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Luspatercept in Low-Risk Myelodysplastic Syndrome: A Real-World Single Institution Case Series. Luspatercept治疗低风险骨髓增生异常综合征:真实世界单一机构病例系列。
Pub Date : 2022-12-01 DOI: 10.1007/s44228-022-00016-4
Shamis Khan, Sara Taveras Alam, Rosa Torres Ramos, John Etumbani Mbue, Effrosyni Apostolidou, Gustavo A Rivero, Sarvari Venkata Yellapragada
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引用次数: 1
'Standing on the Shoulders of the Giants': Dr Rainer Storb. “站在巨人的肩膀上”:Rainer Storb博士。
Pub Date : 2022-12-01 DOI: 10.1007/s44228-022-00018-2
Mohamad Mohty, Rainer Storb
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引用次数: 1
The EBMT Immune Effector Cell Nursing Guidelines on CAR-T Therapy: A Framework for Patient Care and Managing Common Toxicities. CAR-T治疗的EBMT免疫效应细胞护理指南:患者护理和管理常见毒性的框架。
Pub Date : 2022-09-01 DOI: 10.1007/s44228-022-00004-8
Rose Ellard, Michelle Kenyon, Daphna Hutt, Erik Aerts, Maaike de Ruijter, Christian Chabannon, Mohamad Mohty, Silvia Montoto, Elisabeth Wallhult, John Murray

Chimeric antigen receptor T-cell (CAR T) therapy is a new and rapidly developing field. Centers across the world are gaining more experience using these innovative anti-cancer treatments, transitioning from the 'bench' to the 'bedside', giving benefit to an increasing number of patients. For those with some refractory hematological malignancies, CAR-T may offer a treatment option that was not available a few years ago.CAR-T therapy is an immune effector cell and precision/personalized medicine treatment which is tailored to the individual patient and associated with a variety of unique adverse events and toxicities that necessitate specialist nursing/medical vigilance in an appropriate clinical setting. Subtle unrecognized signs and symptoms can result in rapid deterioration and, possibly, life threatening cardiorespiratory and/or neurological sequelae.These guidelines have been prepared for nurses working in cellular therapy in inpatient, outpatient and ambulatory settings. Many nurses will encounter cellular therapy recipients indirectly, during the referral process, following discharge, and when patients are repatriated back to local centers. The aim of these guidelines is to provide all nurses with a practice framework to enable recognition, monitoring and grading of CAR-T therapy-associated toxicities, and to support and nurse these highly complex patients with confidence.They have been developed under the auspices of several bodies of the European society for Blood and Marrow Transplantation (EBMT), by experienced health professionals, and will be a valuable resource to all practitioners working in cellular therapy.

嵌合抗原受体T细胞(CAR - T)治疗是一个新兴的快速发展的领域。世界各地的医疗中心正在利用这些创新的抗癌治疗方法获得更多的经验,从“实验室”过渡到“床边”,使越来越多的患者受益。对于一些难治性恶性血液病患者,CAR-T可能提供了一种几年前无法获得的治疗选择。CAR-T疗法是一种免疫效应细胞和精确/个性化的药物治疗,针对个体患者量身定制,并与各种独特的不良事件和毒性相关,需要在适当的临床环境中进行专科护理/医疗警惕。未被识别的细微体征和症状可导致迅速恶化,并可能导致危及生命的心肺和/或神经系统后遗症。这些指导方针是为在住院、门诊和门诊环境中从事细胞治疗工作的护士准备的。许多护士会在转诊过程中、出院后以及患者被遣返回当地中心时间接遇到细胞疗法接受者。这些指南的目的是为所有护士提供一个实践框架,以便识别、监测和分级CAR-T治疗相关的毒性,并信心十足地支持和护理这些高度复杂的患者。它们是在欧洲血液和骨髓移植学会(EBMT)的几个机构的主持下,由经验丰富的保健专业人员开发的,将成为从事细胞治疗的所有从业人员的宝贵资源。
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引用次数: 6
FIRE Study: Real-World Effectiveness and Safety of Ibrutinib in Clinical Practice in Patients with CLL and MCL. FIRE研究:伊鲁替尼在CLL和MCL患者临床实践中的有效性和安全性。
Pub Date : 2022-09-01 Epub Date: 2022-09-14 DOI: 10.1007/s44228-022-00015-5
Caroline Dartigeas, Borhane Slama, Margaret Doyle, Christoph Tapprich, Claire Albrecht, Sandrine Dupuis, Robert Wapenaar, Charlotte Schmidt-Hieber, Veronique Leblond

The FIRE study investigated the real-world effectiveness and safety of ibrutinib in prospectively observed patients with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) and mantle cell lymphoma (MCL) in France. Patients were mostly relapsed/refractory with high-risk features. First-line CLL/SLL patients had del17p and/or TP53 mutations. In this interim analysis, the median follow-up time for patients with CLL/SLL and MCL was 17.7 and 15.1 months, respectively. In the effectiveness populations for CLL/SLL (n = 200) and MCL (n = 59), the median progression-free survival was not estimable and 12.4 months, respectively; the 12-month overall survival rates were 88.5% and 65.8%, respectively. Treatment-emergent adverse events of interest for patients with CLL/SLL (n = 202) and MCL (n = 59) included: infections and infestations (53.5% and 32.2%), major bleeding (5.0% and 5.1%), and atrial fibrillation (5.9% and 8.5%); 135 (66.8%) and 20 (33.9%) patients were continuing treatment at the time of data cutoff. Future analyses will report on longer-term follow-up (Trial registration: ClinicalTrials.gov, NCT03425591. Registered 1 February 2018-Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT03425591 ).

FIRE研究调查了伊鲁替尼在法国对慢性淋巴细胞白血病(CLL)/小淋巴细胞淋巴瘤(SLL)和套细胞淋巴瘤(MCL)的前瞻性观察患者的实际有效性和安全性。患者多为复发/难治性高危患者。一线CLL/SLL患者有del17p和/或TP53突变。在这项中期分析中,CLL/SLL和MCL患者的中位随访时间分别为17.7个月和15.1个月。在CLL/SLL (n = 200)和MCL (n = 59)的有效人群中,中位无进展生存期分别为12.4个月和不可估计;12个月总生存率分别为88.5%和65.8%。CLL/SLL (n = 202)和MCL (n = 59)患者在治疗中出现的不良事件包括:感染和感染(53.5%和32.2%)、大出血(5.0%和5.1%)和房颤(5.9%和8.5%);截至数据截止时,135例(66.8%)和20例(33.9%)患者仍在继续治疗。未来的分析将报告长期随访(试验注册:ClinicalTrials.gov, NCT03425591)。2018年2月1日注册-回顾性注册,https://clinicaltrials.gov/ct2/show/NCT03425591)。
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引用次数: 2
Antiemetic Strategies in Patients Who Undergo Hematopoietic Stem Cell Transplantation. 接受造血干细胞移植患者的止吐策略。
Pub Date : 2022-09-01 Epub Date: 2022-07-11 DOI: 10.1007/s44228-022-00012-8
Sayako Yuda, Shigeo Fuji, Bipin Savani, Katie S Gatwood

Hematopoietic stem cell transplantation (HSCT) is an integral part of the treatment strategy in patients with a hematological disorder. Chemotherapy-induced nausea and vomiting (CINV) is still an issue in patients who undergo HSCT. While several guidelines for the antiemetic therapy against CINV have been published, there is no detailed information about appropriate antiemetic drugs for each conditioning regimen in HSCT. Various studies reported that the triplet of 5-HT3RA, NK1RA, and dexamethasone appears useful in HSCT. However, each antiemetic has unique adverse effects or interactions with specific drugs. Here, we review the literature relating to clinical trials on the prevention of CINV, and summarize the information to clarify the benefit of antiemetic regimens.

造血干细胞移植(HSCT)是血液系统疾病患者治疗策略的一个组成部分。化疗引起的恶心和呕吐(CINV)仍然是接受HSCT患者的一个问题。虽然已经发表了一些针对CINV的止吐治疗指南,但没有关于HSCT中每种调理方案合适的止吐药物的详细信息。各种研究报道,5-HT3RA、NK1RA和地塞米松的三联体在HSCT中是有用的。然而,每种止吐药都有独特的副作用或与特定药物的相互作用。在此,我们回顾了与CINV预防临床试验相关的文献,并总结了相关信息,以阐明止吐方案的益处。
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引用次数: 4
High Expression of the Costimulatory Checkpoint Factor DNAM-1 by CD4+ T-Cells from Multiple Myeloma Patients Refractory to Daratumumab-Containing Regimens. 对含daratumumab治疗方案难治性多发性骨髓瘤患者CD4+ t细胞中共刺激检查点因子DNAM-1的高表达
Pub Date : 2022-09-01 Epub Date: 2022-08-09 DOI: 10.1007/s44228-022-00013-7
Katrine Fladeland Iversen, Line Nederby, Thomas Lund, Torben Plesner

Multiple myeloma is an incurable disease characterized by unregulated growth of malignant plasma cells in the bone marrow (BM). Tumor-induced dysfunction of T-cells may be responsible for immune evasion and failure of immunotherapy. Therefore, a better understanding of the phenotype of T-cells at the tumor site is needed. We assessed the expression of immune regulatory receptors on T-cell subsets from peripheral blood (PB) and BM using multicolor flow cytometry. Paired PB and BM samples were collected from newly diagnosed, treatment-naïve myeloma patients (n = 19) and patients progressing during treatment with the CD38 monoclonal antibody daratumumab alone or in combination with other anti-myeloma drugs (n = 39). We observed that CD4+ T-cells from both PB and BM of patients relapsing on daratumumab have a higher expression of the costimulatory checkpoint receptor DNAM-1. The potential role of DNAM-1+CD4+ T-cells in the development of resistance to daratumumab needs further exploration. We also observed that the inhibitory checkpoint receptor TIGIT is more frequently expressed by BM CD8+ T-cells from myeloma patients than PD-1 and CTLA-4, which supports the hypothesis that TIGIT may play a central role in the immune escape of the malignant plasma cells.

多发性骨髓瘤是一种无法治愈的疾病,其特征是骨髓中恶性浆细胞(BM)生长不受调节。肿瘤诱导的t细胞功能障碍可能是免疫逃避和免疫治疗失败的原因。因此,需要更好地了解肿瘤部位t细胞的表型。我们使用多色流式细胞术评估了外周血(PB)和BM中免疫调节受体在t细胞亚群上的表达。从新诊断的treatment-naïve骨髓瘤患者(n = 19)和在单独使用CD38单克隆抗体daratumumab或联合其他抗骨髓瘤药物治疗期间进展的患者(n = 39)中收集成对的PB和BM样本。我们观察到,服用达拉单抗复发的PB和BM患者的CD4+ t细胞表达更高的共刺激检查点受体DNAM-1。DNAM-1+CD4+ t细胞在daratumumab耐药发展中的潜在作用有待进一步探索。我们还观察到,来自骨髓瘤患者的BM CD8+ t细胞比PD-1和CTLA-4更频繁地表达抑制检查点受体TIGIT,这支持了TIGIT可能在恶性浆细胞的免疫逃逸中发挥核心作用的假设。
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引用次数: 0
Organizational Challenges in the Pediatric Onco-hematology Units During the First and Second Wave of the COVID-19 Pandemic: A National Survey in Italy. 在COVID-19大流行的第一波和第二波期间,儿科肿瘤血液科的组织挑战:意大利的一项全国调查。
Pub Date : 2022-09-01 DOI: 10.1007/s44228-022-00010-w
Matteo Amicucci, Valentina Biagioli, Elena Rostagno, Marta Canesi, Anna Bergadano, Debora Botta, Moreno Crotti Partel

This study aimed to describe and compare, at a national level, the measures implemented in the pediatric onco-hematology units and the number of infections among patients and healthcare staff during the first and second wave of the COVID-19 pandemic in Italy. A multicenter, descriptive, online survey was conducted between15th March and 15th April 2020 (T1) and between 1 and 31st January 2021 (T2). All the Italian Pediatric Oncology and Hematology Association (AIEOP) centers were invited to participate in the study. Data of the pre-pandemic, first, and second phase were compared. Thirty-six of the 48 AIEOP centers completed the survey (75%). Several organizational, screening, and swab measures were implemented by AIEOP centers to prevent the SARS-CoV-2 infection among patients and visitors. During the pandemic, there was a significant reduction in the number of onco-hematology inpatient beds (p < 0.001), including inpatient beds dedicated to hematopoietic stem cell transplantation (HSCT), and consultations in the outpatient clinics (p < 0.001). During the first wave, 37 pediatric patients with cancer tested positive for SARS-CoV-2 versus 174 patients during the second wave. The reduction in routine services was also greater in the second than in the first wave. All the AIEOP centers showed the capacity to adapt and promptly respond to both waves of the pandemic.

本研究旨在描述和比较意大利在第一波和第二波COVID-19大流行期间儿科肿瘤血液学单位实施的措施以及患者和医护人员的感染数量。在2020年3月15日至4月15日(T1)和2021年1月1日至31日(T2)之间进行了一项多中心描述性在线调查。所有意大利儿科肿瘤和血液学协会(AIEOP)中心被邀请参加这项研究。比较了大流行前、第一阶段和第二阶段的数据。48个AIEOP中心中有36个完成了调查(75%)。AIEOP中心实施了一些组织、筛查和棉签措施,以防止患者和访客感染SARS-CoV-2。在大流行期间,肿瘤血液学住院床位的数量显著减少(p
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引用次数: 2
Ethnic Disparities in AL Amyloidosis Outcomes Among Hospitalized Patients in the United States. 美国住院患者AL淀粉样变结局的种族差异
Pub Date : 2022-09-01 Epub Date: 2022-07-29 DOI: 10.1007/s44228-022-00014-6
Samer Al Hadidi, Deepa Dongarwar, Hamisu Salihu, Carolina Schinke, Sharmilan Thanendrarajan, Maurizio Zangari, Frits van Rhee
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引用次数: 1
Clinical, Diagnostic and Prognostic Characteristics of Primary Cutaneous Gamma Delta T-cell Lymphomas. 原发性皮肤γ δ t细胞淋巴瘤的临床、诊断和预后特点。
Pub Date : 2022-06-22 eCollection Date: 2022-06-01 DOI: 10.1007/s44228-022-00011-9
Ibrahim N Muhsen, Riad El Fakih, Mehdi Hamadani, Hillard M Lazarus, Mohamed A Kharfan-Dabaja, Mahmoud Aljurf

Primary cutaneous γδ T-cell lymphoma (PCGDTL) is a rare subtype of non-Hodgkin lymphoma (NHL) that arises from T-cells with γδ T-cell receptors. The exact incidence of PCGDTL is unknown, as it is usually lumped with other cutaneous lymphomas, which are also uncommon. It is one of the peripheral T-cell lymphoma (PTCL) subtypes which is known to have a dismal prognosis due to poor response and the paucity of available therapies. Despite the rarity and uncertainties of PCGDTL, a number of studies over the past decade were published about the pathologic, diagnostic, cytogenetic and clinical features of this disease. These diagnostic advances will open the doors to explore new therapeutics for this rare entity, specifically targeted and immune therapies. In this review, we highlight these advances, summarize the contemporary treatment approaches, and shed the light on future potential therapeutic targets.

原发性皮肤γδ t细胞淋巴瘤(PCGDTL)是一种罕见的非霍奇金淋巴瘤(NHL)亚型,起源于具有γδ t细胞受体的t细胞。PCGDTL的确切发病率尚不清楚,因为它通常与其他皮肤淋巴瘤合并,这也不常见。它是周围t细胞淋巴瘤(PTCL)亚型之一,由于反应差和缺乏可用的治疗方法,已知预后不佳。尽管PCGDTL的罕见性和不确定性,在过去十年中发表了许多关于该疾病的病理、诊断、细胞遗传学和临床特征的研究。这些诊断的进步将为探索这种罕见的实体的新疗法,特别是靶向和免疫疗法打开大门。在这篇综述中,我们强调了这些进展,总结了当代的治疗方法,并阐明了未来潜在的治疗靶点。
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引用次数: 5
Retrospective Multicenter Real-Life Study on the First-Line Treatment of Classical Hodgkin Lymphoma in Argentina. 阿根廷经典霍奇金淋巴瘤一线治疗的回顾性多中心现实研究。
Pub Date : 2022-06-22 eCollection Date: 2022-06-01 DOI: 10.1007/s44228-022-00008-4
Carolina Mahuad, Otero Victoria, Korin Laura, Martinez Enriqueta, Warley Fernando, García Rivello Hernán, Cristaldo Nancy, Kohan Dana, Zerga Marta, Garate Gonzalo, Vicente Repáraz María de Los Ángeles, Aizpurua Florencia, Rojas Bilbao Erica, Cerana Susana, Funes Maria Eugenia, Plaza Iliana, Foncuberta Cecilia, Vijnovich Baron Anahí, Cranco Santiago, Vitriu Adriana, Gomez Mariela, Lavalle Justina, Casali Claudia, Clavijo Manuela, Melillo Luciana, Cabral Lorenzo Maria Cecilia, Miroli Augusto, Fischman Laura, Pavlove Maximiliano, Miodosky Marcela, Cugliari Silvana

There are no data in Argentina on the response rates to first-line treatment of classical Hodgkin Lymphoma (cHL) outside clinical trials. A total of 498 patients from 7 public and private hospitals in Argentina were retrospectively examined. The median follow-up was 37.4 months (CI 95% 17.7-63.5). The median time from diagnosis to treatment was 22 days (IQR 14-42), which was significantly longer in public hospitals (49.3 (IC 95% 38.5-60.2) versus 32.5 (IC 95% 27-38); p = 0.0027). A total of 96.8% of patients were treated with ABVD.:84.3% achieved complete remission (CR) and 6.02% partial remission (PR), being the CR rate higher in private hospitals. End-of-treatment metabolic CR was achieved in 85.4% (n = 373). The interim PET scan was widely used in our cohort (70.5%; n = 351), but in only 23.3% (n = 116) was the treatment strategy response-adapted. The 5-year progression-free survival (PFS) was 76% (CI 95% 70-81). The 2 and 5-years-OS rates were 91% (CI 95% 88-94%) and 85% (CI 95% 80-89%), respectively. No differences in OS were found between public and private institutions (p = 0.27). This is one of the largest retrospective cHL cohorts reported. In Argentina ABVD is the chemotherapy regimen of choice and, although it is well tolerated, it is not exempt from toxicity. We showed that early initiation of treatment impacts the induction results. Although the use of PET scan is widespread, only a minority of patients was treated with respons- adapted strategies. The use of PET-guided treatment is strongly encouraged.

在阿根廷,临床试验之外没有关于经典霍奇金淋巴瘤(cHL)一线治疗反应率的数据。回顾性分析了来自阿根廷7家公立和私立医院的498名患者。中位随访时间为37.4个月(CI 95% 17.7-63.5)。从诊断到治疗的中位时间为22天(IQR 14-42),公立医院明显更长(49.3天(IQR为38.5-60.2)比32.5天(IQR为27-38);p = 0.0027)。96.8%的患者接受ABVD治疗,其中84.3%达到完全缓解(CR), 6.02%达到部分缓解(PR),在私立医院中CR率较高。治疗结束时代谢CR达到85.4% (n = 373)。中期PET扫描在我们的队列中被广泛使用(70.5%;N = 351),但只有23.3% (N = 116)的治疗策略符合疗效。5年无进展生存率(PFS)为76% (CI 95% 70-81)。2年和5年os率分别为91% (CI 95% 88-94%)和85% (CI 95% 80-89%)。公立和私立机构的OS无差异(p = 0.27)。这是报道的最大的回顾性cHL队列之一。在阿根廷,ABVD是首选的化疗方案,尽管它耐受性良好,但也不能免除毒性。我们发现早期开始治疗会影响诱导结果。虽然PET扫描的使用是广泛的,但只有少数患者接受了反应适应策略的治疗。强烈鼓励使用pet引导治疗。
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引用次数: 1
期刊
Clinical Hematology International
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