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Nutrition of patients undergoing haematopoietic stem cell transplantation. 造血干细胞移植患者的营养。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025345
B Kašperová, M Mego, S Čierniková, A Ševčíková, S Kašperová, A Vranovský, L Drgoňa

Background: Haematopoietic stem cell transplantation is associated with increased demands for adequate caloric intake and heightened risk of macronutrient and micronutrient depletion. Carbohydrates, proteins, and fats represent a key source of energy and structural components for transplanted patients, who often have limited oral intake because of mucositis and loss of appetite and increased nutritional requirements due to catabolism, inflammation, and tissue regeneration. Deficiencies in vitamins and trace elements occurring in some patients play a crucial role in enzymatic reactions, antioxidant defense, immune function, and tissue repair. Preparative regimen also causes damage of the intestinal mucosa and, in combination with antibiotic therapy, reduces the diversity of the microbiome. According to the latest evidence, patient nutrition has an impact on the short- and long-term outcomes of transplantation. Recognition of malnutrition and catabolism in these patients is difficult in routine practice; closer analysis of body composition and early intervention by a clinical nutritionist may be helpful. Adequate nutrient replacement is an important aspect of maintaining nutritional balance and good recovery.

Aim: The aim of this article is to provide an overview of nutrition, its specific components and nutritional disorders in oncology patients, as well as to summarize specific complications of aggressive treatment in patients undergoing hematopoietic stem cell transplantation and underline the need for early nutritional intervention in this group of patients.

背景:造血干细胞移植与对足够热量摄入的需求增加以及宏量营养素和微量营养素消耗的风险增加有关。碳水化合物、蛋白质和脂肪是移植患者能量和结构成分的主要来源,这些患者通常由于黏膜炎和食欲不振而口服摄入有限,并且由于分解代谢、炎症和组织再生而增加营养需求。一些患者缺乏维生素和微量元素,在酶反应、抗氧化防御、免疫功能和组织修复中起着至关重要的作用。制备方案也会引起肠黏膜的损伤,并与抗生素治疗相结合,减少微生物组的多样性。根据最新的证据,患者的营养对移植的短期和长期结果都有影响。在常规实践中很难识别这些患者的营养不良和分解代谢;对身体成分进行更仔细的分析并由临床营养学家进行早期干预可能会有所帮助。充足的营养补充是维持营养平衡和良好恢复的重要方面。目的:本文的目的是概述肿瘤患者的营养,其特定成分和营养失调,以及总结在接受造血干细胞移植的患者积极治疗的特定并发症,并强调对这组患者进行早期营养干预的必要性。
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引用次数: 0
Commentary on the CheckMate 9ER clinical trial. 对CheckMate 9ER临床试验的评论。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025307
I- Richter, J Bartoš, J Dvořák
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引用次数: 0
Real-world PD-L1 testing, fi rst-line therapy for advanced NSCLC, and fi rst-line pembrolizumab monotherapy utilization and outcomes for metastatic NSCLC in the Czech Republic. 真实世界PD-L1测试,晚期NSCLC的一线治疗,以及捷克共和国转移性NSCLC的一线派姆单抗单药治疗的使用和结果。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025192
M L Santorelli, M Chrápavá, K Hrabcová, G Bencina, J Blažek, M Bratová, O Fibigr, O Fischer, L Koubková, J Krejčí, A Ryška, P Zemanová, T Burke

Background: This study aimed to describe real-world PD-L1 testing and first-line (1L) treatment patterns for advanced non-small cell lung cancer (NSCLC), and clinical outcomes for metastatic NSCLC after 1L pembrolizumab monotherapy became reimbursed in the Czech Republic (February 2019).

Patients and methods: This descriptive noninterventional study drew on two Czech lung cancer registries. We examined PD-L1 testing patterns and results in the KELLY registry for samples submitted on/after 1-Feb-2019 from adult patients with advanced NSCLC. Using the TULUNG registry, we summarized 1L targeted therapies initiated on/after 1-Feb-2019 for advanced NSCLC, in addition to characteristics and outcomes for patients treated with 1L pembrolizumab monotherapy for metastatic NSCLC, PD-L1 tumor proportion score (TPS) ≥ 50%, and no known EGFR/ALK alterations. Real-world time on treatment (rwToT) and overall survival (OS) were determined using Kaplan-Meier curves. The data cutoff was 16-Sept-2021.

Results: The percentage of NSCLC samples in the KELLY registry tested for PD-L1 expression increased from 70.5% in 2019 to 84.4% in 2021. Pembrolizumab monotherapy was the most common 1L targeted therapy in 2019-2021 for patients with advanced NSCLC and PD-L1 TPS ≥ 50% (N = 315), administered to 70-80% each year. Of 235 patients with metastatic NSCLC who received 1L pembrolizumab monotherapy, median age was 69 years, 54% were men, 52% were current smokers, and 28% had squamous NSCLC. Median rwToT was 8.5 months (95% CI; 6.7-10.1), with 6- and 12-month on-treatment rates of 59% and 36%, respectively, for 199 patients with ≥ 6 months of follow-up. With added national registry mortality data, estimated median OS was 13.7 months (12.3-17.7); 6- and 12-month OS rates were 70% and 59%, respectively.

Conclusions: The rates of PD-L1 testing increased from 2019 to 2021. Median OS among patients with metastatic NSCLC and PD-L1 TPS ≥ 50% treated with pembrolizumab was lower than in clinical trials, likely due to differences between real-world patients and trial participants in age, smoking status, performance status, and squamous histology.

背景:本研究旨在描述晚期非小细胞肺癌(NSCLC)的现实世界PD-L1检测和一线(1L)治疗模式,以及捷克共和国(2019年2月)1L派姆单抗单药治疗获得报销后转移性NSCLC的临床结果。患者和方法:这项描述性非介入性研究利用了两个捷克肺癌登记处。我们研究了2019年2月1日/之后提交的成年晚期非小细胞肺癌患者的KELLY注册样本的PD-L1检测模式和结果。使用TULUNG注册表,我们总结了2019年2月1日/之后开始治疗晚期NSCLC的1L靶向治疗,以及接受1L pembrolizumab单药治疗的转移性NSCLC患者的特征和结果,PD-L1肿瘤比例评分(TPS)≥50%,无已知EGFR/ALK改变。使用Kaplan-Meier曲线确定实际治疗时间(rwToT)和总生存期(OS)。数据截止日期为2021年9月16日。结果:KELLY注册表中PD-L1表达检测的NSCLC样本百分比从2019年的70.5%上升到2021年的84.4%。2019-2021年,对于PD-L1 TPS≥50% (N = 315)的晚期NSCLC患者,派姆单抗单药治疗是最常见的1L靶向治疗,每年给药率为70-80%。235例接受1L派姆单抗单药治疗的转移性NSCLC患者,中位年龄为69岁,54%为男性,52%为吸烟者,28%为鳞状NSCLC。中位rwToT为8.5个月(95% CI;6.7-10.1),随访≥6个月的199例患者,6个月和12个月的治疗率分别为59%和36%。加上国家登记死亡率数据,估计中位生存期为13.7个月(12.3-17.7个月);6个月和12个月的OS率分别为70%和59%。结论:PD-L1检测率从2019年到2021年有所上升。pembrolizumab治疗的转移性NSCLC和PD-L1 TPS≥50%的患者的中位OS低于临床试验,可能是由于真实患者和试验参与者在年龄、吸烟状况、表现状况和鳞状组织学上的差异。
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引用次数: 0
Monoclonal gammopathy of clinical significance - a group name for diseases caused by monoclonal immunoglobulin and/ or free light chains. A change in the approach to non-malignant gammopathies. 具有临床意义的单克隆伽玛病——由单克隆免疫球蛋白和/或游离轻链引起的疾病的总称。非恶性伽玛病治疗方法的改变。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025336
Z Adam, Z Řehák, M Krejčí, I Boichuk, L Pour

Background: The group of monoclonal gammopathies includes both malignant diseases, such as multiple myeloma and Waldenström's macroglobulinemia, and benign proliferations of plasma cells or lymphoplasmacytic cells with the formation of usually low concentrations of monoclonal immunoglobulin and/or free light chains. The term monoclonal gammopathy of undetermined significance (MGUS) is used for conditions with non-malignant proliferation.

Aim: In the last 25 years, understanding of the various forms of diseases caused by products of benign lymphoplasmacytic or plasma cell proliferation has significantly increased. The following terms have been used for them: monoclonal gammopathy of renal significance, monoclonal gammopathy of cutaneous significance, monoclonal gammopathy of neurological significance. In the years 2018-2020, the international hematology community accepted the group name monoclonal gammopathy of clinical significance for all conditions in which organ damage is etiopathogenetically related to the products (MGCS) of these cell clones. The text presents an overview of all clinical units that belong to this group and briefly discusses the etiopathogenesis and therapy of these diseases.

Conclusion: MGCS is a new term that deserves to be incorporated into the new International Classification of Diseases, which would help improve epidemiological information about these very rare diseases. The same drugs used to treat malignant gammopathies are used in the treatment of MGCS. Patients with MGCS are discriminated, because their diagnoses are not included in the registration studies of new drugs. Therefore, targeted treatment can only be used with the approval of the healthcare payer.

背景:单克隆伽玛病既包括恶性疾病,如多发性骨髓瘤和Waldenström的巨球蛋白血症,也包括浆细胞或淋巴浆细胞的良性增生,通常形成低浓度的单克隆免疫球蛋白和/或游离轻链。未确定意义的单克隆γ病(MGUS)一词用于非恶性增殖的条件。目的:在过去的25年里,对良性淋巴浆细胞或浆细胞增殖产物引起的各种疾病的认识有了显著的提高。以下术语已用于它们:肾脏意义的单克隆伽玛病,皮肤意义的单克隆伽玛病,神经意义的单克隆伽玛病。在2018-2020年期间,国际血液学界接受了具有临床意义的单克隆伽玛病(monoclonal gammopathy)这一组名称,用于与这些细胞克隆的产物(MGCS)相关的器官损伤的所有病症。本文提出了属于这一组的所有临床单位的概述,并简要地讨论了这些疾病的发病机制和治疗。结论:MGCS是一个新的术语,值得纳入新的《国际疾病分类》,这将有助于改善这些非常罕见的疾病的流行病学信息。用于治疗恶性伽玛病的相同药物也用于治疗MGCS。MGCS患者受到歧视,因为他们的诊断不包括在新药注册研究中。因此,有针对性的治疗只能在医疗保健支付方批准的情况下使用。
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引用次数: 0
Characteristics of cytostatic therapy in geriatric patients. 老年患者细胞抑制剂治疗的特点。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025368
P Oláh, K Rejlekova

Background: Chemotherapy is a key therapeutic modality in the treatment of malignant diseases. While it represents an effective tool in managing these conditions, it is simultaneously a toxic treatment associated with numerous adverse effects, particularly in fragile patients, such as the elderly. Adverse effects of cytotoxic therapy are among the key factors contributing to the poor prognosis of older cancer patients, often resulting in treatment delays, dose reductions, or even premature termination of therapy. The increased susceptibility of geriatric patients to the toxic effects of chemotherapy arises from their unique characteristics. Alterations in the pharmacokinetics of anticancer drugs in elderly patients represent one of the main mechanisms of chemotherapy toxicity in this patient population. The presence of comorbidities further elevates the risk of adverse effects and diminishes the body's tolerance to treatment. As a consequence, there may be an increased vulnerability to organ-specific toxicity of cytotoxic agents. Due to the presence of multiple comorbidities, older patients are often exposed to polypharmacy, which represents an additional risk factor for chemotoxicity due to the increased likelihood of drug interactions at both the pharmacodynamic and pharmacokinetic levels. Identifying at-risk patients through the application of comprehensive geriatric assessment and scoring tools provides an approach to evaluating chemotherapy-related risks, thereby supporting the personalization of treatment and the reduction of toxicity.

Aim: The article provides an analysis of the various mechanisms of chemotherapy toxicity in elderly patients, the factors contributing to their vulnerability, as well as the possibilities for safely administering cytotoxic agents while preserving their therapeutic efficacy in this patient population.

背景:化疗是恶性肿瘤治疗的重要手段。虽然它是管理这些疾病的有效工具,但它同时是一种有毒的治疗方法,与许多不良反应有关,特别是对脆弱的患者,如老年人。细胞毒性治疗的不良反应是导致老年癌症患者预后不良的关键因素之一,往往导致治疗延误、剂量减少,甚至过早终止治疗。老年患者对化疗毒性作用的易感性增加是由于其独特的特点。老年患者体内抗癌药物药代动力学的改变是该患者群体化疗毒性的主要机制之一。合并症的存在进一步增加了不良反应的风险,降低了身体对治疗的耐受性。因此,细胞毒性药物对器官特异性毒性的易感性可能会增加。由于多种合并症的存在,老年患者经常暴露于多种药物,这是化学毒性的另一个危险因素,因为在药效学和药代动力学水平上药物相互作用的可能性增加。通过应用综合老年评估和评分工具来识别高危患者,为评估化疗相关风险提供了一种方法,从而支持个性化治疗和减少毒性。目的:本文分析了老年患者化疗毒性的各种机制,导致其易感性的因素,以及在此患者群体中安全使用细胞毒性药物同时保持其治疗效果的可能性。
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引用次数: 0
Importance of circulating tumor DNA in colorectal cancer. 循环肿瘤DNA在结直肠癌中的重要性。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko202532
B Tolmáči, A Řehulková, P Žuff, J Klein

Background: Space still exists in the management of patients with colorectal cancer (CRC) for improving risk stratification and thus the precision of treatment tailoring. Quite promising in this regard are biomarkers acquired via liquid biopsy, which is a non-invasive method of body fluid draw, most commonly peripheral blood. A variety of biomarkers associated with the tumor are analyzed, which can have either prognostic or predictive value. Circulating tumor DNA (ctDNA) is one of the most explored tumor biomarkers. Initially, its utility spectrum was only in advanced or metastatic cancers and consisted of molecular profiling and detecting acquired resistance to treatment. Nowadays, the use of circulating tumor DNA has shifted to earlier cancer stages, where it can identify minimal residual disease or diagnose colorectal cancer early. Existing studies show promising potential of these biomarkers, but more information needs to be gathered and information from ongoing studies needs to be obtained in order to use them in everyday practice.

Aim: In this review article, we will discuss ctDNA, its aspects, diag- nostic possibilities and current use in CRC.

背景:在结直肠癌(CRC)患者的管理中,仍存在着提高风险分层和治疗精准度的空间。在这方面很有希望的是通过液体活检获得生物标志物,这是一种非侵入性的体液提取方法,最常见的是外周血。分析了与肿瘤相关的各种生物标志物,这些标志物可以具有预后或预测价值。循环肿瘤DNA (ctDNA)是目前研究最多的肿瘤生物标志物之一。最初,它的效用范围仅用于晚期或转移性癌症,包括分子谱分析和检测获得性治疗耐药性。如今,循环肿瘤DNA的使用已经转移到早期癌症阶段,在那里它可以识别最小的残留疾病或早期诊断结直肠癌。现有的研究表明这些生物标记物有很大的潜力,但需要收集更多的信息,并且需要从正在进行的研究中获得信息,以便在日常实践中使用它们。目的:在本文中,我们将讨论ctDNA,它的方面,诊断的可能性和目前在结直肠癌中的应用。
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引用次数: 0
Impact and detection of human cytomegalovirus in oncological diseases. 巨细胞病毒在肿瘤疾病中的作用及检测。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025254
A Fried, J Strmisková, K Magerová, M Hendrych, M Bartošík, L Moráňová

Background: Human cytomegalovirus (hCMV) is a widely prevalent herpesvirus that typically remains asymptomatic in immunocompetent individuals. However, in immunocompromised patients, it can cause severe clinical complications. In the context of cancer, hCMV exhibits oncomodulatory effects, influencing tumor growth, immune response, and treatment efficacy. Growing evidence suggests that therapeutic strategies targeting hCMV could improve cancer patient prognosis. Nevertheless, detecting this virus in tumor tissue or body fluids remains challenging, with results often varying depending on the methodology used and the type of sample analyzed.

Aim: This study provides a comprehensive overview of the role of hCMV in cancer. It describes the hCMV genome and the functions of its key proteins, focusing on their involvement in oncomodulation. The study thoroughly examines the mechanisms of viral interactions with cellular signaling pathways, the effects of infection and reactivation on the clinical course of cancer, and pays special attention to the impact of hCMV on glioblastoma, including studies assessing the effectiveness of antiviral therapy. Furthermore, standard diagnostic methods, including immunohistochemistry, ELISA, and polymerase chain reaction, are discussed, along with the most commonly used commercially available diagnostic kits approved for clinical practice. The study concludes by summarizing the key challenges associated with hCMV diagnosis and treatment in oncology and explores future therapeutic approaches, including the development of dendritic cell vaccines.

背景:人巨细胞病毒(hCMV)是一种广泛流行的疱疹病毒,通常在免疫正常的个体中保持无症状。然而,在免疫功能低下的患者中,它可引起严重的临床并发症。在癌症的背景下,hCMV表现出肿瘤调节作用,影响肿瘤生长、免疫反应和治疗效果。越来越多的证据表明,针对hCMV的治疗策略可以改善癌症患者的预后。然而,在肿瘤组织或体液中检测这种病毒仍然具有挑战性,结果往往因所使用的方法和分析的样本类型而异。目的:本研究全面概述了hCMV在癌症中的作用。它描述了hCMV基因组及其关键蛋白的功能,重点是它们参与肿瘤调节。该研究深入探讨了病毒与细胞信号通路相互作用的机制,感染和再激活对癌症临床进程的影响,并特别关注hCMV对胶质母细胞瘤的影响,包括评估抗病毒治疗效果的研究。此外,标准的诊断方法,包括免疫组织化学,ELISA和聚合酶链反应,讨论,以及最常用的商业诊断试剂盒批准临床实践。该研究总结了肿瘤中与hCMV诊断和治疗相关的主要挑战,并探索了未来的治疗方法,包括开发树突状细胞疫苗。
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引用次数: 0
Integrated clinical-bio logical infrastructure for precision oncology in pancreatic adenocarcinoma - experience with REDCap implementation in an academic setting. 胰腺腺癌精确肿瘤学的综合临床-生物基础设施- REDCap在学术环境中实施的经验。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025464
P Moravčík, P Weselá, V Kocandová, I Aćimović, J Hlavsa, V Procházka, J Vlažný, R Bárta, Z Kala, P Vaňhara, M Eid

Background: Pancreatic ductal adenocarcinoma (PDAC) is one of the most aggressive solid malignancies, characterized by poor five-year survival and limited options for early detection. The development of precision oncology and personalized treatment requires structured and longitudinal collection of clinical and biological data. For this purpose, the REDCap web-based data management platform was utilized.

Materials and methods: A project initiated by the Faculty of Medicine, Masaryk University, and University Hospital Brno aims to establish an integrated clinico-biological ecosystem for patients with PDAC. Data are managed within the REDCap system and include demographic characteristics, clinical features, treatment courses, molecular-genetic results, and survival outcomes. The pilot biobank stores patient-derived samples for the development of 3D models (organoids and spheroids). Data are analyzed using machine learning and artificial intelligence methods.

Results: A structured database linking clinical data with biological materials has been established. To date, detailed longitudinal data have been collected from 117 patients with pancreatic (predominantly PDAC) and extrahepatic biliary tract tumors. The database records diagnostic and therapeutic procedures as well as molecular-genetic profiling data and their relationship to treatment response. It serves as a foundation for the development of predictive models and biomarker validation.

Discussion: The project demonstrates the feasibility of comprehensive data collection within a university hospital setting. A major benefit is the ability to monitor treatment trajectories and implement precision oncology principles in clinical practice. Challenges include capacity and logistical demands, as well as the need for harmonization of input data. The initiative has the potential to expand into a national research infrastructure for PDAC.

Conclusion: The established infrastructure represents a foundation for a data-driven approach to PDAC management. By integrating clinical data and biological models, it contributes to the advancement of personalized care and provides a platform for research and decision support in oncology.

背景:胰腺导管腺癌(PDAC)是最具侵袭性的实体恶性肿瘤之一,其特点是5年生存率低,早期发现的选择有限。精确肿瘤学和个性化治疗的发展需要结构化和纵向收集临床和生物学数据。为此,利用基于web的REDCap数据管理平台。材料和方法:由马萨里克大学医学院和布尔诺大学医院发起的一个项目旨在为PDAC患者建立一个综合的临床-生物生态系统。数据在REDCap系统内进行管理,包括人口统计学特征、临床特征、治疗过程、分子遗传学结果和生存结果。试点生物库存储来自患者的样本,用于开发3D模型(类器官和球体)。使用机器学习和人工智能方法分析数据。结果:建立了临床数据与生物材料的结构化数据库。迄今为止,已经收集了117例胰腺(主要是PDAC)和肝外胆道肿瘤患者的详细纵向数据。该数据库记录了诊断和治疗过程以及分子遗传谱数据及其与治疗反应的关系。它是开发预测模型和生物标志物验证的基础。讨论:该项目展示了在大学医院环境中进行综合数据收集的可行性。一个主要的好处是能够监测治疗轨迹,并在临床实践中实施精确的肿瘤学原则。挑战包括能力和后勤需求,以及统一输入数据的需要。该计划有可能扩展为PDAC的国家研究基础设施。结论:已建立的基础设施为数据驱动的PDAC管理方法奠定了基础。通过整合临床数据和生物学模型,它有助于推进个性化护理,并为肿瘤学研究和决策支持提供平台。
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引用次数: 0
The role of art-making as a part of treatment in women with breast cancer. 艺术创作在女性乳腺癌治疗中的作用。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025377
E Zbiňovská, H Hobzová

Background: Comprehensive treatment of patients with cancer includes various forms of psychosocial support aimed at improving the quality of life of patients. Psychosocial interventions help manage psychological distress and physiological symptoms associated with cancer and its treatment. These interventions include art therapy, conducted by a qualified art therapist, and independent art-making, which does not require the presence of a therapist. Both interventions have proven to be effective ways to improve psychological and some physiological symptoms in patients. Current research suggests that art interventions enable patients to express and process their emotions, reduce stress, and enhance emotional well-being. Art therapy and art-making have the potential to significantly reduce symptoms of depression, anxiety, pain, and fatigue in patients. Benefits are also seen in the development of coping strategies, strengthening feelings of control, and improving interpersonal relationships. Randomized clinical trials and systematic reviews confirm positive effects on psychological resilience and overall quality of life. However, research highlights the need for higher methodological quality and addressing heterogeneity in applied art interventions.

Aim: The aim of this paper is to present current knowledge on the effects of art therapy and independent art-making on psychological and physiological symptoms in cancer patients. The work focuses on identifying the therapeutic potential of these interventions and evaluating their impact on patients' quality of life. Our intention is to contribute to a deeper understanding of the effects of art-making in the context of psycho-oncological care and to support its implementation into treatment strategies.

背景:癌症患者的综合治疗包括各种形式的社会心理支持,旨在提高患者的生活质量。心理社会干预有助于控制与癌症及其治疗相关的心理困扰和生理症状。这些干预措施包括由合格的艺术治疗师进行的艺术治疗,以及不需要治疗师在场的独立艺术创作。这两种干预措施已被证明是改善患者心理和某些生理症状的有效方法。目前的研究表明,艺术干预使患者能够表达和处理他们的情绪,减轻压力,增强情绪健康。艺术疗法和艺术创作有可能显著减轻患者的抑郁、焦虑、疼痛和疲劳症状。好处还体现在应对策略的发展、控制感的加强和人际关系的改善上。随机临床试验和系统评价证实了对心理弹性和整体生活质量的积极影响。然而,研究强调需要更高的方法质量和解决应用艺术干预的异质性。目的:本文旨在介绍艺术治疗和独立艺术创作对癌症患者心理和生理症状的影响的最新知识。这项工作的重点是确定这些干预措施的治疗潜力,并评估它们对患者生活质量的影响。我们的目的是为了更深入地了解艺术创作在心理肿瘤治疗中的作用,并支持其在治疗策略中的实施。
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引用次数: 0
Description of TEMPI syndrome in Waldenström's macroglobulinemia. Waldenström巨球蛋白血症中TEMPI综合征的描述。
Q4 Medicine Pub Date : 2025-01-01 DOI: 10.48095/ccko2025283
Z Adam, L N Leová, M Patočková, S Rajecká, S Nehyba, M Borský, J Kotašková, V Kubeš, J Kissová, J Vaníček, Z Pazdičová, Z Řehák, J Foukal, A Čermák, Z Adamová, L Zdražilová-Dubská, K Starý, I Boichuk, L Pour

Background: TEMPI syndrome (telangiectasia, erythrocytosis with increased erythropoietin, monoclonal gammopathy, perinephritic fluid collections, and intrapulmonary shunts) was described by David Sykes in 2011. By the end of March 2025, we have found descriptions of 35 cases of TEMPI syndrome in the literature. Non-IgM monoclonal gammopathy of clinical significance was diagnosed in 23 patients, multiple myeloma in 10 patients and Waldenström's macroglobulinemia in only 2 cases. Sykes estimated the median interval from the first symptoms to the diagnosis at 10 years. For many years, these patients were treated for a misdiagnosis of secondary erythrocytosis or primary polycythemia.

Observation: In 2015, an increase in hemoglobin and hematocrit concentrations was found in the patient. The diagnosis was concluded as secondary erythrocytosis in obstructive bronchopulmonary disease. Telangiectasias, which appeared soon after the detection of erythrocytosis, were misinterpreted as a manifestation of hepatopathy. The patient was treated with therapeutic phlebotomy. In 2024, symptoms of pleural effusion, ascites, swelling of the perineum and genitals appeared. This was the reason for admission to the hospital.

Results: CT scan in 2024 revealed pleural effusions, large perinephritic fluid collections and confirmed clinically evident swelling of the perineum and genitals (pelvic effusion). This gave rise to the suspicion of TEMPI syndrome. The results of morphological, flow-cytometric and molecular biological examination of the bone marrow and high concentrations of total immunoglobulin type IgM (40.6 g/L) and monoclonal immunoglobulin type IgM (23.6 g/L) with cryoglobulin properties corresponded to Waldenström's macroglobulinemia. Contrast echocardiography confirmed the existence of arteriovenous shunts, which could not be localized by other methods. Other abnormalities included very low levels of vitamin B12 and folic acid. Laboratory examination did not show significant endocrinopathy. After therapeutic plasmapheresis, treatment with rituximab, bendamustine and dexamethasone was started in December 2024.

Conclusion: In each differential diagnosis of secondary erythrocytosis, it is necessary to examine monoclonal immunoglobulin to exclude its cause in TEMPI or POEMS syndrome. A large perinephritic fluid collection is diagnostic of only two diagnoses, TEMPI syndrome or renal lymphangiomatosis, while other renal diseases can cause a smaller volume of perirenal fluid collection.

背景:David Sykes于2011年描述了TEMPI综合征(毛细血管扩张、红细胞增多伴促红细胞生成素升高、单克隆γ - γ病、肾周液收集和肺内分流)。截至2025年3月底,我们在文献中发现了35例TEMPI综合征的描述。有临床意义的非igm单克隆伽玛病23例,多发性骨髓瘤10例,Waldenström巨球蛋白血症2例。赛克斯估计,从首次出现症状到确诊的中间时间间隔为10年。多年来,这些患者被误诊为继发性红细胞增多症或原发性红细胞增多症。观察:2015年发现患者血红蛋白和红细胞压积浓度升高。诊断为阻塞性支气管肺疾病继发性红细胞增多症。在检测到红细胞增多后不久出现的毛细血管扩张被误认为是肝病的表现。病人接受治疗性静脉切开术治疗。2024年出现胸腔积液、腹水、会阴和生殖器肿胀等症状。这就是他被送进医院的原因。结果:2024年CT示胸腔积液,大量肾周积液,临床证实会阴及生殖器肿胀(盆腔积液)。这引起了对TEMPI综合症的怀疑。骨髓形态学、流式细胞术和分子生物学检查结果显示,总免疫球蛋白型IgM (40.6 g/L)和单克隆免疫球蛋白型IgM (23.6 g/L)具有低温球蛋白特性,符合Waldenström的巨球蛋白血症。超声心动图造影证实了动静脉分流的存在,其他方法无法定位。其他异常包括维生素B12和叶酸水平非常低。实验室检查未见明显内分泌病变。治疗性血浆置换后,于2024年12月开始使用利妥昔单抗、苯达莫司汀和地塞米松治疗。结论:继发性红细胞增多症在TEMPI或POEMS综合征的鉴别诊断中,均需检测单克隆免疫球蛋白以排除其病因。大量肾周积液仅可诊断为TEMPI综合征或肾淋巴管瘤病,而其他肾脏疾病可引起肾周积液量较小。
{"title":"Description of TEMPI syndrome in Waldenström's macroglobulinemia.","authors":"Z Adam, L N Leová, M Patočková, S Rajecká, S Nehyba, M Borský, J Kotašková, V Kubeš, J Kissová, J Vaníček, Z Pazdičová, Z Řehák, J Foukal, A Čermák, Z Adamová, L Zdražilová-Dubská, K Starý, I Boichuk, L Pour","doi":"10.48095/ccko2025283","DOIUrl":"https://doi.org/10.48095/ccko2025283","url":null,"abstract":"<p><strong>Background: </strong>TEMPI syndrome (telangiectasia, erythrocytosis with increased erythropoietin, monoclonal gammopathy, perinephritic fluid collections, and intrapulmonary shunts) was described by David Sykes in 2011. By the end of March 2025, we have found descriptions of 35 cases of TEMPI syndrome in the literature. Non-IgM monoclonal gammopathy of clinical significance was diagnosed in 23 patients, multiple myeloma in 10 patients and Waldenström's macroglobulinemia in only 2 cases. Sykes estimated the median interval from the first symptoms to the diagnosis at 10 years. For many years, these patients were treated for a misdiagnosis of secondary erythrocytosis or primary polycythemia.</p><p><strong>Observation: </strong>In 2015, an increase in hemoglobin and hematocrit concentrations was found in the patient. The diagnosis was concluded as secondary erythrocytosis in obstructive bronchopulmonary disease. Telangiectasias, which appeared soon after the detection of erythrocytosis, were misinterpreted as a manifestation of hepatopathy. The patient was treated with therapeutic phlebotomy. In 2024, symptoms of pleural effusion, ascites, swelling of the perineum and genitals appeared. This was the reason for admission to the hospital.</p><p><strong>Results: </strong>CT scan in 2024 revealed pleural effusions, large perinephritic fluid collections and confirmed clinically evident swelling of the perineum and genitals (pelvic effusion). This gave rise to the suspicion of TEMPI syndrome. The results of morphological, flow-cytometric and molecular biological examination of the bone marrow and high concentrations of total immunoglobulin type IgM (40.6 g/L) and monoclonal immunoglobulin type IgM (23.6 g/L) with cryoglobulin properties corresponded to Waldenström's macroglobulinemia. Contrast echocardiography confirmed the existence of arteriovenous shunts, which could not be localized by other methods. Other abnormalities included very low levels of vitamin B12 and folic acid. Laboratory examination did not show significant endocrinopathy. After therapeutic plasmapheresis, treatment with rituximab, bendamustine and dexamethasone was started in December 2024.</p><p><strong>Conclusion: </strong>In each differential diagnosis of secondary erythrocytosis, it is necessary to examine monoclonal immunoglobulin to exclude its cause in TEMPI or POEMS syndrome. A large perinephritic fluid collection is diagnostic of only two diagnoses, TEMPI syndrome or renal lymphangiomatosis, while other renal diseases can cause a smaller volume of perirenal fluid collection.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"38 4","pages":"283-301"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145081864","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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Klinicka Onkologie
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