Mycosis fungoides

真菌病真菌
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  • 文章类型: Journal Article
    背景:由于大多数T细胞淋巴瘤缺乏CD20表达,表现出异常CD20表达的真菌病(MF)病例非常罕见。
    目的:我们的目标是全面评估临床,组织病理学,7例CD20阳性MF患者的预后特征。
    方法:这项回顾性研究检查了7例CD20异常表达的MF。该研究提供了人口统计的细节,临床特征,组织病理学和治疗结果。关键时间点包括MF的初始诊断,CD20表达的检测和随访,平均随访46个月。
    结果:异常CD20阳性MF的诊断平均年龄为58.6岁,首次MF诊断后约5.6年。CD20检测后,患者表现为晚期疾病阶段,需要化疗等治疗,Brentuximabvedotin,和异基因造血干细胞移植。四名病人死于淋巴瘤,平均生存时间为52个月。
    结论:MF中CD20异常表达罕见,但提示进展过程与不良预后相关。这通常需要全身化疗,在某些情况下,异基因造血干细胞移植。这项研究提供了对临床属性的重要见解,疾病进展,以及CD20表达异常的MF患者的治疗选择。需要进一步的研究来验证新兴疗法的有效性,并增强我们对这一独特MF亚组特有的潜在机制和预后决定因素的理解。
    BACKGROUND: As the majority of T-cell lymphomas lack CD20 expression, cases of mycosis fungoides (MF) exhibiting aberrant CD20 expression are exceedingly uncommon.
    OBJECTIVE: We aim to comprehensively evaluate the clinical, histopathological, and prognostic features of 7 patients diagnosed with CD20-positive MF.
    METHODS: This retrospective study examines seven cases of MF with aberrant CD20 expression. The study provides details of demographics, clinical features, histopathology and treatment outcomes. Key time points include initial diagnosis of MF, detection of CD20 expression and follow-up, with a mean follow-up of 46 months.
    RESULTS: Aberrant CD20-positive MF was diagnosed at an average age of 58.6 years, approximately 5.6 years after first MF diagnosis. Following CD20 detection, patients presented with advanced disease stages, requiring treatments such as chemotherapy, brentuximab vedotin, and allogeneic hematopoietic stem cell transplantation. Four patients died from lymphoma, with an average survival time of 52 months.
    CONCLUSIONS: Aberrant CD20 expression in MF is rare but indicates a progressive course associated with poor prognosis. This often requires systemic chemotherapy and, in certain instances, allogeneic hematopoietic stem cell transplantation. This study provides important insights into the clinical attributes, disease progression, and treatment options for MF patients with aberrant CD20 expression. Further research is necessary to validate the effectiveness of emerging therapies and enhance our understanding of the underlying mechanisms and prognostic determinants specific to this unique MF subgroup.
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  • 文章类型: Journal Article
    背景:真菌病(MF)和Sezary综合征(SS)占所有皮肤T细胞淋巴瘤诊断的一半以上。当前的风险分层主要基于TNMB分期,很少有研究调查临床检查的预后价值。目前晚期疾病的全身治疗包括免疫调节药物,化疗,和HADC抑制剂。很少有临床试验或回顾性研究比较不同药物的疗效。方法:在这里,我们对2013-2023年在北京协和医院诊断为MF/SS的92例患者的预后因素和治疗结果进行了回顾性分析.结果:Cox回归分析确定年龄≥50岁,白细胞≥8×109/L,血清LDH≥250U/L,β2-MG≥4.50mg/L,IV期与总生存率降低有关,年龄≥50岁,血清LDH≥250U/L和IV期与无进展生存期降低相关.Kaplan-Meier分析确定免疫调节治疗与更长的无进展生存期相关。结论:这些结果为预测晚期MF/SS患者的预后和选择适当的治疗方法提供了新的因素。
    Background: Mycosis fungoides (MF) and Sezary Syndrome (SS) comprise over half of all Cutaneous T-cell lymphoma diagnoses. Current risk stratification is largely based on TNMB staging, few research investigated the prognostic value of clinical exams. Current systemic therapy for advanced disease includes immunomodulatory drugs, chemotherapy, and HADC inhibitors. Few clinical trials or retrospective research compared the efficacy of different drugs.Method: Here, we performed a retrospective analysis of prognostic factors and treatment outcomes of 92 patients diagnosed with MF/SS at the Peking Union Medical College Hospital from 2013-2023.Results: Cox regression analysis identified that age ≥ 50 years, WBC ≥ 8 × 109/L, serum LDH ≥ 250U/L, β2-MG ≥ 4.50 mg/L, and stage IV were associated with reduced overall survival, age ≥ 50 years, serum LDH ≥ 250U/L and stage IV were associated with reduced progression free survival. Kaplan-Meier analysis established that immunomodulatory therapy was associated with longer progression free survival.Conclusion: These results suggested new factors in predicting prognosis and selecting appropriate treatments in patients with advanced MF/SS.
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  • 文章类型: Journal Article
    原发性皮肤淋巴瘤(PCLs),尤其是霉菌病(MF),构成重大的诊断和治疗挑战。这项研究旨在将MF患者的初始组织学特征与病程和生存率相关联。对83例诊断为早期MF的患者进行了一项回顾性前瞻性队列研究,萨格勒布UHC,2003年1月至2012年12月。分析的组织病理学参数包括苔藓样真皮淋巴细胞浸润,Pautrier微脓肿,淋巴细胞异型性.每100个角质形成细胞具有超过30个监护淋巴细胞的患者表现出较差的总体和无进展生存期。此外,那些超过50%的非典型淋巴细胞表现出更快的进展速度。密集的苔藓样皮肤浸润和大量淋巴细胞“饲养者”在诊断后五年内显着增加了死亡风险。这项研究并未完全证实有关大型Pautrier微脓肿的预后价值的假设,但强调了致密苔藓样浸润的重要性。该研究确定了早期MF的新的潜在组织病理学预后因素,这表明需要更大规模的研究来证实这些发现。此类预测因子的识别可以增强预后分层并指导针对MF患者的更量身定制的治疗方法。
    Primary cutaneous lymphomas (PCLs), especially mycosis fungoides (MF), pose significant diagnostic and therapeutic challenges. This study aims to correlate initial histological features with the disease course and survival in MF patients. A retrospective-prospective cohort study was conducted on 83 patients diagnosed with early-stage MF at the Departments of Dermatovenerology and Pathology, UHC Zagreb, from January 2003 to December 2012. The analyzed histopathological parameters included lichenoid dermal lymphocyte infiltrate, Pautrier microabscesses, and lymphocyte atypia. Patients with more than 30 guardian lymphocytes per 100 keratinocytes exhibited worse overall and progression-free survival. Furthermore, those with over 50% atypical lymphocytes demonstrated a faster progression rate. A dense lichenoid dermal infiltrate and a high count of lymphocyte \"keepers\" significantly increased the mortality risk within five years of diagnosis. This study did not fully confirm the hypothesis regarding the prognostic value of large Pautrier microabscesses but highlighted the importance of dense lichenoid infiltrates. The study identified new potential histopathological prognostic factors in early-stage MF, suggesting the need for larger studies to confirm these findings. The identification of such predictors could enhance the prognostic stratification and guide more tailored therapeutic approaches for MF patients.
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  • 文章类型: Journal Article
    Brentuximabvedotin(BV),抗CD30抗体和单甲基奥瑞他汀E的缀合物,已成为难治性CD30真菌病(MF)和原发性皮肤间变性大细胞淋巴瘤(pcALCL)的有希望的治疗选择。BV已被证明是安全和有效的治疗霍奇金淋巴瘤和外周T细胞淋巴瘤。这个多中心,prospective,单臂I/II期研究评估了BV在日本CD30+皮肤淋巴瘤患者中的疗效,即CD30+皮肤T细胞淋巴瘤。参与者分为两组:CD30MF或pcALCL患者(队列1,n=13)和除队列1以外的CD30淋巴增生性疾病患者(队列2,n=3)。研究人群包括完整的分析集(FAS),改进的FAS(mFAS),和安全分析集(SAF)。在队列1和1+2中鉴定这些组,分别标记为FAS1和FAS2、mFAS1和mFAS2以及SAF1和SAF2。每个治疗周期持续3周,根据治疗反应,在第三个周期后,BV持续多达16个周期。主要终点是独立审查论坛(IRF)确定的4个月客观缓解率(ORR4)。FAS1的ORR4为69.2%,FAS2为62.5%(P<0.0001)。ORR的次要终点,使用全球反应评分(FAS1中为53.8%)和改良的严重性加权评估工具(FAS1中为62.5%)进行评估,使用IRF,提供了与主要发现相当的结果。SAF1中≥3级不良事件(≥15%)的发生率为3例患者(23%)的周围神经病变和2例患者(15%)的发热和嗜酸性粒细胞增多。总之,BV显示出良好的疗效,耐受性,日本复发性或难治性CD30+原发性皮肤T细胞淋巴瘤患者的安全性和安全性。该试验已在大学医院医学信息网络临床试验登记处注册,日本(协议ID:UMIN000034205)。
    Brentuximab vedotin (BV), a conjugate of anti-CD30 antibody and monomethyl auristatin E, has emerged as a promising treatment option for refractory CD30+ mycosis fungoides (MF) and primary cutaneous anaplastic large-cell lymphoma (pcALCL). BV has been shown to be safe and effective in treating Hodgkin\'s lymphoma and peripheral T-cell lymphoma. This multicenter, prospective, single-arm phase I/II study evaluated the efficacy of BV in Japanese patients with CD30+ cutaneous lymphomas, namely CD30+ cutaneous T-cell lymphoma. Participants were divided into two groups: those with CD30+ MF or pcALCL (cohort 1, n = 13) and those with CD30+ lymphoproliferative disorders other than those in cohort 1 (cohort 2, n = 3). The studied population included the full analysis set (FAS), modified FAS (mFAS), and safety analysis set (SAF). These sets were identified in cohorts 1 and 1 + 2 and labeled FAS1 and FAS2, mFAS1 and mFAS2, and SAF1 and SAF2, respectively. Each treatment cycle lasted 3 weeks, and BV was continued for up to 16 cycles after the third cycle based on treatment response. The primary endpoint was the 4-month objective response rate (ORR4) determined by the Independent Review Forum (IRF). ORR4 was 69.2% for FAS1 and 62.5% for FAS2 (P < 0.0001). Secondary endpoints of ORR, assessed using the global response score (53.8% in FAS1) and modified severity-weighted assessment tool (62.5% in FAS1), using the IRF, provided results comparable to the primary findings. The incidence of ≥grade 3 adverse events (≥15%) in SAF1 was peripheral neuropathy in three patients (23%) and fever and eosinophilia in two patients (15%). In conclusion, BV showed favorable efficacy, tolerability, and safety profile in Japanese patients with relapsed or refractory CD30+ primary cutaneous T-cell lymphoma. The trial was registered with University Hospital Medical Information Network Clinical Trials Registry, Japan (protocol ID: UMIN000034205).
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  • 文章类型: Journal Article
    经批准的皮肤T细胞淋巴瘤(CTCL)全身疗法的反应率徘徊在30%左右,暗示未满足的需求。这项研究描述了CTCL患者体外光置换(ECP)的真实世界治疗模式和反应率。
    在美国对2017年1月1日至2019年2月28日期间开始ECP并接受至少3个月ECP治疗的CTCL成人进行了图表回顾。每季度收集临床结果,为期18个月。
    52例患者主要为白种人。一半为男性;中位年龄为69岁。大多数患者患有Sézary综合征(50%)或真菌病(36.5%)。近40%的患者患有IV期疾病;33%的患者有淋巴结受累。19名患者(36.5%)获得了响应(受影响的BSA减少>50%);中位响应时间为6.5个月。在6个月(N=22)时,被评为至少最小改善的患者百分比为59.5%,9个月时75.0%(N=24),ECP开始后12个月(N=15)为60.0%。
    尽管本研究中接受ECP治疗的人群比最近的试验年龄更大,并且患有更晚期的疾病,反应率相当。这些真实世界的发现支持ECP作为CTCL患者的有效治疗选择。
    UNASSIGNED: Response rates of approved systemic therapies for cutaneous T-cell lymphoma (CTCL) hover near 30%, suggesting unmet need. This study describes real-world treatment patterns and response rates of extracorporeal photopheresis (ECP) in CTCL patients.
    UNASSIGNED: A chart review was conducted in the United States of adults with CTCL who initiated ECP between January 1, 2017, and February 28, 2019, and received at least three months of ECP treatment as monotherapy or concomitant therapy. Clinical outcomes were collected quarterly for up to 18 months.
    UNASSIGNED: The 52 patients were predominantly Caucasian. Half were male; median age was 69 years. Most patients had Sézary syndrome (50%) or mycosis fungoides (36.5%). Nearly 40% of patients had stage IV disease; 33% had lymph node involvement. Nineteen patients (36.5%) achieved response (>50% reduction in BSA affected); median time to response was 6.5 months. The percentage of patients rated as at least minimally improved was 59.5% at 6 months (N = 22), 75.0% at 9 months (N = 24), and 60.0% at 12 months (N = 15) after ECP initiation.
    UNASSIGNED: Despite the ECP treated population in this study being older and having more advanced-stage disease than recent trials, response rates were comparable. These real-world findings support ECP as an effective treatment option for CTCL patients.
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  • 文章类型: Journal Article
    真菌病(MF)是最常见的原发性皮肤T细胞淋巴瘤(CTCL),其病因尚未完全了解。白细胞介素(IL)-35是属于IL-12家族的抑制性细胞因子。血浆和肿瘤微环境中IL-35的升高会增加肿瘤发生,并表明不同类型恶性肿瘤的预后不良。这项研究的目的是评估MF患者与健康对照的组织和血清中IL-35的表达水平。这项病例对照研究包括35例患者,牌匾,和肿瘤MF以及30名健康对照。对患者进行全面评估,在开始治疗前采集血清样本和皮损活检。通过ELISA技术测量血清和组织活检中的IL-35水平。MF患者的组织和血清IL-35水平均明显高于对照组(P<0.001),MF患者的组织IL-35明显高于血清IL-35(P<0.001)。与男性患者和无复发性疾病患者相比,女性患者和复发性MF患者的组织IL-35明显更高(P<0.001)。由于MF中组织和血清IL-35水平均增加,IL-35可能在MF的发病机制中起作用。IL-35可以是MF的有用诊断标记。组织IL-35也可以是疾病复发的指标。
    Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma (CTCL) with its etiology not yet fully understood. Interleukin (IL)-35 is an inhibitory cytokine that belongs to the IL-12 family. Elevated IL-35 in the plasma and the tumor microenvironment increases tumorigenesis and indicates poor prognosis in different types of malignancies. The objective of this study is to estimate the expression levels of IL-35 in tissue and serum of MF patients versus healthy controls. This case-control study included 35 patients with patch, plaque, and tumor MF as well as 30 healthy controls. Patients were fully assessed, and serum samples and lesional skin biopsies were taken prior to starting treatment. The IL-35 levels were measured in both serum and tissue biopsies by ELISA technique. Both tissue and serum IL-35 levels were significantly higher in MF patients than in controls (P < 0.001) and tissue IL-35 was significantly higher than serum IL-35 in MF patients (P < 0.001). Tissue IL-35 was significantly higher in female patients and patients with recurrent MF compared to male patients and those without recurrent disease (P < 0.001). Since both tissue and serum IL-35 levels are increased in MF, IL-35 is suggested to have a possible role in MF pathogenesis. IL-35 can be a useful diagnostic marker for MF. Tissue IL-35 can also be an indicator of disease recurrence.
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  • 文章类型: Journal Article
    背景:真菌病(MF)是皮肤T细胞淋巴瘤的最常见类型。
    目的:本研究旨在评估干扰素(IFN)α-2a联合光疗对早期MF的疗效和安全性。
    方法:13例早期MF患者接受3百万IU的IFNα-2a皮下注射联合光疗,每周3次,持续6个月。通过治疗后1、3和6个月的体表面积(BSA)评分和改良的严重程度加权评估工具(mSWAT)评分的变化来衡量治疗效果。治疗前后进行皮肤病变的组织病理学检查。
    结果:治疗3个月后,所有13名患者均获得部分缓解,BSA和mSWAT评分显著低于基线(p<0.001)。六个月后,BSA和mSWAT评分显著低于基线(p<0.001)和3个月后(p<0.05)。11例患者达到完全缓解,2例患者达到部分缓解(总体缓解率,100%)。组织病理学检查显示,表皮和真皮中非典型淋巴细胞的数量显着减少。无严重不良反应发生。
    结论:IFNα-2a联合光疗可能是治疗早期MF的有效和安全的替代方法。
    Background: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma.
    Objectives: This study was conducted to evaluate efficacy and safety of interferon (IFN) α-2a combined with phototherapy for early-stage MF.
    Methods: Thirteen patients with early-stage MF received subcutaneous injections of IFN α-2a at 3 million IU combined with phototherapy three times per week for 6 months. Treatment efficacy was measured by changes in body surface area (BSA) score and modified severity-weighted assessment tool (mSWAT) score at 1, 3, and 6 months after treatment. Histopathologic examinations of skin lesions were performed before and after treatment.
    Results: After 3 months of treatment, all 13 patients achieved a partial response, and BSA and mSWAT scores were significantly lower than those at baseline (p < 0.001). After 6 months, BSA and mSWAT scores were significantly lower than those at baseline (p < 0.001) and after 3 months (p < 0.05). Eleven patients achieved complete remission and two patients achieved a partial response (overall response rate, 100%). Histopathologic examination showed a significant decrease in the number of atypical lymphocytes in both epidermis and dermis. No severe adverse effects occurred.
    Conclusion: IFN α-2a in combination with phototherapy may be an effective and safe alternative modality for early-stage MF.
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  • 文章类型: Editorial
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