Hodgkin's lymphoma

霍奇金淋巴瘤
  • 文章类型: Journal Article
    大约10%-30%的霍奇金淋巴瘤(HL)患者在一线标准治疗后经历复发或难治性(R/R)疾病。Brentuximabvedotin(BV)和免疫检查点抑制剂(ICIs)在R/RHL的挽救治疗中具有重要作用。然而,BV和/或ICI治疗难治性HL的后续治疗具有挑战性.
    我们回顾性分析了两个机构的R/RHL患者,经历过BV或ICI治疗失败,此后接受放疗(RT)。总反应率(ORR),响应持续时间(DOR),无进展生存期(PFS),分析总生存期(OS)。
    总的来说,纳入19例患者。一线全身治疗包括阿霉素,博来霉素,长春碱,和达卡巴嗪(ABVD,84.2%);AVD加ICIs(10.5%);和博来霉素,依托泊苷,阿霉素,环磷酰胺,长春新碱,丙卡巴嗪,和泼尼松(BEACOPP,5.3%)。一线治疗后,15例(78.9%)和4例(21.1%)患者患有难治性疾病和复发,分别。R/RHL诊断后,六(31.6%),两个(10.5%),11例(57.9%)患者同时接受BV和ICIs,BV单药治疗,和ICI单一疗法,分别。所有患者均接受强度调节RT(n=12,63.2%)或体积调节电弧治疗(VMAT;n=7,36.8%)。ORR和完全缓解(CR)率为100%;DOR至RT的中位数为17.2个月(范围,7.9-46.7个月)。两名患者在辐射领域外表现出进展;一名患者在领域内广泛,场外,节点,结外复发.中位随访时间为16.2个月(范围,9.2-23.2个月),1年PFS和OS分别为84.4%和100%,分别。PFS与结外受累(P=0.019)和大体肿瘤体积(P=0.044)相关。所有患者耐受RT良好,无≥3级不良事件。
    RT对于治疗BV或ICIs难治性HL是有效且安全的,并且有可能成为HL综合策略的一部分。
    UNASSIGNED: Approximately 10%-30% of patients with Hodgkin\'s lymphoma (HL) experience relapse or refractory (R/R) disease after first-line standard therapy. Brentuximab vedotin (BV) and immune checkpoint inhibitors (ICIs) have important roles in the salvage treatment of R/R HL. However, subsequent treatment for HL refractory to BV and/or ICI treatment is challenging.
    UNASSIGNED: We retrospectively analyzed patients in two institutions who had R/R HL, experienced BV or ICI treatment failure, and received radiotherapy (RT) thereafter. The overall response rate (ORR), duration of response (DOR), progression-free survival (PFS), and overall survival (OS) were analyzed.
    UNASSIGNED: Overall, 19 patients were enrolled. First-line systemic therapy comprised doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD, 84.2%); AVD plus ICIs (10.5%); and bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone (BEACOPP, 5.3%). After first-line therapy, 15 (78.9%) and four patients (21.1%) had refractory disease and relapsed, respectively. After R/R HL diagnosis, six (31.6%), two (10.5%), and 11 (57.9%) patients received BV and ICIs concurrently, BV monotherapy, and ICI monotherapy, respectively. All patients received intensity-modulated RT (n = 12, 63.2%) or volumetric modulated arc therapy (VMAT; n = 7, 36.8%). The ORR as well as the complete response (CR) rate was 100%; the median DOR to RT was 17.2 months (range, 7.9-46.7 months). Two patients showed progression outside the radiation field; one patient had extensive in-field, out-of-field, nodal, and extranodal relapse. With a median follow-up time of 16.2 months (range, 9.2-23.2 months), the 1-year PFS and OS were 84.4% and 100%, respectively. PFS was associated with extranodal involvement (P = 0.019) and gross tumor volume (P = 0.044). All patients tolerated RT well without adverse events of grade ≥ 3.
    UNASSIGNED: RT is effective and safe for treating HL refractory to BV or ICIs and has the potential to be part of a comprehensive strategy for HL.
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  • 文章类型: Journal Article
    背景:经典霍奇金淋巴瘤(HL)是一种独特的恶性生长,具有良好的初始预后。然而,10-30%的患者在缓解后仍会复发。已经成为肿瘤进展焦点的一种主要细胞功能是自噬。该过程可以在压力条件下保持细胞稳态。多项研究表明,自噬可能在HL的发展中起作用。因此,本综述旨在探讨化疗对HL细胞自噬的影响,以及自噬对HL的影响。
    方法:进行了与已发布的PRISMA扩展范围审查(PRISMA-ScR)相一致的范围审查。在MEDLINE数据库和Cochrane中央对照试验注册中心(CENTRAL)上进行了文献检索。对所有结果进行检索和筛选,并对所得文章进行了叙述合成。
    结果:结果显示,某些癌症化疗也会诱导自噬通量。虽然HL上的数据有限,由于这些药物的作用机制相似,我们可以推断出类似的关系。然而,这种自噬活性的增加可能反映了增加肿瘤生长的机制或细胞补偿以抑制其生长。尽管有证据支持这两种观点,我们认为自噬允许癌细胞抵抗细胞死亡,主要是由于细胞毒性药物引起的DNA损伤。
    结论:自噬反映了细胞对生存的适应,并解释了为什么化疗通常会诱导自噬功能。然而,自噬抑制需要进一步研究,因为它提出了可行的治疗策略,特别是针对HL化疗方案可能产生的耐药人群。
    BACKGROUND: Classical Hodgkin Lymphomas (HL) are a unique malignant growth with an excellent initial prognosis. However, 10-30% of patients will still relapse after remission. One primary cellular function that has been the focus of tumor progression is autophagy. This process can preserve cellular homeostasis under stressful conditions. Several studies have shown that autophagy may play a role in developing HL. Therefore, this review aimed to explore chemotherapy\'s effect on autophagy in HL, and the effects of autophagy on HL.
    METHODS: A scoping review in line with the published PRISMA extension for scoping reviews (PRISMA-ScR) was conducted. A literature search was conducted on the MEDLINE database and the Cochrane Central Register of Controlled Trials (CENTRAL). All results were retrieved and screened, and the resulting articles were synthesized narratively.
    RESULTS: The results showed that some cancer chemotherapy also induces autophagic flux. Although the data on HL is limited, since the mechanisms of action of these drugs are similar, we can infer a similar relationship. However, this increased autophagy activity may reflect a mechanism for increasing tumor growth or a cellular compensation to inhibit its growth. Although evidence supports both views, we argued that autophagy allowed cancer cells to resist cell death, mainly due to DNA damage caused by cytotoxic drugs.
    CONCLUSIONS: Autophagy reflects the cell\'s adaptation to survive and explains why chemotherapy generally induces autophagy functions. However, further research on autophagy inhibition is needed as it presents a viable treatment strategy, especially against drug-resistant populations that may arise from HL chemotherapy regimens.
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  • 文章类型: Case Reports
    霍奇金淋巴瘤是一种B细胞肿瘤,通常表现为在数周至数月内逐渐进展的淋巴结病。然而,我们介绍了一例霍奇金淋巴瘤的特殊病例,其特征是在一小时内淋巴结病异常迅速发展。一名30岁的男性出现左颈肿胀,在一个小时内发生,然后在三天内保持稳定,促使一项调查显示广泛的淋巴结病与霍奇金淋巴瘤一致。该病例概述了及时识别和调查霍奇金淋巴瘤异常表现的重要性,强调加快诊断和干预的必要性。
    Hodgkin\'s lymphoma is a B-cell neoplasm that typically manifests with gradual lymphadenopathy progression over weeks to months. However, we present an exceptional case of Hodgkin\'s lymphoma marked by an unusually rapid development of lymphadenopathy within an hour. A 30-year-old male presented with a left neck swelling that occurred within an hour and then remained stable in size for three days, prompting an investigation revealing widespread lymphadenopathy consistent with Hodgkin\'s lymphoma. This case outlines the importance of recognizing and investigating unusual presentations of Hodgkin\'s lymphoma promptly, emphasizing the necessity for expedited diagnosis and intervention.
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  • 文章类型: Case Reports
    噬血细胞淋巴组织细胞增生症是导致免疫系统过度激活的炎性病症。它可能是零星的或家族的。家族亚型与各种遗传突变有关,通常是年轻人的疾病。这里我们报道了一个成人的HLH病例,发生在成功治疗的血液恶性肿瘤的背景下。在工作中,他还被发现有致病性STXBP2突变,提示家族起源的HLH。迄今为止,只有少数成人发病的家族性HLH病例被揭露。
    Hemophagocytic Lymphohistiocytosis is an inflammatory condition which results in over activation of the immune system. It could be either sporadic or familial. The familial subtype is linked with various genetic mutations and is commonly a disease of the young. Here we report a case of HLH in an adult, occurring in the background of a successfully treated hematological malignancy. Upon workup, he was also found to have pathogenic STXBP2 mutation, suggesting HLH of familial origin. To date, only few cases of adult-onset familial HLH have been brought to light.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    文章“博来霉素缺陷对霍奇金淋巴瘤患者生存的影响:一项回顾性研究”在巴西的一个中心介绍了新诊断的霍奇金淋巴瘤(HL)的AVD化疗方案的经验。虽然是一项小型的回顾性研究,这项研究的结果为医学界提供了巴西HL的真实数据。在早期和晚期,ABVD仍然是新诊断HL患者的护理标准。较新的靶向分子也已用于与现有药物的新型组合。然而,在由于2017年巴西供应不足而暂时缺乏博来霉素的情况下,导致使用不完全的ABVD方案,没有博来霉素,即HL的AVD。然而,Soldi等人。利用机会回顾性研究是否省略博来霉素导致低于正常的治疗或不必要的效果。
    The article \"The impact of bleomycin deficit on survival in Hodgkin\'s lymphoma patients: A retrospective study\" have presented the experience of AVD chemotherapy regimen in newly diagnosed Hodgkin\'s lymphoma (HL) in a single center in Brazil. Though being a small retrospective study, results from this study have provided the medical community a real-world data on HL in Brazil. ABVD has remained the standard of care for patients of newly diagnosed HL both in early and advance stages. Newer targeted molecules have also come for use in novel combinations with existing drugs. However, in a situation of temporary scarcity of bleomycin due to lack of supply during 2017 in Brazil led to use of incomplete ABVD regimen without bleomycin, i.e. AVD for HL. However, Soldi et al. utilized the opportunity to retrospectively study if the omission of bleomycin leads to subnormal treatment or unwarranted effects.
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  • 文章类型: Journal Article
    淋巴瘤的瘙痒通常与霍奇金淋巴瘤(HL)和血管免疫母细胞T细胞淋巴瘤(AITL)有关,并严重影响患者的生活质量。最近的证据表明,致痒细胞因子似乎在慢性疾病的发生中起着重要作用。本研究旨在探讨淋巴瘤患者瘙痒相关的细胞因子,为潜在的治疗靶点提供依据。收集了60名淋巴瘤患者的血清样本,包括47例霍奇金淋巴瘤(HL)和13例血管免疫母细胞性T细胞淋巴瘤(AITL),作为观察组(淋巴瘤组,LP组,n=60)。此外,来自8名健康供体的血清样本(HD组,收集n=8)用于比较。在淋巴瘤组中,患者被分层为瘙痒患者(LWP组,n=30)和无瘙痒的(LWOP组,n=30)基于皮肤瘙痒症状的存在。与HD组相比,LP组中观察到多种细胞因子水平显著升高(p<0.01)。LWP组患者血清IL-31水平较高(p<0.001),IL-1β(P=0.039),和IL-1α(P=0.037)与LWOP组比较。值得注意的是,晚期AITL患者(IV期)的血清IL-31水平高于早期AITL患者(I-III期,P<0.05)。在亚组分析中,AITL组瘙痒患者血清MIG和CTACK水平高于HL组,而PDGF-BB水平显着降低(p<0.05)。血清IL-31、IL-1β水平升高,和IL-1α与淋巴瘤相关的瘙痒有关。还强调了HL和AITL亚组之间的血清细胞因子谱的差异。这些发现为治疗淋巴瘤相关瘙痒的临床干预提供了有价值的见解。
    Pruritus of lymphoma is commonly associated with both Hodgkin lymphoma (HL) and angioimmunoblastic T cell lymphoma (AITL) and critically affects the life quality of patient. Recent evidence suggests that the pruritogenic cytokines seem to play a significant role in the genesis of chronic. This study aims to investigate the cytokines associated with itching in lymphoma patients and provide the basis for potential therapeutic targets. Serum samples were collected from 60 lymphoma patients, including 47 with Hodgkin lymphoma (HL) and 13 with angioimmunoblastic T-cell lymphoma (AITL), serving as the observation group (lymphoma group, LP group, n = 60). Additionally, serum samples from 8 healthy donors (HD group, n = 8) were collected for comparison. Within the lymphoma group, patients were stratified into those with pruritus (LWP group, n = 30) and those without pruritus (LWOP group, n = 30) based on the presence of skin pruritus symptoms. Elevated levels of multiple cytokines were significantly observed in the LP group in comparison to the HD group (p < 0.01). Patients in LWP group exhibited higher serum levels of IL-31 (p < 0.001), IL-1β (P = 0.039), and IL-1α (P = 0.037) compared to LWOP group. Notably, serum IL-31 levels were higher in advanced AITL patients (stage IV) than in early AITL patients (stage I-Ⅲ, P < 0.05). In subgroup analysis, patients with pruritus in the AITL group exhibited higher serum levels of MIG and CTACK compared to HL group, whereas PDGF-BB levels were significantly lower (p < 0.05). Elevated serum levels of IL-31, IL-1β, and IL-1α are linked to lymphoma-associated pruritus. Differences in serum cytokine profiles between HL and AITL subgroups are also highlighted. These findings offer valuable insights for clinical intervention in managing lymphoma-related pruritus.
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  • 文章类型: Journal Article
    目的:评估C反应蛋白(CRP)的预后价值,在诊断和随访期间,在圣塔卡萨德圣保罗医院血液科治疗的霍奇金淋巴瘤患者,并将血清CRP水平与疾病分期和治疗反应相关联。
    方法:对2012年2月至2016年1月间诊断为霍奇金淋巴瘤的71例病历进行回顾性研究。随后排除了三名患者,共68例患者进行分析。CRP水平>1mg/dl被认为是升高的。
    结果:患者主要为男性(61.8%),平均年龄为34岁。53例(78%)患者晚期,(76.5%)有B症状。基线CRP升高与B症状(p=0.02)和晚期(p=0.015)的可能性更大相关。第5周期和第6周期化疗后CRP水平低的患者与完全缓解相关(分别为p=0.04和p=0.03)。治疗难治性患者的死亡风险更高(p=0.002)。
    结论:CRP对霍奇金淋巴瘤患者的随访具有重要的临床意义,其中高水平与晚期疾病和/或B症状的存在相关。CRP水平被认为是治疗反应的预测因子。治疗期间持续存在高CRP值与难治性相关。
    OBJECTIVE: To assess the prognostic value of C-Reactive Protein (CRP), at diagnosis and during follow-up, of patients with Hodgkin´s Lymphoma treated at the Hematology Service of the Santa Casa de São Paulo Hospital, and to correlate serum CRP levels with disease stage and treatment response.
    METHODS: A retrospective study involving review of 71 medical records of patients diagnosed with Hodgkin´s Lymphoma between February 2012 and January 2016 was performed. Three patients were subsequently excluded, giving a total of 68 patients for analysis. A level of CRP > 1mg/dl was considered elevated.
    RESULTS: Patients were predominantly male (61.8%) and mean age was 34 years. Fifty-three (78%) patients had advanced stage and (76.5%) had B symptoms. Elevated baseline CRP was associated with greater likelihood of B symptoms (p= 0.02) and of advanced stage (p= 0.015). Patients with Low CRP level after 5th and 6th cycles of chemotherapy was associated with complete response (p=0.04 and p=0.03, respectively). Treatment-refractory patients had greater risk of death (p=0.002).
    CONCLUSIONS: CRP is clinically important for follow-up of patients with Hodgkin´s Lymphoma, where high levels were associated with advanced disease and/or presence of B symptoms. CRP level was considered a predictor of treatment response. Persistence of high CRP values during treatment was associated with refractoriness.
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  • 文章类型: Journal Article
    目的:评估C反应蛋白(CRP)的预后价值,在诊断和随访期间,在圣塔卡萨德圣保罗医院血液科治疗的霍奇金淋巴瘤患者,并将血清CRP水平与疾病分期和治疗反应相关联。
    方法:对2012年2月至2016年1月间诊断为霍奇金淋巴瘤的71例病历进行回顾性研究。随后排除了三名患者,共68例患者进行分析。CRP水平>1mg/dl被认为是升高的。
    结果:患者主要为男性(61.8%),平均年龄为34岁。53例(78%)患者晚期,(76.5%)有B症状。基线CRP升高与B症状(p=0.02)和晚期(p=0.015)的可能性更大相关。第5周期和第6周期化疗后CRP水平低的患者与完全缓解相关(分别为p=0.04和p=0.03)。治疗难治性患者的死亡风险更高(p=0.002)。
    结论:CRP对霍奇金淋巴瘤患者的随访具有重要的临床意义,其中高水平与晚期疾病和/或B症状的存在相关。CRP水平被认为是治疗反应的预测因子。治疗期间持续存在高CRP值与难治性相关。
    OBJECTIVE: To assess the prognostic value of C-Reactive Protein (CRP), at diagnosis and during follow-up, of patients with Hodgkin´s Lymphoma treated at the Hematology Service of the Santa Casa de São Paulo Hospital, and to correlate serum CRP levels with disease stage and treatment response.
    METHODS: A retrospective study involving review of 71 medical records of patients diagnosed with Hodgkin´s Lymphoma between February 2012 and January 2016 was performed. Three patients were subsequently excluded, giving a total of 68 patients for analysis. A level of CRP > 1 mg/dl was considered elevated.
    RESULTS: Patients were predominantly male (61.8 %) and mean age was 34 years. Fifty-three (78 %) patients had advanced stage and (76.5 %) had B symptoms. Elevated baseline CRP was associated with greater likelihood of B symptoms (p = 0.02) and of advanced stage (p = 0.015). Patients with Low CRP level after 5th and 6th cycles of chemotherapy was associated with complete response (p = 0.04 and p = 0.03, respectively). Treatment-refractory patients had greater risk of death (p = 0.002).
    CONCLUSIONS: CRP is clinically important for follow-up of patients with Hodgkin´s Lymphoma, where high levels were associated with advanced disease and/or presence of B symptoms. CRP level was considered a predictor of treatment response. Persistence of high CRP values during treatment was associated with refractoriness.
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  • 文章类型: Journal Article
    霍奇金淋巴瘤(HL)治疗的现代焦点涉及预防继发性器官损伤。尽管放射治疗领域合理化,许多患者仍发展为晚期放射相关心脏毒性,且严重,需要介入治疗.目前尚无指南指导这些复杂患者的管理,这些患者通常患有多种并发心脏病变。尽管拥有比一般人群更大的死亡风险,心脏手术在治疗辐射相关性心脏病中具有重要作用。这篇综述总结了放射治疗后HL幸存者心脏手术的相关文献,强调这一群体特有的好处和风险。还探讨了与HL患者相关的放射相关心脏病的病理生理学和表现。
    Much of the modern focus of Hodgkin\'s Lymphoma (HL) treatment involves the prevention of secondary organ injury. Despite rationalisations of radiotherapy fields, many patients still develop late radiation-related cardiotoxicity that is severe and requires interventional management. No guidelines exist to direct management of these complex patients who often present with multiple concurrent cardiac pathologies. Despite possessing a greater mortality risk than in the general population, cardiac surgery has an important role in treating radiation-associated heart disease. This review summarises the body of literature surrounding cardiac surgery in HL survivors post-radiotherapy, highlighting the benefits and risks unique to this cohort. The pathophysiology and presentation of radiation-associated heart disease is also explored in relation to HL patients.
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