Paraneoplastic disorders

  • 免疫检查点抑制剂(ICIs)是一种癌症免疫疗法,可以增强人体自身的免疫系统来治疗癌症。ICI治疗,然而,可引起免疫相关不良事件(irAE),可影响任何器官,导致显著的发病率和死亡率。神经irAEs(nirAEs)很少见,并且比中枢神经系统更常见地影响周围神经系统。治疗取决于神经系统表现的严重程度,通常包括停止ICI和开始作为一线的类固醇治疗;还使用了其他治疗方法。NirAE和心脏性irAE具有较高的致死率,其背后是早期识别和适当管理的重要性。本章回顾了与ICI治疗相关的神经免疫相关不良事件的临床表现以及诊断和治疗方式。
    Immune checkpoint inhibitors (ICIs) are cancer immunotherapies that enhance the body\'s own immune system to treat cancer. ICI treatment, however, can cause immune-related adverse events (irAEs) that can affect any organ, resulting in significant morbidity and mortality. Neurologic irAEs (nirAEs) are rare and can affect the peripheral nervous system more commonly than the central nervous system. Treatment is dependent on the severity of the neurologic manifestations and often includs discontinuation of the ICI and initiation of steroid therapy as the first line; other treatments have also been used. NirAEs and cardiac irAEs have higher fatality rates underlying the importance of early recognition and appropriate management. This chapter reviews the clinical manifestations of neurologic immune-related adverse events associated with ICI treatment as well as diagnostic and therapeutic modalities.
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  • 文章类型: Journal Article
    心血管肿瘤学是一个动态的领域。研究表明,癌症本身会损害心脏,独立于癌症治疗相关的心功能不全(CTRCD)。这项研究的目的是确定癌症中报告的心血管异常的性质,不包括CTRCD。范围审查搜索包括患有实体瘤恶性肿瘤的成年人的心血管异常,排除CTRCD和血栓事件。三个数据库(CINAHL,Embase,Medline)进行了搜索,辅以手工搜索。所有筛选和数据提取均由两名研究人员完成,并就任何冲突达成共识。鉴于所确定的研究的异质性,数据综合是叙事。搜索确定了42366项研究。在重复数据删除和标题/摘要筛选之后,对195项研究进行了全文合格性评估。44项研究包括在最终分析中。有19项前瞻性观察性研究,13项回顾性研究,9例病例报告和3个横断面研究。确定的异常类型包括心肌病(16,包括Takotsubo(9)),自主神经系统(ANS)功能障碍(10),生物标志物紊乱(9),减少心肌劳损(6)和其他(3)。由于变量研究设计,患病率尚未确定.心血管异常与发病率相关(胸痛,呼吸困难,疲劳)和缩短的预后。结论:(1)有证据表明实体瘤恶性肿瘤患者的心血管功能障碍,与CTRCD不同。患有实体瘤的人患心脏病的比率更高,即使新诊断和治疗幼稚。(2)异常主要表现为心肌病,ANS功能障碍和升高的生物标志物水平与显著症状相关。(3)治疗计划需要考虑这些风险,扩大筛选标准。
    Cardio-oncology is a dynamic field. Research has suggested that cancer itself can damage the heart, independent of cancer treatment-related cardiac dysfunction (CTRCD). The aim of this study was to establish the nature of cardiovascular abnormalities reported in cancer, excluding CTRCD. Scoping review search included cardiovascular abnormalities in adults with solid tumour malignancies, and excluded CTRCD and thrombotic events. Three databases (CINAHL, Embase, Medline) were searched, supplemented by a handsearch. All screening and data extraction was done by two researchers with consensus reached for any conflicts. Given the heterogeneous nature of the studies identified, data synthesis was narrative. The search identified 42 366 studies. Following deduplication and title/abstract screening, 195 studies were assessed for full-text eligibility. Forty-four studies are included in the final analysis. There are 19 prospective observational studies, 13 retrospective studies, 9 case reports and 3 cross-sectional studies. Types of abnormality identified include cardiomyopathy (16, including Takotsubo (9)), autonomic nervous system (ANS) dysfunction (10), biomarker disturbances (9), reduced myocardial strain (6) and others (3). Due to variable study design, the prevalence was not determined. Cardiovascular abnormalities were associated with morbidity (chest pain, dyspnoea, fatigue) and shortened prognosis. In conclusion: (1) There is evidence for cardiovascular dysfunction in patients with solid tumour malignancies, distinct from CTRCD. People with solid tumours have higher rates of cardiac disease, even when newly diagnosed and treatment naïve. (2) Abnormalities manifest mainly as cardiomyopathies, ANS dysfunction and raised biomarker levels and are associated with significant symptoms. (3) Treatment plans need to take account of these risks, and widen criteria for screening.
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  • 文章类型: Journal Article
    BACKGROUND: Paraneoplastic neurological syndromes (PNS) are rare neurological conditions and they are mostly triggered by autoimmune mechanisms. Cancer-related retinopathies (CAR) are even rarer and commonly related with breast tumor in woman. This limits our knowledge about pathophysiology of CAR. In this study, we question the association between histopathological findings and onconeural antibodies in breast cancer.
    METHODS: Thirty-two patients with newly diagnosed breast cancer admitted to the oncology outpatient clinic were included in the study. None of the participants have visual complaints. After the neurological examination of the patients, two tubes of 5 cc venous blood were obtained by screening onconeuronal antibodies. Samples were investigated in ASDETAE (İstanbul University Experimental Medicine Research Institute).
    RESULTS: Patients included in the study included one patinet (3.1%) with grade 1, 14 patients (43.8%) with grade 2 and 17 patients (53.1%) with grade 3 invasive breast cancer. Perineural invasion was detected in 5 (15.6%) patients. Progesterone receptor positivity was found in 26 (81.2%) patients and estrogen receptor positivity was found in 27 (84.4%) patients. In 7 (21.9%) patients, CERBB2 was positive and in 25 (78.1%) patients, Ki 67 was positive. A total of 12 (37.5%) patients had onconeuroneal antibody positivity. Antibody positivity was significantly higher in patients with high grade tumor (p=0.008).
    CONCLUSIONS: There may be a relationship between tumor grade and the presence of onconeuronal antibodies in breast cancer patients. By the detection of new biochemical markers, significant contribution can be made to the early diagnosis and treatment of underlying cancer.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    我们介绍了两名在每种情况下都患有经典副肿瘤综合征并伴有多种中枢神经系统(CNS)自身抗体的患者。多种抗体的存在使检测到恶性肿瘤的可能性更大,并且两名患者随后均被诊断为小细胞肺癌(SCLC)。我们强调,多种CNS自身抗体的存在增加了检测恶性肿瘤的可能性,但是尽管抗体谱相似,临床表现和对治疗的反应可能会有所不同。临床医生应警惕有多种中枢神经系统自身抗体的患者需要寻找隐匿性恶性肿瘤。
    We present two patients who presented with classical paraneoplastic syndromes with multiple central nervous system (CNS) autoantibodies in each case. The presence of multiple antibodies made the detection of a malignancy more likely and both patients were subsequently diagnosed with small cell lung carcinoma (SCLC). We highlight that the presence of multiple CNS autoantibodies increases the likelihood of detecting a malignancy but that the clinical presentation and response to treatment can vary despite similar antibody profiles. Clinicians should be alert to the need to search for occult malignancy in patients with multiple CNS autoantibodies.
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  • 文章类型: Journal Article
    此病例介绍描述了一名患有转移性恶性胰岛素瘤的65岁女性,她经历了频繁的低血糖发作,给她的生活质量带来了巨大的痛苦和负担。该报告描述了如何改变医疗管理以及如何为患者提供支持以减轻这些负担。特别是,该报告强调了在胰岛素瘤中首次使用闪光葡萄糖监测的记录,这有利于减少使用柳叶刀的不适和与低血糖发作相关的焦虑。这个案例展示了个性化方法的好处,与其他专业的合作,并建议进一步评估闪光血糖监测在姑息治疗中的应用。
    This case presentation describes a 65-year-old woman with metastatic malignant insulinoma who experienced frequent episodes of hypoglycaemia causing significant distress and burden to her quality of life. The report describes how medical management was altered and how the patient was supported in order to alleviate these burdens. In particular, the report highlights the first documented use of flash glucose monitoring in insulinoma, which was beneficial in reducing the discomfort of lancet use and anxiety associated with hypoglycaemic episodes. This case demonstrates the benefits of an individualised approach, collaboration with other specialisms and recommends further evaluation of the use of flash glucose monitoring in palliative care.
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  • 文章类型: Journal Article
    For many laboratories, autoimmune encephalopathy (AE) panels are send-out tests. These tests are expensive, and ordering patterns vary greatly. There is also a lack of consensus on which panel to order and poor understanding of the clinical utility of these panels. These challenges were presented to our newly formed, multidisciplinary, diagnostic stewardship committee (DSC). Through this collaboration, we developed an algorithm for ordering AE panels; combining diagnostic criteria with practice guidelines.
    We analyzed test-ordering patterns in 2018 and calculated a true-positive rate based on clinical presentation and panel interpretation. An evidence-based approach was combined with input from the Department of Neurology to synthesize our algorithm. Efficacy of the algorithm (number of panels ordered, cost, and true positives) was assessed before and after implementation.
    In 2018, 77 AE-related panels were ordered, costing $137 510. The true-positive rate was 10%, although ordering multiple, similar panels for the same patient was common. Before implementing the algorithm (January 1-July 31, 2019), 55 panels were ordered, costing $105 120. The total true-positive rate was 3.6%. After implementation, 23 tests were ordered in a 5-month period, totaling $50 220. The true-positive rate was 13%.
    With the DSC-directed mandate, we developed an algorithm for ordering AE panels. Comparison of pre- and postimplementation data showed a higher true-positive rate, indicating that our algorithm was able to successfully identify the at-risk population for AE disorders. This was met with a 43% decrease in the number of tests ordered, with total cost savings of $25 000 over 5 months.
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  • 文章类型: Case Reports
    Eosinophilic dermatosis of hematological malignancy is a paraneoplastic skin eruption associated with chronic lymphocytic leukemia and other B-cell malignancies. It clinically resembles an insect bite reaction and it can precede the symptoms of the hematological malignancy or be related to a more aggressive course. Different treatments have been proposed, but partial response and recurrence are frequent. Herein, we describe a case of eosinophilic dermatosis associated with mantle cell lymphoma with remission after lenalidomide therapy.
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  • 文章类型: Case Reports
    We describe the first case of myasthenia gravis as a possible paraneoplastic manifestation of ovarian cancer preceding its diagnosis.
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  • 文章类型: Journal Article
    In 208 children with opsoclonus-myoclonus syndrome (OMS), CSF IgG oligoclonal bands (OCB) and 22 immunomarkers in CSF and 21 in serum/blood were measured. In 36 untreated OMS, 58% were OCB(+), whereas 55% of treated OMS were OCB(-). OCB positivity or negativity did not alter concentrations or frequencies of immunomarkers. The phenotypes of OCB(+) and OCB(-) patients were not distinctive. CSF B cells were expanded in untreated OMS regardless of OCB positivity. These data reveal a much higher frequency of OCB positivity in untreated OMS than previously realized and a disconnect between intrathecal OCB and inflammatory mediator production.
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