non-Hodgkin lymphoma

非霍奇金淋巴瘤
  • 文章类型: Journal Article
    弥漫性大B细胞淋巴瘤(DLBCL)占人类免疫缺陷综合征(PLWH)患者非霍奇金淋巴瘤病例的一半。病毒血症的相互作用,免疫失调和与致癌病毒共感染在PLWH(HIV-DLBCL)中DLBCL的发病机理中起作用。这篇范围审查旨在描述HIV-DLBCL的分子景观,研究生物标志物对临床结果的影响,并描述用于表征HIV-DLBCL的技术。这篇综述包括2001年至2023年发表的32篇论文。HIV-DLBCL的样品相对较小(16-110)。队列效应影响分子特征的频率,因此它们对生存的影响尚不清楚。分子特征与HIV无关的DLBCL不同。最常评估的特征是细胞起源(81.3%的研究)。体细胞突变研究最少(占研究的6.3%)。总的来说,HIV-DLBCL中的生物标志物鉴定需要使用更强大的技术(如下一代测序)从更大或合并的样品中获得更丰富的数据。
    Diffuse large B-cell lymphoma (DLBCL) accounts for half of non-Hodgkin lymphoma cases in people living with human immunodeficiency syndrome (PLWH). The interplay of viremia, immune dysregulation and co-infection with oncogenic viruses play a role in pathogenesis of DLBCL in PLWH (HIV-DLBCL). This scoping review aimed to describe the molecular landscape of HIV-DLBCL, investigate the impact of biomarker on clinical outcomes and describe technologies used to characterise HIV-DLBCL. Thirty-two papers published between 2001 and 2023 were included in this review. Samples of HIV-DLBCL were relatively small (16-110). Cohort effects influenced frequencies of molecular characteristics hence their impact on survival was not clear. Molecular features were distinct from HIV-unrelated DLBCL. The most frequently assessed characteristic was cell of origin (81.3% of studies). Somatic mutations were the least researched (6.3% of studies). Overall, biomarker identification in HIV-DLBCL requires broader richer data from larger or pooled samples using more powerful techniques such as next-generation sequencing.
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  • 文章类型: Journal Article
    背景:非细菌性血栓性心内膜炎(NBTE)是一种罕见的疾病,其特征是心脏瓣膜上有无菌植被,通常与风湿病有关,自身免疫性疾病,和晚期实体恶性肿瘤。相关临床病症的早期诊断和治疗是强制性的,虽然它们通常不会消除瓣膜植被,抗凝对预防栓塞事件至关重要。尽管变化,由于复发性栓塞事件和原发疾病的严重程度,NBTE的预后通常是不利的,典型的晚期癌症。
    方法:我们介绍了一例57岁男性患者,他在急诊科就诊,有5天的双侧数字水肿和颜色变化发作(从苍白到发紫)的病史。体格检查显示四肢远端有红细胞增多症,提示考虑继发性雷诺综合征。尽管有药物治疗,进行性手指缺血导致多个手指截肢。在病因调查期间,抗凝试验和自身免疫分析结果为阴性.进行了经食管超声心动图检查,显示二尖瓣前小叶上有不规则的高回声肿块,无瓣膜功能障碍,胸部计算机断层扫描增强扫描显示右气管旁淋巴结肿大。气管旁淋巴结经胸穿刺活检的组织病理学分析显示弥漫性大B细胞淋巴瘤。患者接受了积极的R-CHOP化疗,实现有利的完整响应。
    结论:这是一个特殊情况,涉及NBTE和雷诺现象的发生,作为先前健康的年轻人的最初副肿瘤表现。NBTE与淋巴组织增生相关的报道非常罕见,文献中描述的病例不到10例。据我们所知,这是首例与弥漫性大B细胞淋巴瘤特异性相关的NBTE病例.
    BACKGROUND: Non-bacterial thrombotic endocarditis (NBTE) is a rare condition marked by sterile vegetations on cardiac valves, often linked to rheumatologic diseases, autoimmune disorders, and advanced solid malignancies. An early diagnosis and treatment of the associated clinical condition are mandatory, although they do not usually eliminate valvular vegetations, making anticoagulation essential to prevent embolic events. Despite variability, the prognosis of NBTE is usually unfavorable due to recurrent embolic events and the severity of the primary condition, typically advanced cancer.
    METHODS: We present a case of a 57 years-old male who presented to the emergency department with a 5-day history of painful bilateral digital edema and color change episodes (from pallor to cyanosis). Physical examination revealed erythrocyanosis in the distal extremities, prompting consideration of secondary Raynaud syndrome. Despite medical therapy, progressive digital ischemia led to multiple finger amputations. During etiological investigation, anticoagulation tests and autoimmune analysis yielded negative results. A transesophageal echocardiogram was performed, revealing an irregular hyperechogenic mass on the anterior leaflet of the mitral valve without valve dysfunction, and a thoracic computed tomography scan with contrast showed an enlarged right paratracheal lymph node. Histopathological analysis from a transthoracic needle biopsy of the paratracheal lymph node revealed diffuse large B-cell lymphoma. The patient underwent aggressive R-CHOP chemotherapy, achieving a favorable complete response.
    CONCLUSIONS: This is a particular case involving the occurrence of NBTE and Raynaud phenomenon as the initial paraneoplastic manifestations in a previously healthy young man. Reports of NBTE associated with lymphoproliferative conditions are quite rare, with fewer than ten cases described in the literature. To our knowledge, this is the first case of NBTE specifically associated with diffuse large B-cell lymphoma.
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  • 文章类型: Meta-Analysis
    皮下(SC)利妥昔单抗对非霍奇金淋巴瘤(NHL)的疗效和安全性的作用还不够明确。本研究的目的是进行系统评价和荟萃分析,评估皮下利妥昔单抗治疗NHL的疗效和安全性。
    根据PubMed中的设置搜索词进行了全面搜索,WebofScience,Embase和CochraneCENTRAL至2022年10月12日,以确定皮下利妥昔单抗治疗NHL的相关研究。疗效和安全性结果包括完全缓解(CR)加上未确认的完全缓解(CRu),不良事件(AE),≥3级不良事件,严重不良事件(SAE),给药相关反应(ARR),不良反应发生率。
    在总共758项研究中,9项试验合格。接受SC利妥昔单抗的NHL患者的CR/CRu为57%,弥漫性大B细胞淋巴瘤(DLBCL)占55%,滤泡性淋巴瘤(FL)占54%。对安全性进行的荟萃分析表明,使用SC利妥昔单抗的NHL患者的AE为85%,≥3级不良事件发生率为38%,SAE为27%,ARR为33%。结果还显示SC利妥昔单抗具有中性粒细胞减少症和恶心的高风险。
    对于NHL患者,利妥昔单抗与常规治疗的疗效无显著差异,而皮下注射可以缩短在医院的暴露时间,降低感染风险。
    UNASSIGNED: The role of subcutaneous (SC) rituximab in the efficacy and safety to non-Hodgkin lymphoma (NHL) is not clear enough. The purpose of this study was to conduct a systematic review and meta-analysis, to assess the efficacy and safety of subcutaneous rituximab to NHL.
    UNASSIGNED: A full-scale search was carried out based on the set search terms in PubMed, Web of Science, Embase and Cochrane CENTRAL until 12 October 2022 to identify relevant studies of subcutaneous rituximab for NHL. The efficacy and safety outcomes included complete response (CR) plus unconfirmed complete response (CRu), adverse events (AEs), grade ≥3 AEs, serious adverse events (SAEs), administration-related reactions (ARRs), adverse reaction rates.
    UNASSIGNED: From a total of 758 studies, 9 trials were eligible. The CR/CRu of patients with NHL receiving SC rituximab was 57%, 55% for Diffuse large B-cell lymphoma (DLBCL) and 54% for Follicular lymphoma (FL). The meta-analysis performed on safety demonstrated that AEs of NHL patients with SC rituximab was 85%, grade ≥3 AEs was 38%, SAE was 27% and ARR was 33%. The result also showed that SC rituximab had a high risk of neutropenia and nausea.
    UNASSIGNED: For NHL patients, there is no significant difference in the efficacy between subcutaneous rituximab and conventional therapy, while subcutaneous injection can shorten exposure time in the hospital and reduce the risk of infection.
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  • 文章类型: Case Reports
    非霍奇金淋巴瘤(NHL)的原发性结外罕见,非常罕见的原发性基因座之一是肾脏,即原发性肾淋巴瘤(PRL),大约是0,1-0,7%,死亡率高达75%。这里,作者分享了一名58岁的男性,他的影像学检查显示右肾上极有肿块,肝脏第六段浸润。患者接受了多学科栓塞和肾切除术。组织病理学显示肾脏和肝脏中的间变性淋巴样细胞,并导致使用CHOP方案进行6个周期的化疗。
    Primary extra-nodal of Non-Hodgkin Lymphomas (NHL) are rare, and one of the very rare primary loci is renal, namely Primary renal lymphoma (PRL), which is about 0,1-0,7%, and the mortality rate reaches as high as 75%. Here, the author shares a 58-year-old man with an imaging test indicating a mass from the upper pole of the right renal with a sixth segment of the liver infiltration. The patient underwent multidisciplinary embolization and nephrectomy. Histopathologic show the anaplastic lymphoid cells in the kidney and the hepar and lead to chemotherapy with a CHOP regimen for six cycles.
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  • 文章类型: Case Reports
    Lambert-Eaton肌无力综合征(LEMS)是一种罕见的自身免疫性疾病,其特征是针对突触前神经肌肉接头的自身抗体。它导致肌肉无力和自主神经功能障碍。LEMS可以是特发性的或与肿瘤疾病相关的,通常是小细胞肺癌。此病例报告描述了一名患有非霍奇金淋巴瘤的男性中罕见的副肿瘤LEMS。
    一名57岁的非霍奇金淋巴瘤男性,表现为进行性肌无力,反射减弱,和自主神经症状。诊断显示LEMS具有针对电压门控钙通道的自身抗体。免疫抑制治疗和淋巴瘤治疗导致他的病情显着改善。
    该病例突出了非霍奇金淋巴瘤患者中罕见的副肿瘤LEMS的发生。识别和及时管理LEMS以及淋巴瘤治疗可以导致显著的临床改善,强调需要提高对这种复杂关联的认识。
    UNASSIGNED: Lambert-Eaton myasthenia syndrome (LEMS) is a rare autoimmune disorder characterized by autoantibodies targeting presynaptic neuromuscular junctions. It results in muscle weakness and autonomic dysfunction. LEMS can be idiopathic or associated with neoplastic diseases, often small-cell lung cancer. This case report describes a rare instance of paraneoplastic LEMS in a man with non-Hodgkin lymphoma.
    UNASSIGNED: A 57-year-old male with non-Hodgkin lymphoma presented with progressive muscle weakness, diminished reflexes, and autonomic symptoms. Diagnosis revealed LEMS with autoantibodies against voltage-gated calcium channels. Immunosuppressive therapy and lymphoma treatment led to significant improvement in his condition.
    UNASSIGNED: This case highlights the rare occurrence of paraneoplastic LEMS in a patient with non-Hodgkin lymphoma. Recognition and timely management of LEMS alongside lymphoma treatment can lead to significant clinical improvement, emphasizing the need for increased awareness of such complex associations.
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  • 文章类型: Journal Article
    Polatuzumabvedotin(PV)是一种抗体-药物偶联物,在治疗弥漫性B细胞淋巴瘤(DLBCL)和高级B细胞淋巴瘤(HGBCL)中显示出有希望的结果。本摘要根据现有的临床数据总结了目前对PV在这些恶性肿瘤中使用的理解。多项临床试验已经评估了PV作为复发/难治性DLBCL和HGBCL联合治疗方案的一部分。关键的第二阶段研究,GO29365表明,与不适合干细胞移植的复发性/难治性DLBCL患者相比,PV与苯达莫司汀和利妥昔单抗(BR)的组合显着改善了无进展生存期和总生存期。随后,在这种情况下,美国食品和药物管理局加速批准了PV。PV的作用机制涉及靶向CD79b,在B细胞恶性肿瘤中表达的细胞表面受体,并将细胞毒性剂单甲基奥瑞他汀E递送至表达CD79b的细胞。这种方法增强了对癌细胞的选择性杀伤,同时保留了正常细胞。光伏的安全性通常是可控的,包括输液相关反应在内的不良事件,血细胞减少,周围神经病变,和感染。总的来说,PV已成为复发/难治性DLBCL和HGBCL患者的有价值的治疗选择,当与适当的化疗方案结合使用时,可改善结局。正在进行的研究和临床试验正在进一步探索PV在各种治疗环境中的潜力,包括一线治疗和与其他新型药物的组合。
    Polatuzumab vedotin (PV) is an antibody-drug conjugate that has shown promising results in the treatment of diffuse B-cell lymphoma (DLBCL) and high-grade B-cell lymphoma (HGBCL). This abstract summarizes the current understanding of PV\'s use in these malignancies based on available clinical data. Multiple clinical trials have evaluated PV as a part of combination therapy regimens in relapsed/refractory DLBCL and HGBCL. The pivotal Phase II study, GO29365, demonstrated that PV in combination with bendamustine and rituximab (BR) significantly improved progression-free survival and overall survival compared to BR alone in patients with relapsed/refractory DLBCL who ineligible for stem cell transplantation were. Subsequently, the US Food and Drug Administration granted accelerated approval to PV in this setting. PV\'s mechanism of action involves targeting CD79b, a cell surface receptor expressed in B-cell malignancies, and delivering the cytotoxic agent monomethyl auristatin E to CD79b-expressing cells. This approach enhances the selective killing of cancer cells while sparing normal cells. The safety profile of PV is generally manageable, with adverse events including infusion-related reactions, cytopenia, peripheral neuropathy, and infections. Overall, PV has emerged as a valuable treatment option for patients with relapsed/refractory DLBCL and HGBCL, offering improved outcomes when combined with appropriate chemotherapy regimens. Ongoing research and clinical trials are further exploring PV\'s potential in various treatment settings, including frontline therapy and in combination with other novel agents.
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  • 文章类型: Journal Article
    血清副蛋白(PP)的存在通常与浆细胞发育不良有关,Waldenstrom巨球蛋白血症/淋巴浆细胞性淋巴瘤,和冷球蛋白血症.然而,PP也经常在其他高和低级别B细胞恶性肿瘤中报道。由于这些报告稀疏且异构,缺乏对这个主题的总体看法,因此,我们进行了完整的文献回顾,以详细说明其特征,并强调与PP相关的淋巴瘤实体之间的差异和相似性。在这些设置中,IgM和IgG是常见的PP亚型,并且它们的血清浓度通常很低,甚至在没有免疫固定的情况下无法检测到。副蛋白血症及其患病率的相关性,以及IgG与IgG的影响IgMPP,在B-NHL亚型和CLL中似乎不同。尽管如此,副蛋白血症几乎总是与晚期疾病相关,以及免疫表型,遗传,和临床特征,影响预后。事实上,据报道,PP是预后不良的独立预后指标。以上要求实施临床实践,随着副蛋白血症的评估,在病人的检查中。的确,需要更多的研究来阐明导致更具侵袭性疾病的生物学机制。此外,副蛋白血症的意义,在靶向治疗的时代,应在前瞻性试验中进行评估.
    The presence of a serum paraprotein (PP) is usually associated with plasma-cell dyscrasias, Waldenstrom Macroglobulinemia/lymphoplasmacytic lymphoma, and cryoglobulinemia. However, PP is also often reported in other high- and low-grade B-cell malignancies. As these reports are sparse and heterogeneous, an overall view on this topic is lacking, Therefore, we carried out a complete literature review to detail the characteristics, and highlight differences and similarities among lymphoma entities associated with PP. In these settings, IgM and IgG are the prevalent PP subtypes, and their serum concentration is often low or even undetectable without immunofixation. The relevance of paraproteinemia and its prevalence, as well as the impact of IgG vs. IgM PP, seems to differ within B-NHL subtypes and CLL. Nonetheless, paraproteinemia is almost always associated with advanced disease, as well as with immunophenotypic, genetic, and clinical features, impacting prognosis. In fact, PP is reported as an independent prognostic marker of poor outcome. All the above call for implementing clinical practice, with the assessment of paraproteinemia, in patients\' work-up. Indeed, more studies are needed to shed light on the biological mechanism causing more aggressive disease. Furthermore, the significance of paraproteinemia, in the era of targeted therapies, should be assessed in prospective trials.
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  • 文章类型: Systematic Review
    在过去的五十年里,有报道称,在美国和全球范围内,非霍奇金淋巴瘤(NHL)的发病率和死亡率均有所上升.解决流行病学多样性的能力,NHL的预后和治疗取决于准确和一致的分类系统的使用。历史上,由于缺乏非霍奇金淋巴瘤的系统分类学,NHL的统一治疗受到阻碍.在1982年之前,有6种竞争的分类方案与NHL的术语竞争:Rappaport,Lukes-Collins,基尔,世界卫生组织,英国,和Dorfman系统没有就哪个系统在临床相关性方面最令人满意达成共识,科学的准确性和可重复性,为发病率信息的摘要者提出了一项艰巨的任务。1982年,美国国家癌症研究所赞助了一个研讨会,该研讨会开发了一种工作配方,旨在:1)为临床医生提供各种类型的NHL的预后信息,和2)提供了一种通用语言,可用于比较世界各地各种治疗中心的临床试验。研究表明,预后取决于肿瘤分期和组织学,而不是主要的定位。2本研究利用了国家癌症研究所PDQ对世界卫生组织(WHO)更新的REAL(修订后的欧美淋巴瘤)分类的适应3淋巴增殖性疾病,和SEER*Stat8.3.6数据库(2019年8月8日发布),用于1975-2016年诊断年。在这篇文章中,我们利用40年的数据来检查发病率的模式,生存和死亡,以及美国NHL的选定细胞生物行为特征。
    目的:-更新美国非霍奇金淋巴瘤的发病率和患病率趋势,检查,比较和对比生存和死亡率的短期和长期模式,并考虑NHL结节和结外细分的解剖位置的结果影响,利用选定的ICD-O-3组织学类型,按年龄分层,性别,种族/民族,舞台,细胞行为形态学和组织学类型,队列进入时间段和疾病持续时间,利用美国国家癌症研究所SEER*Stat8.3.6项目的统计数据库诊断年1975-2016.4方法。-回顾一下,基于人群的队列研究,使用国家癌症研究所(NCI)监测的全国代表性数据,流行病学,和最终结果(SEER)计划评估1975-2016年诊断年份的384,651例NHL病例,比较多个年龄变量,性别,种族,舞台,细胞行为形态学,队列进入时间段,疾病持续时间和组织学类型。分析了两个队列的相对生存统计数据:1975-1995年和1996-2016年。生存统计数据来自SEER*Stat数据库:发病率-SEER9研究数据,2018年11月提交(1975-2016年)《卡特里娜飓风/丽塔人口调整》于2019年4月发布,基于2018年11月提交。
    结果:-发病率,相对频率分布,按年龄划分的存活率和死亡率,性别,阶段和细胞行为形态,总结了1975-2016年国家癌症研究所SEER计划(SEERStat8.3.6)中记录的2个进入时间段内的成人淋巴结(N)和结外(EN)NHL的情况.识别趋势随时间的变化,这些发现与预后相关,包括短期和长期观察(实际),预期和相对生存,观察到的中位数和相对生存率,每1000人的死亡率和超额死亡率。
    结论:-SEER发病率的趋势,患病率,按年龄划分的存活率和死亡率,性别,种族,舞台,细胞行为形态学,队列进入时间段,相对频率和百分比分布,在1975-2016年的时间框架内,我们对结节性(N)和结外(EN)非霍奇金淋巴瘤提供了当前的流行病学和医学精算风险评估框架。
    During the past 5 decades, there have been reports of increases in the incidence and mortality rates of non-Hodgkin lymphoma (NHL) in the United States and globally. The ability to address the epidemiologic diversity, prognosis and treatment of NHL depends on the use of an accurate and consistent classification system. Historically, uniform treatment for NHL has been hampered by the lack of a systematic taxonomy of non-Hodgkin lymphoma. Before 1982, there were 6 competing classification schemes with contending terminologies for NHL: the Rappaport, Lukes-Collins, Kiel, World Health Organization, British, and Dorfman systems without consensus as to which system is most satisfactory regarding clinical relevance, scientific accuracy and reproducibility and presenting a difficult task for abstractors of incidence information. In 1982, the National Cancer Institute sponsored a workshop1 that developed a working formulation designed to: 1) provide clinicians with prognostic information for the various types of NHLs, and 2) provide a common language that might be used to compare clinical trials from various treatment centers around the world. Studies imply that prognosis is dependent on tumor stage and histology rather than the primary localization per se.2 This study utilizes the National Cancer Institute PDQ adaptation of the World Health Organization\'s (WHO) updated REAL (Revised European American Lymphoma) classification3 of lymphoproliferative diseases, and the SEER*Stat 8.3.6 database (released Aug 8, 2019) for diagnosis years 1975-2016. In this article, we make use of 40 years of data to examine patterns of incidence, survival and mortality, and selected cell bio-behavioral characteristics of NHL in the United States.
    OBJECTIVE: -To update trends in incidence and prevalence in the United States of non-Hodgkin lymphoma, examine, compare and contrast short and long-term patterns of survival and mortality, and consider the outcome impacts of anatomic location of NHL nodal and extranodal subdivisions, utilizing selected ICD-O-3 histologic oncotypes stratified by age, sex, race/ethnicity, stage, cell behavioral morphology and histologic typology, cohort entry time-period and disease duration, employing the statistical database of the National Cancer Institute SEER*Stat 8.3.6 program for diagnosis years 1975-2016.4 Methods.- A retrospective, population-based cohort study using nationally representative data from the National Cancer Institute\'s (NCI) Surveillance, Epidemiology, and End Results (SEER) program to evaluate 384,651 NHL cases for diagnosis years 1975-2016 comparing multiple variables of age, sex, race, stage, cell behavioral morphology, cohort entry time-period, disease duration and histologic oncotype. Relative survival statistics were analyzed in two cohorts: 1975-1995 and 1996-2016. Survival statistics were derived from SEER*Stat Database: Incidence - SEER 9 Regs Research Data, November 2018 Submission (1975-2016) released April 2019, based on the November 2018 submission.
    RESULTS: - Incidence rates, relative frequency distributions, survival and mortality by age, sex, stage and cell behavioral morphology, of adult nodal (N) and extranodal (EN) NHL in 2 entrant time-periods as recorded in the SEER Program of the National Cancer Institute for diagnosis years 1975-2016 (SEER Stat 8.3.6) are summarized. Shifts in trends over time are identified, and the findings are correlated with prognosis, including short and long-term observed (actual), expected and relative survival, median observed and relative survival, mortality rates and excess death rates per 1000 people.
    CONCLUSIONS: - Trends in SEER incidence, prevalence, survival and mortality by age, sex, race, stage, cell behavioral morphology, cohort entry time-period, relative frequency and percent distribution, were examined to provide a current epidemiologic and medical-actuarial risk assessment framework for nodal (N) and extranodal (EN) non-Hodgkin\'s lymphoma in the 1975-2016 timeframe.
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  • 文章类型: Journal Article
    喉是非霍奇金淋巴瘤(NHL)的罕见结外部位。间变性淋巴瘤激酶(ALK)阳性B细胞淋巴瘤是NHL的一种罕见且侵袭性形式。一名19岁的男性带着球状的感觉来到耳鼻喉科,声音嘶哑,颈淋巴结肿大和体重减轻。喉部70度刚性内窥镜检查显示血管,不规则,由右臂会厌褶皱引起的粘膜下肿块,导致喉气道几乎完全阻塞。PET-CT显示声门上的高代谢病变,颈部淋巴结,颈椎,肋骨和腹部淋巴结。对声门上肿块以及增大的颈部淋巴结进行活检,显示ALK阳性的大B细胞NHL。在这份报告中,我们提出了一个罕见的情况下,ALK阳性大B细胞的喉NHL,讨论它的临床,放射学和病理学特征。还介绍了有限的文献综述。有必要开发一个数据库来描述影响喉的淋巴瘤,并且该病例报告增加了对这种罕见实体的现有知识。
    Larynx is an uncommon extranodal site for non-Hodgkin lymphoma (NHL). Anaplastic lymphoma kinase (ALK)-positive B-cell lymphoma is a rare and aggressive form of NHL. A 19-year-old male presented to the ENT department with globus sensation, hoarseness, cervical lymphadenopathy and weight loss. A 70-degree rigid endoscopic examination of the larynx showed a vascular, irregular, submucosal mass arising from the right aryepiglottic fold causing near complete obstruction of the laryngeal airway. PET-CT showed hypermetabolic lesions in the supraglottis, cervical lymph nodes, cervical spine, ribs and abdominal lymph nodes. Biopsy was taken from the supraglottic mass as well as the enlarged cervical lymph nodes, which revealed ALK-positive large B-cell NHL. In this report, we present a rare case of ALK-positive large B-cell NHL of the larynx, discussing its clinical, radiological and pathological features. A limited review of literature is also presented. There is a need to develop a database for the description of lymphomas affecting the larynx and this case report adds to the existing knowledge of this rare entity.
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  • 文章类型: Case Reports
    一名72岁的中国男性出现单侧左眼全葡萄膜炎,然后通过脉络膜视网膜活检和免疫组织化学诊断为双侧T细胞原发性玻璃体视网膜淋巴瘤(T-PVRL)。诊断时未发现中枢神经系统或全身受累。尽管开始静脉和鞘内化疗和玻璃体内甲氨蝶呤,该疾病最终扩散到另一只眼睛,随后复发和全身转移。据我们所知,文献中没有报道在硅胶填充的眼睛中治疗T-PVRL的病例。T-PVRL非常罕见,大多数PVRL是恶性B细胞变体。这个案例突出了这个侵略性实体在整个治疗过程中遇到的挑战,包括在硅油填充的眼睛中给予玻璃体内甲氨蝶呤。突出了T-PVRL的低总体生存率和严峻的预后。因此,我们建议立即进行组织活检,并立即开始全身化疗和玻璃体内甲氨蝶呤.
    A 72-year-old Chinese male presented with unilateral left eye panuveitis, then diagnosed as bilateral T-cell primary vitreoretinal lymphoma (T-PVRL) through chorioretinal biopsy and immunohistochemistry. No CNS nor systemic involvement was found at diagnosis. Despite initiating intravenous and intrathecal chemotherapy and intravitreal methotrexate, the disease eventually spread to the fellow eye with subsequent recurrence and systemic metastasis. To our knowledge, no cases of T-PVRL treated in a silicone-filled eye were reported in the literature. T- PVRL is exceedingly rare, with most PVRL being the malignant B-cell variant. This case highlights the challenges encountered throughout the treatment course of this aggressive entity, including the administration of intravitreal methotrexate in a silicone oil-filled eye. The poor overall survival rate and grim prognosis of T-PVRL are highlighted. Therefore, we recommend prompt tissue biopsy and immediate initiation of systemic chemotherapy and intravitreal methotrexate.
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