Primary mediastinal large B-cell lymphoma (PMBCL) is a rare subtype of diffuse large B-cell lymphoma. Radiation therapy (RT) has served as the primary treatment option for PMBCL; however, its role has been questioned with the advent of intensified immunochemotherapy. This study aimed to investigate the role of consolidative RT in the primary treatment of PMBCL.

Methods: This single-center retrospective study analyzed the survival outcomes of 65 patients newly diagnosed with PMBCL. The patients were divided into three treatment groups: (1) EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab), (2) R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), and (3) R-CHOP with consolidative RT.

Results: The objective response and complete remission rates were 86.2% and 63.1%, respectively, with 3-year progression-free survival (PFS) and overall survival (OS) rates of 72% and 81%, respectively. All patients in the R-CHOP + RT group achieved an objective response with better PFS) than those who did not receive consolidative RT (p = 0.028), although there was no significant difference in OS (p = 0.102). Consolidative RT benefited patients with an initially bulky disease or insufficient end-of-treatment response. The predictive value of 18F-fluorodeoxyglucose positron-emission tomography-computed tomography (PET-CT) in assessing the treatment response in PMBCL was revalidated, showing that patients who achieved negative end-of-treatment PET-CT had significantly better survival outcomes than others.

Conclusions: R-CHOP is a useful alternative regimen when intensified chemotherapy is not feasible. Consolidative RT should be considered in cases with an initially bulky disease and insufficient end-of-treatment response.

目的:原发性纵隔大b细胞淋巴瘤(PMBCL)是一种罕见的弥漫性大b细胞淋巴瘤亚型。放射治疗(RT)已成为PMBCL的主要治疗选择;然而,随着强化免疫化疗的出现,其作用受到质疑。本研究旨在探讨巩固性放疗在PMBCL初级治疗中的作用。方法:本单中心回顾性研究分析了65例新诊断的PMBCL患者的生存结局。患者分为3个治疗组:(1)EPOCH-R(依托泊苷、强的松、长春新碱、环磷酰胺、阿霉素、利妥昔单抗),(2)R-CHOP(利妥昔单抗、环磷酰胺、阿霉素、长春新碱、强的松),(3)R-CHOP合并巩固放疗。结果:客观缓解率和完全缓解率分别为86.2%和63.1%,3年无进展生存期(PFS)和总生存期(OS)分别为72%和81%。R-CHOP + RT组的所有患者均获得客观缓解,PFS优于未接受巩固RT的患者(p = 0.028),尽管OS无显著差异(p = 0.102)。巩固性放疗使最初病情严重或治疗结束反应不足的患者受益。18f -氟脱氧葡萄糖正电子发射断层扫描-计算机断层扫描(PET-CT)在评估PMBCL治疗反应中的预测价值被再次验证,显示治疗结束时PET-CT阴性的患者的生存结果明显优于其他患者。结论:当强化化疗不可行时,R-CHOP是一种有效的替代方案。在最初疾病大而治疗结束反应不足的情况下,应考虑巩固放疗。
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引用次数: 0
Real-world treatment patterns, outcomes, and economic costs by lines of therapy in patients with newly diagnosed multiple myeloma: a nationwide population-based cohort study in South Korea. 新诊断多发性骨髓瘤患者的现实世界治疗模式、结果和经济成本:韩国一项基于全国人群的队列研究。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-04-15 DOI: 10.1007/s44313-025-00069-3
Sung-Soo Park, YoungJu Park, Soomin Yoon, Doik Lee, Jihyeon Jeong, Kihyun Kim

Purpose: Given the notable increase in the incidence of multiple myeloma (MM) in Asia and advent of innovative treatments, this study aims to provide a comprehensive understanding of the treatment patterns, outcomes, and economic burden of MM across the lines of therapy (LOTs) in South Korea.

Methods: This retrospective cohort study was conducted using data from the National Health Insurance claims data provided by the Health Insurance Review and Assessment Database. An identification algorithm was developed to detect the regimens and LOTs. Treatment patterns and outcomes were assessed as real-world treatment sequence, treatment duration (rwTD), time to next-line treatment (rwTTNT), and overall survival (rwOS). Economic burden was assessed as healthcare resource utilization (HCRU) and the cost incurred per person per month.

Results: This study included 11,450 patients who were newly diagnosed with MM between January 2010 and December 2019. The observed real-world LOT patterns reflect the changes in South Korea's reimbursement scheme. Mean treatment-free intervals decreased from 11.59 months (SD 16.23) to 2.77 months (SD 6.14) from the first LOT (LOT 1) to LOT 5. Median rwTTNT decreased from 26.61 months (95% CI: 25.69-27.57) to 12.40 months (95% CI: 11.55-13.49), and median rwOS decreased from 61.88 months (95% CI: 59.11-65.46) to 13.65 months (95% CI: 11.88-16.22). The HCRU and associated costs increased substantially with the LOT advancement.

Conclusion: This large-scale observational study offers comprehensive insights into the real-world treatment of MM in South Korea. The study findings highlight the progressive nature of MM and increasing economic burden of advanced lines of treatment, underscoring the necessity for optimized treatment strategies.

目的:鉴于亚洲多发性骨髓瘤(MM)发病率的显著增加和创新治疗方法的出现,本研究旨在全面了解韩国多发性骨髓瘤(MM)的治疗模式、结果和经济负担。方法:采用健康保险审查与评估数据库提供的国民健康保险理赔数据进行回顾性队列研究。开发了一种识别算法来检测方案和lot。治疗模式和结果评估为真实世界的治疗顺序、治疗持续时间(rwTD)、到下一线治疗的时间(rwTTNT)和总生存期(rwOS)。经济负担评估为卫生保健资源利用率(HCRU)和每人每月发生的成本。结果:该研究纳入了2010年1月至2019年12月期间新诊断为MM的11,450例患者。观察到的现实世界的LOT模式反映了韩国报销计划的变化。从第一个LOT (LOT 1)到LOT 5,平均无治疗间隔从11.59个月(SD 16.23)减少到2.77个月(SD 6.14)。中位rwTTNT从26.61个月(95% CI: 25.69-27.57)下降到12.40个月(95% CI: 11.55-13.49),中位rwOS从61.88个月(95% CI: 59.11-65.46)下降到13.65个月(95% CI: 11.88-16.22)。随着LOT的发展,HCRU和相关成本大幅增加。结论:这项大规模观察性研究为韩国MM的现实治疗提供了全面的见解。研究结果强调了多发性骨髓瘤的进行性和先进治疗方法的经济负担增加,强调了优化治疗策略的必要性。
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引用次数: 0
Analysis of hemolysis-associated acute myeloid leukemia genes obtained using weighted gene co-expression network analysis and a Mendelian randomization study. 利用加权基因共表达网络分析和孟德尔随机化研究获得溶血相关急性髓性白血病基因分析。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-04-11 DOI: 10.1007/s44313-025-00073-7
Rui Zhang, Yan Zang, Linguo Wan, Hui Yu, Zhanshan Cha, Haihui Gu

Purpose: We used bioinformatics methods and Mendelian randomization (MR) analysis to investigate the hub genes involved in acute myeloid leukemia (AML) and their causal relationship with hemolysis, to explore a new direction for molecular biology research of AML.

Methods: We first differentially analyzed peripheral blood samples from 62 healthy volunteers and 65 patients with AML from the Gene Expression Omnibus database to obtain differentially expressed genes (DEGs), and intersected them with genes sourced from weighted gene co-expression network analysis (WGCNA) and the GeneCards database to obtain target genes. Target genes were screened using protein-protein interaction (PPI) network analysis and ROC curves to identify genes associated with AML. Finally, we analyzed the correlation between genes and immune cells and the relationship between toll-like receptor 4 (TLR4) and AML using MR.

Results: We compared peripheral blood expression profiles using an array of 62 healthy volunteers (GSE164191) and 65 patients with AML (GSE89565) (M0:25; M1:11; M2:10; M3:1; M4:7; M4 eo t [16;16] ou inv [16]:4; M5:6; M6:1) and obtained 7,339 DEGs (3,733 upregulated and 3,606 downregulated). We intersected these DEGs with 4,724 genes from WGCNA and 1,330 genes related to hemolysis that were identified in the GeneCards database to obtain 190 target genes. After further screening these genes using the PPI network, we identified TLR4, PTPRC, FCGR3B, STAT1, and APOE, which are closely associated with hemolysis in patients with AML. Finally, we found a causal relationship between TLR4 and AML occurrence using MR analysis (p < 0.05).

Conclusion: We constructed a WGCNA-based co-expression network and identified hemolysis-associated AML genes.

目的:利用生物信息学方法和孟德尔随机化(Mendelian randomization, MR)分析探讨急性髓性白血病(acute myeloid leukemia, AML)相关枢纽基因及其与溶血的因果关系,为AML分子生物学研究探索新的方向。方法:首先对来自基因表达Omnibus数据库的62名健康志愿者和65名AML患者的外周血样本进行差异分析,获得差异表达基因(deg),并将其与来自加权基因共表达网络分析(WGCNA)和GeneCards数据库的基因交叉,获得靶基因。利用蛋白-蛋白相互作用(PPI)网络分析和ROC曲线筛选靶基因,鉴定与AML相关的基因。最后,我们使用mr分析了基因与免疫细胞之间的相关性以及toll样受体4 (TLR4)与AML之间的关系。结果:我们比较了62名健康志愿者(GSE164191)和65名AML患者(GSE89565)的外周血表达谱(M0:25;M1:11;M2:10;M3:1;M4:7;[16;16] [au:] [au:]M5:6;M6:1),得到7339个deg(3733个上调,3606个下调)。我们将这些deg与来自WGCNA的4724个基因和GeneCards数据库中鉴定的1330个与溶血相关的基因进行交叉,获得190个靶基因。在使用PPI网络进一步筛选这些基因后,我们确定了TLR4、PTPRC、FCGR3B、STAT1和APOE,它们与AML患者的溶血密切相关。最后,我们通过MR分析发现TLR4与AML发生之间存在因果关系(p < 0.05)。结论:我们构建了基于wgna的共表达网络,并鉴定出溶血相关的AML基因。
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引用次数: 0
Very low-dose vemurafenib maintenance for cardiac Erdheim Chester disease. 非常低剂量vemurafenib维持心脏Erdheim Chester病。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-04-11 DOI: 10.1007/s44313-025-00075-5
Abhijeet Kumar Agrawal, Pronamee Borah, P D Rath, Rahul Naithani
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引用次数: 0
Correction: Evidence‑based Korean guidelines for the clinical management of multiple myeloma: addressing 12 key clinical questions. 更正:韩国多发性骨髓瘤临床治疗循证指南:解决 12 个关键临床问题。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-04-08 DOI: 10.1007/s44313-025-00074-6
Sung-Hoon Jung, Youngil Koh, Min Kyoung Kim, Jin Seok Kim, Joon Ho Moon, Chang-Ki Min, Dok Hyun Yoon, Sung-Soo Yoon, Je-Jung Lee, Chae Moon Hong, Ka-Won Kang, Jihyun Kwon, Kyoung Ha Kim, Dae Sik Kim, Sung Yong Kim, Sung-Hyun Kim, Yu Ri Kim, Young Rok Do, Yeung-Chul Mun, Sung-Soo Park, Young Hoon Park, Ho Jin Shin, Hyeon-Seok Eom, Sang Eun Yoon, Sang Mee Hwang, Won Sik Lee, Myung-Won Lee, Jun Ho Yi, Ji Yun Lee, Ji Hyun Lee, Ho Sup Lee, Sung-Nam Lim, Jihyang Lim, Ho-Young Yhim, Yoon Hwan Chang, Jae-Cheol Jo, Jinhyun Cho, Hyungwoo Cho, Yoon Seok Choi, Hee Jeong Cho, Ari Ahn, Jong Han Choi, Hyun Jung Kim, Kihyun Kim
{"title":"Correction: Evidence‑based Korean guidelines for the clinical management of multiple myeloma: addressing 12 key clinical questions.","authors":"Sung-Hoon Jung, Youngil Koh, Min Kyoung Kim, Jin Seok Kim, Joon Ho Moon, Chang-Ki Min, Dok Hyun Yoon, Sung-Soo Yoon, Je-Jung Lee, Chae Moon Hong, Ka-Won Kang, Jihyun Kwon, Kyoung Ha Kim, Dae Sik Kim, Sung Yong Kim, Sung-Hyun Kim, Yu Ri Kim, Young Rok Do, Yeung-Chul Mun, Sung-Soo Park, Young Hoon Park, Ho Jin Shin, Hyeon-Seok Eom, Sang Eun Yoon, Sang Mee Hwang, Won Sik Lee, Myung-Won Lee, Jun Ho Yi, Ji Yun Lee, Ji Hyun Lee, Ho Sup Lee, Sung-Nam Lim, Jihyang Lim, Ho-Young Yhim, Yoon Hwan Chang, Jae-Cheol Jo, Jinhyun Cho, Hyungwoo Cho, Yoon Seok Choi, Hee Jeong Cho, Ari Ahn, Jong Han Choi, Hyun Jung Kim, Kihyun Kim","doi":"10.1007/s44313-025-00074-6","DOIUrl":"10.1007/s44313-025-00074-6","url":null,"abstract":"","PeriodicalId":46224,"journal":{"name":"Blood Research","volume":"60 1","pages":"23"},"PeriodicalIF":2.3,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11979037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143812490","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
Frequent association of malignant effusions in plasmablastic lymphoma: a single-institutional experience of nine cases in Taiwan. 浆细胞性淋巴瘤常伴有恶性渗出:台湾一家医院九例病例的经验。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-04-07 DOI: 10.1007/s44313-025-00070-w
Bo-Jung Chen, Yu-Ting Kuo, Sheng-Tsung Chang, Khin-Than Win, Shang-Wen Chen, Sheng-Yen Hsiao, Yin-Hsun Feng, Yen-Chuan Hsieh, Shih-Sung Chuang

Purpose: Plasmablastic lymphoma (PBL) is a rare, aggressive lymphoma that is characterized by terminal B-cell differentiation. In the West, PBL usually occurs in patients with immunodeficiencies, particularly those induced by human immunodeficiency virus (HIV) infection. We investigated the clinicopathological features of PBL at a single institute in Taiwan, where HIV infection is rare.

Methods: This retrospective chart review identified PBL cases that were treated at a single institute in southern Taiwan between 2008 and 2024.

Results: We identified nine patients (four males and five females; median age 71 years). Of the eight patients tested for HIV, only one tested positive. Pathologically, the tumors showed plasmablastic morphology and immunophenotype, and three (33%) cases tested positive for Epstein-Barr virus. Six (67%) patients presented with Stage IV disease, including five (56%) with malignant effusion. Six patients were treated with chemotherapy and the remaining three received only supportive care. During a median follow-up of 10 months, five patients died of progressive disease, two died of unrelated diseases, and two were alive with PBL relapse.

Conclusion: In Taiwan, PBL constitutes a rare and aggressive clinical condition and is frequently associated with malignant effusion. In contrast to Western patients, the PBL in most patients from Taiwan was unrelated to HIV infection.

目的:浆母细胞淋巴瘤(PBL)是一种罕见的侵袭性淋巴瘤,以终末b细胞分化为特征。在西方,PBL通常发生在免疫缺陷患者,特别是由人类免疫缺陷病毒(HIV)感染引起的患者。我们在HIV感染罕见的台湾一所研究所调查PBL的临床病理特征。方法:回顾性分析2008年至2024年间在台湾南部同一所医院治疗的PBL病例。结果:我们确定了9例患者(4男5女;中位年龄71岁)。在接受艾滋病毒检测的8名患者中,只有1人呈阳性。病理上,肿瘤表现为浆母细胞形态和免疫表型,3例(33%)病例检测为eb病毒阳性。6例(67%)患者表现为IV期疾病,包括5例(56%)恶性积液。6名患者接受化疗,其余3名患者仅接受支持性治疗。在中位随访10个月期间,5例患者死于进展性疾病,2例死于无关疾病,2例PBL复发存活。结论:在台湾,PBL是一种罕见且具侵袭性的临床疾病,且常与恶性积液有关。与西方患者相比,台湾大多数患者的PBL与HIV感染无关。
{"title":"Frequent association of malignant effusions in plasmablastic lymphoma: a single-institutional experience of nine cases in Taiwan.","authors":"Bo-Jung Chen, Yu-Ting Kuo, Sheng-Tsung Chang, Khin-Than Win, Shang-Wen Chen, Sheng-Yen Hsiao, Yin-Hsun Feng, Yen-Chuan Hsieh, Shih-Sung Chuang","doi":"10.1007/s44313-025-00070-w","DOIUrl":"10.1007/s44313-025-00070-w","url":null,"abstract":"<p><strong>Purpose: </strong>Plasmablastic lymphoma (PBL) is a rare, aggressive lymphoma that is characterized by terminal B-cell differentiation. In the West, PBL usually occurs in patients with immunodeficiencies, particularly those induced by human immunodeficiency virus (HIV) infection. We investigated the clinicopathological features of PBL at a single institute in Taiwan, where HIV infection is rare.</p><p><strong>Methods: </strong>This retrospective chart review identified PBL cases that were treated at a single institute in southern Taiwan between 2008 and 2024.</p><p><strong>Results: </strong>We identified nine patients (four males and five females; median age 71 years). Of the eight patients tested for HIV, only one tested positive. Pathologically, the tumors showed plasmablastic morphology and immunophenotype, and three (33%) cases tested positive for Epstein-Barr virus. Six (67%) patients presented with Stage IV disease, including five (56%) with malignant effusion. Six patients were treated with chemotherapy and the remaining three received only supportive care. During a median follow-up of 10 months, five patients died of progressive disease, two died of unrelated diseases, and two were alive with PBL relapse.</p><p><strong>Conclusion: </strong>In Taiwan, PBL constitutes a rare and aggressive clinical condition and is frequently associated with malignant effusion. In contrast to Western patients, the PBL in most patients from Taiwan was unrelated to HIV infection.</p>","PeriodicalId":46224,"journal":{"name":"Blood Research","volume":"60 1","pages":"22"},"PeriodicalIF":2.3,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11977078/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796649","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Efficacy and safety of thalidomide with hydroxyurea in sickle cell anemia: a quasi-experimental clinical trial. 沙利度胺联合羟基脲治疗镰状细胞性贫血的疗效和安全性:一项准实验性临床试验。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-04-01 DOI: 10.1007/s44313-025-00068-4
Priyanka Samal, Anindita Paul, Harshwardhan Bahirat, Ajit Kumar Bishoyi, Venkatarao Epari

Background: The clinical course of sickle cell anemia (SCA) is variable, with chronic hemolysis and end-organ damage caused by microvascular occlusion. We evaluated the efficacy and safety of thalidomide plus hydroxyurea (HU) compared with HU alone to determine whether the combination provides a superior clinical benefit and safety profile.

Methods: This was an open-label quasi-experimental clinical trial (Clinical Trials Registry of India, CTRI Registration Number 2023/04/065682). Patients with SCA aged > 12 years and postmenopausal females aged > 45 years were allocated 1:1 to receive either HU (20 mg/kg/day) and thalidomide (50 mg/day) in Group A or HU (20 mg/kg/day) only in Group B.

Results: The frequency of vaso-occlusive crises (VOCs), transfusion requirements, variations in hematological parameters (hemoglobin [Hb], fetal hemoglobin [HbF], and sickle hemoglobin [HbS]), and side effects between the groups were assessed over 12 months. Repeated-measures analysis of variance was used to determine changes across the observation period. The mean age of the 66 patients diagnosed with SCA (homozygous HbS mutation) was 32.9 (standard deviation ± 11.5) years, and 57.6% were males. Over the 12-month observation period, Group A had significantly fewer VOCs (3.48 ± 2.81) and packed red blood cell transfusions (3.61 ± 2.19) than Group B (11.36 ± 4.20 VOCs; 13.27 ± 3.70 transfusions) (p = 0.0001). There was a significant increase in Hb (8.2 ± 1.8 to 11.8 ± 1.2 g/dL), a decrease in HbS% (72.5 ± 5.5 to 64.5 ± 5.4), and a rise in HbF% (18.9 ± 5.1 to 28.4 ± 5.6) (p < 0.0001) in Group A.

Conclusion: Combining thalidomide with HU significantly reduced VOCs and transfusion requirements, improved Hb and HbF%, and decreased HbS levels.

背景:镰状细胞性贫血(SCA)的临床病程是多变的,可伴有慢性溶血和微血管闭塞引起的终末器官损害。我们评估了沙利度胺联合羟基脲(HU)与单独使用HU的疗效和安全性,以确定联合使用是否具有更好的临床获益和安全性。方法:这是一项开放标签准实验临床试验(印度临床试验注册中心,CTRI注册号2023/04/065682)。年龄为> - 12岁的SCA患者和年龄为> - 45岁的绝经后女性按1:1分配,A组接受HU (20 mg/kg/天)和沙利度胺(50 mg/天)治疗,b组只接受HU (20 mg/kg/天)治疗。结果:在12个月内评估两组间血管闭塞危像(VOCs)的频率、输血需求、血液学参数(血红蛋白[Hb]、胎儿血红蛋白[HbF]和镰状血红蛋白[HbS])的变化以及副作用。重复测量方差分析用于确定整个观察期的变化。66例确诊为SCA (HbS纯合子突变)的患者平均年龄为32.9岁(标准差±11.5)岁,男性占57.6%。在12个月的观察期内,A组的VOCs(3.48±2.81)和红细胞充血(3.61±2.19)明显低于B组(11.36±4.20);13.27±3.70次)(p = 0.0001)。Hb升高(8.2±1.8至11.8±1.2 g/dL), HbS%降低(72.5±5.5至64.5±5.4),HbF%升高(18.9±5.1至28.4±5.6)(p)结论:沙利度胺联合HU可显著降低VOCs和输血需求,改善Hb和HbF%,降低HbS水平。
{"title":"Efficacy and safety of thalidomide with hydroxyurea in sickle cell anemia: a quasi-experimental clinical trial.","authors":"Priyanka Samal, Anindita Paul, Harshwardhan Bahirat, Ajit Kumar Bishoyi, Venkatarao Epari","doi":"10.1007/s44313-025-00068-4","DOIUrl":"10.1007/s44313-025-00068-4","url":null,"abstract":"<p><strong>Background: </strong>The clinical course of sickle cell anemia (SCA) is variable, with chronic hemolysis and end-organ damage caused by microvascular occlusion. We evaluated the efficacy and safety of thalidomide plus hydroxyurea (HU) compared with HU alone to determine whether the combination provides a superior clinical benefit and safety profile.</p><p><strong>Methods: </strong>This was an open-label quasi-experimental clinical trial (Clinical Trials Registry of India, CTRI Registration Number 2023/04/065682). Patients with SCA aged > 12 years and postmenopausal females aged > 45 years were allocated 1:1 to receive either HU (20 mg/kg/day) and thalidomide (50 mg/day) in Group A or HU (20 mg/kg/day) only in Group B.</p><p><strong>Results: </strong>The frequency of vaso-occlusive crises (VOCs), transfusion requirements, variations in hematological parameters (hemoglobin [Hb], fetal hemoglobin [HbF], and sickle hemoglobin [HbS]), and side effects between the groups were assessed over 12 months. Repeated-measures analysis of variance was used to determine changes across the observation period. The mean age of the 66 patients diagnosed with SCA (homozygous HbS mutation) was 32.9 (standard deviation ± 11.5) years, and 57.6% were males. Over the 12-month observation period, Group A had significantly fewer VOCs (3.48 ± 2.81) and packed red blood cell transfusions (3.61 ± 2.19) than Group B (11.36 ± 4.20 VOCs; 13.27 ± 3.70 transfusions) (p = 0.0001). There was a significant increase in Hb (8.2 ± 1.8 to 11.8 ± 1.2 g/dL), a decrease in HbS% (72.5 ± 5.5 to 64.5 ± 5.4), and a rise in HbF% (18.9 ± 5.1 to 28.4 ± 5.6) (p < 0.0001) in Group A.</p><p><strong>Conclusion: </strong>Combining thalidomide with HU significantly reduced VOCs and transfusion requirements, improved Hb and HbF%, and decreased HbS levels.</p>","PeriodicalId":46224,"journal":{"name":"Blood Research","volume":"60 1","pages":"21"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11961824/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143755019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical features and outcomes of JAK2 unmutated erythrocytosis. JAK2非突变红细胞增多症的临床特征和结果。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-03-31 DOI: 10.1007/s44313-025-00072-8
Jeong Suk Koh, Wonhyoung Seo, Sora Kang, Myung-Won Lee, Ik-Chan Song, Deog-Yeon Jo
{"title":"Clinical features and outcomes of JAK2 unmutated erythrocytosis.","authors":"Jeong Suk Koh, Wonhyoung Seo, Sora Kang, Myung-Won Lee, Ik-Chan Song, Deog-Yeon Jo","doi":"10.1007/s44313-025-00072-8","DOIUrl":"10.1007/s44313-025-00072-8","url":null,"abstract":"","PeriodicalId":46224,"journal":{"name":"Blood Research","volume":"60 1","pages":"20"},"PeriodicalIF":2.3,"publicationDate":"2025-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958889/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754910","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
Seroprevalence of SARS-CoV-2 antibodies in patients with hematological and oncological diseases in early 2024. 2024 年初血液病和肿瘤病患者的 SARS-CoV-2 抗体血清流行率。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-03-28 DOI: 10.1007/s44313-025-00067-5
Louise M Cremer, Jannik Stemler, Rosanne Sprute, Sebastian Herrmann, Theresa Markus, Jon Salmanton-García, Lutz Gieselmann, Veronica Di Cristanziano, Henning Gruell, Oliver A Cornely, Sibylle C Mellinghoff

Introduction: COVID-19 remains a major threat to immunocompromised individuals. The determination of circulating SARS-CoV-2 antibodies in patients at high risk for severe course of SARS-CoV-2 infection is important for estimating the vaccine-induced humoral immune response. Therefore, we assessed the status quo after winter to analyze the need for booster vaccinations.

Methods: Anti-spike IgG levels of 46 hospitalized patients with hematological and oncological diseases, measured between 21th December 2023 and 8th February 2024, were compared between subgroups of patients. Demographic data, underlying diseases, antineoplastic treatment, and the number of positive SARS-CoV-2 tests at the University Hospital Cologne were collected.

Results: Patients with different diseases showed varying SARS-CoV-2 spike antibody levels. The highest levels were found in patients with diffuse large cell B-cell lymphoma (DLBCL) and acute leukemia who had not received specific treatment or had just initiated treatment, whereas the lowest levels were found in patients with DLBCL, acute leukemia, and multiple myeloma who had received at least one line of treatment. The geometric mean antibody titers were higher in female patients than in male patients and were highest in patients aged 41-50 years while lowest in those aged 61-70 years.

Conclusion: The data presented confirm broad variations in SARS-CoV-2 anti-spike IgG levels across patients with different hematological and oncological diseases and highlight the complex interference of cancer biology, immune dysfunction, and treatment-related factors in shaping immune responses. Further research is needed to elucidate the mechanisms underlying these variations in antibody levels. We emphasize the need for regular booster vaccinations in this patient group.

导言:COVID-19仍然是免疫功能低下个体的主要威胁。在SARS-CoV-2严重病程高危患者中检测循环中的SARS-CoV-2抗体对于估计疫苗诱导的体液免疫应答具有重要意义。因此,我们评估了冬季后的现状,分析了加强疫苗接种的必要性。方法:对2023年12月21日至2024年2月8日住院的46例血液学和肿瘤学疾病患者的抗尖峰IgG水平进行亚组间比较。收集了科隆大学医院的人口统计数据、基础疾病、抗肿瘤治疗和SARS-CoV-2阳性检测数量。结果:不同疾病患者的SARS-CoV-2刺突抗体水平存在差异。在弥漫性大细胞b细胞淋巴瘤(DLBCL)和急性白血病患者中,未接受特异性治疗或刚刚开始治疗的患者中发现了最高水平,而在DLBCL、急性白血病和多发性骨髓瘤患者中发现了最低水平,这些患者至少接受了一条治疗线。几何平均抗体滴度女性高于男性,41 ~ 50岁最高,61 ~ 70岁最低。结论:这些数据证实了不同血液学和肿瘤学疾病患者的SARS-CoV-2抗刺突IgG水平存在广泛差异,并强调了癌症生物学、免疫功能障碍和治疗相关因素在形成免疫反应中的复杂干扰。需要进一步的研究来阐明这些抗体水平变化的机制。我们强调需要定期加强疫苗接种在这个病人组。
{"title":"Seroprevalence of SARS-CoV-2 antibodies in patients with hematological and oncological diseases in early 2024.","authors":"Louise M Cremer, Jannik Stemler, Rosanne Sprute, Sebastian Herrmann, Theresa Markus, Jon Salmanton-García, Lutz Gieselmann, Veronica Di Cristanziano, Henning Gruell, Oliver A Cornely, Sibylle C Mellinghoff","doi":"10.1007/s44313-025-00067-5","DOIUrl":"10.1007/s44313-025-00067-5","url":null,"abstract":"<p><strong>Introduction: </strong>COVID-19 remains a major threat to immunocompromised individuals. The determination of circulating SARS-CoV-2 antibodies in patients at high risk for severe course of SARS-CoV-2 infection is important for estimating the vaccine-induced humoral immune response. Therefore, we assessed the status quo after winter to analyze the need for booster vaccinations.</p><p><strong>Methods: </strong>Anti-spike IgG levels of 46 hospitalized patients with hematological and oncological diseases, measured between 21th December 2023 and 8th February 2024, were compared between subgroups of patients. Demographic data, underlying diseases, antineoplastic treatment, and the number of positive SARS-CoV-2 tests at the University Hospital Cologne were collected.</p><p><strong>Results: </strong>Patients with different diseases showed varying SARS-CoV-2 spike antibody levels. The highest levels were found in patients with diffuse large cell B-cell lymphoma (DLBCL) and acute leukemia who had not received specific treatment or had just initiated treatment, whereas the lowest levels were found in patients with DLBCL, acute leukemia, and multiple myeloma who had received at least one line of treatment. The geometric mean antibody titers were higher in female patients than in male patients and were highest in patients aged 41-50 years while lowest in those aged 61-70 years.</p><p><strong>Conclusion: </strong>The data presented confirm broad variations in SARS-CoV-2 anti-spike IgG levels across patients with different hematological and oncological diseases and highlight the complex interference of cancer biology, immune dysfunction, and treatment-related factors in shaping immune responses. Further research is needed to elucidate the mechanisms underlying these variations in antibody levels. We emphasize the need for regular booster vaccinations in this patient group.</p>","PeriodicalId":46224,"journal":{"name":"Blood Research","volume":"60 1","pages":"19"},"PeriodicalIF":2.3,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11953495/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143736148","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
Diffuse large B-cell lymphoma presenting as acute adrenal hemorrhage. 弥漫性大b细胞淋巴瘤表现为急性肾上腺出血。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-03-19 DOI: 10.1007/s44313-025-00064-8
Fabio Torres, Uriel Suárez, Paola Pizano
{"title":"Diffuse large B-cell lymphoma presenting as acute adrenal hemorrhage.","authors":"Fabio Torres, Uriel Suárez, Paola Pizano","doi":"10.1007/s44313-025-00064-8","DOIUrl":"10.1007/s44313-025-00064-8","url":null,"abstract":"","PeriodicalId":46224,"journal":{"name":"Blood Research","volume":"60 1","pages":"18"},"PeriodicalIF":2.3,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11923304/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143664936","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Blood Research
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