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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。
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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。
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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
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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。
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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
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引用次数: 0
Clonal hematopoiesis: elements associated with clonal expansion and diseases. 克隆造血:与克隆扩增和疾病相关的因素。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-03-13 DOI: 10.1007/s44313-025-00065-7
Gangpyo Ryu, Youngil Koh, Siddhartha Jaiswal, Sung-Soo Yoon

Clonal hematopoiesis (CH), characterized by the expansion of hematopoietic stem and progenitor cells harboring somatic mutations, has emerged as a significant age-related phenomenon with profound implications for human health. While initially recognized in the 1960s, recent technological advances have revealed its complex nature and widespread prevalence, affecting up to 84% of individuals aged ≥ 70 years. The clinical significance of CH extends beyond its well-established role as a precursor to hematological malignancies, encompassing its association with cardiovascular diseases, chronic kidney disease, and other non-malignant disorders. This comprehensive review synthesizes the current understanding of CH, focusing on recent advances in genetic and molecular mechanisms, particularly the roles of commonly mutated genes such as DNMT3A, TET2, and ASXL1. We address the emerging distinction between myeloid and lymphoid CH, their differential impacts on disease progression, and the complex interplay between CH and inflammation. Special attention is given to newly identified genetic determinants of clonal expansion rates and their implications for disease progression. The review also examines the revolutionary concept of passenger-approximated clonal expansion rate and its utility in understanding CH dynamics. Furthermore, we discuss therapeutic strategies targeting inflammatory pathways and their potential in mitigating CH-associated complications. By integrating recent findings from genetic, molecular, and clinical studies, this review provides a framework for understanding CH as a systemic condition and highlights promising directions for therapeutic interventions.

克隆造血(CH)以造血干细胞和体细胞突变的祖细胞的扩增为特征,已成为一种与年龄相关的重要现象,对人类健康具有深远的影响。虽然最初是在20世纪60年代认识到的,但最近的技术进步揭示了其复杂性和广泛的普遍性,影响到高达84%的≥70岁的个体。CH的临床意义不仅限于其作为血液系统恶性肿瘤的先兆,还包括其与心血管疾病、慢性肾脏疾病和其他非恶性疾病的关联。这篇综述综合了目前对CH的理解,重点介绍了遗传和分子机制的最新进展,特别是常见突变基因如DNMT3A、TET2和ASXL1的作用。我们讨论了髓系和淋巴系CH之间的新区别,它们对疾病进展的不同影响,以及CH与炎症之间复杂的相互作用。特别关注的是新发现的克隆扩增率的遗传决定因素及其对疾病进展的影响。该综述还探讨了乘客近似克隆扩张率的革命性概念及其在理解CH动力学中的应用。此外,我们讨论了针对炎症途径的治疗策略及其在减轻ch相关并发症方面的潜力。通过整合遗传、分子和临床研究的最新发现,本综述为理解CH作为一种系统性疾病提供了一个框架,并强调了治疗干预的有希望的方向。
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引用次数: 0
Correspondence: "Association of CD40 gene polymorphisms and immune thrombocytopenic purpura in the adult Egyptian population". 对应:“CD40基因多态性与埃及成年人群免疫性血小板减少性紫癜的关联”。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-03-07 DOI: 10.1007/s44313-025-00066-6
Elsayed Abdelkreem
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引用次数: 0
Transforming hematological research documentation with large language models: an approach to scientific writing and data analysis. 转化血液学研究文献与大型语言模型:一种方法,以科学写作和数据分析。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-03-06 DOI: 10.1007/s44313-025-00062-w
John Jeongseok Yang, Sang-Hyun Hwang

Large Language Models (LLMs), such as ChatGPT (OpenAI, CA, US), have revolutionized scientific writing and research processes across academic disciplines, providing comprehensive support throughout the entire research lifecycle. Generative artificial intelligence (GAI) tools enhance every aspect of scientific writing, from hypothesis generation and methodology design to data analysis and manuscript preparation. This review examines the applications of LLMs in hematological research, with particular emphasis on advanced techniques, including prompt engineering and retrieval augmented generation (RAG) frameworks. Prompt engineering methods, including zero-shot and few-shot learning along with a chain-of-thought approach, enable researchers to generate more precise context-specific content, especially in scientific writing. Integrating RAG frameworks with the current medical literature and clinical guidelines significantly reduces the risk of misinformation while ensuring alignment with contemporary medical standards. Even though these GAI tools offer remarkable potential for streamlining research writing and enhancing documentation quality, the study also addresses the critical importance of maintaining scientific integrity, ethical considerations, and privacy concerns in hematological research.

大型语言模型(llm),如ChatGPT (OpenAI, CA, US),已经彻底改变了跨学科的科学写作和研究过程,在整个研究生命周期中提供全面的支持。生成式人工智能(GAI)工具增强了科学写作的各个方面,从假设生成和方法设计到数据分析和手稿准备。本文综述了法学硕士在血液学研究中的应用,特别强调了先进的技术,包括快速工程和检索增强生成(RAG)框架。快速的工程方法,包括零射击和少射击学习以及思维链方法,使研究人员能够生成更精确的上下文特定内容,特别是在科学写作中。将RAG框架与当前的医学文献和临床指南相结合,可显著降低错误信息的风险,同时确保与当代医学标准保持一致。尽管这些GAI工具在简化研究写作和提高文档质量方面提供了显著的潜力,但该研究也解决了在血液学研究中维护科学完整性、伦理考虑和隐私问题的关键重要性。
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引用次数: 0
A case of dimethyl sulfoxide-induced seizure in multiple myeloma. 二甲亚砜致多发性骨髓瘤癫痫1例。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-03-04 DOI: 10.1007/s44313-025-00063-9
Ho Cheol Jang, Ga-Young Song, Mihee Kim, Seo-Yeon Ahn, Jae-Sook Ahn, Deok-Hwan Yang, Hyeoung-Joon Kim, Je-Jung Lee, Sung-Hoon Jung
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引用次数: 0
Rosai-Dorfman disease disguised as BRAF V600E-positive histiocytosis - a diagnostic pitfall. Rosai-Dorfman病伪装成BRAF v600e阳性组织细胞增多症——一个诊断陷阱。
IF 2.3 Q2 HEMATOLOGY Pub Date : 2025-02-28 DOI: 10.1007/s44313-025-00061-x
Aishwarya Ravindran, Gaurav Goyal, Diana Morlote, Alexander C Mackinnon
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引用次数: 0
期刊
Blood Research
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