cutaneous T-cell lymphoma

皮肤 T 细胞淋巴瘤
  • 文章类型: Journal Article
    高频超声检查(HFUS)已成为评估皮肤病治疗程序结果的非侵入性且具有成本效益的诊断工具。
    本手稿提供了全面的超声图像集,描绘了与各种皮肤病相关的皮肤病变,包括寻常疣,表皮样囊肿,斑丘疹性皮肤肥大细胞增多症和硬化性苔藓。
    从现有文献的广泛回顾和经验观察的支持下,该研究突出了在正常和病理性皮肤变异中均可观察到的关键超声特征.
    已经证明个体皮肤损伤在HFUS上表现出不同的特征。此外,事实证明,超声检查对于客观评估疾病的严重程度很有价值。
    另外,这项研究有助于更深入地了解HFUS作为一种有价值的皮肤病学诊断工具.
    UNASSIGNED: High-frequency ultrasonography (HFUS) has emerged as a non-invasive and cost-effective diagnostic tool for evaluating the outcomes of dermatological therapeutic procedures.
    UNASSIGNED: This manuscript presents a comprehensive collection of sonographic images depicting cutaneous lesions associated with various dermatoses, including verruca vulgaris, epidermoid cyst, maculopapular cutaneous mastocytosis and lichen sclerosus et atrophicus.
    UNASSIGNED: Drawing from an extensive review of the existing literature and supported by empirical observations, the study highlights key sonographic attributes observable in both normal and pathological skin variants.
    UNASSIGNED: It has been demonstrated that individual skin lesions exhibit distinct characteristics on HFUS. Furthermore, ultrasonographic examination has proven to be valuable for the objective assessment of disease severity.
    UNASSIGNED: Additionally, the study contributes to a deeper understanding of HFUS as a valuable tool in dermatological diagnostics.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    Sezary综合征(SS)是一种罕见但侵袭性的皮肤T细胞淋巴瘤(CTCL)。SS患者具有特征性皮肤病变(红皮病)和白血病期。与CTCL相关的皮疹通常可以模仿常见的良性皮肤病,如牛皮癣,特应性皮炎,等。因此可能会在后期才被诊断出来。我们介绍了一例SS患者,该患者在接受皮肤活检之前用局部类固醇治疗了一年以上的湿疹。检查证实有白血病参与,患者开始接受贝沙罗汀的全身治疗。患者继续对全身治疗有良好的反应。治疗病因不明和/或对治疗无反应的持续性皮疹患者时,初级保健医师和内科医生需要将SS/真菌菌病作为可能的鉴别因素考虑在内,并且应具有较低的阈值,以便早期转诊至皮肤科进行明确诊断.
    Sezary syndrome (SS) is a rare but aggressive type of cutaneous T-cell lymphoma (CTCL). Patients with SS have characteristic skin lesions (erythroderma) and a leukemic phase. The rash associated with CTCLs can often mimic common benign skin conditions such as psoriasis, atopic dermatitis, etc. and therefore can go undiagnosed until later stages. We present a case of a patient with SS who managed eczema for over one year with topical steroids before receiving a skin biopsy. Workup confirmed leukemic involvement, and the patient was started on systemic therapy with bexarotene. The patient continues to have a good response to systemic therapy. When treating patients with persistent rash of uncertain etiology and/or unresponsive to treatment, primary care physicians and internists need to consider SS/Mycosis fungoides as a possible differential and should have a low threshold to initiate early referral to dermatology for definitive diagnosis.
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  • 文章类型: Systematic Review
    背景:肉芽肿样真菌病(GMF)是一种罕见形式的皮肤T细胞淋巴瘤,其特征是肉芽肿性炎症浸润。
    目的:肉芽肿性炎症对疾病预后的影响仍存在争议,因为有有利和不利的结局记录。
    方法:我们对文献中先前描述的116例GMF病例进行了系统评价。
    结果:与经典的Alibert-Bazin型真菌病(MF)相反,GMF的皮肤病变倾向于累及远端四肢(小腿,脚,手)在疾病进程的早期。在文学中,30%的GMF患者发生器官转移,最常见的是肺部。进展期的中位时间为25个月。
    结论:GMF是真菌的侵袭性形式。因此,应在就诊时考虑筛查远处转移,并在随访期间重复筛查.
    BACKGROUND: Granulomatous mycosis fungoides (GMF) is a rare form of cutaneous T-cell lymphoma characterized by a granulomatous inflammatory infiltrate.
    OBJECTIVE: The impact of granulomatous inflammation on the prognosis of the disease remains controversial as there have been both favorable and unfavorable outcomes documented.
    METHODS: We performed a systematic review of 116 GMF cases previously described in the literature.
    RESULTS: In contrast to the classic Alibert-Bazin type of mycosis fungoides (MF), cutaneous lesions in GMF tend to involve distal extremities (lower legs, feet, hands) early in the disease course. In the literature, 30% of GMF patients developed organ metastasis, most frequently to the lung. The median time to stage progression was 25 months.
    CONCLUSIONS: GMF is an aggressive form of MF. Therefore, screening for distant metastases should be considered at presentation and repeated during follow-up.
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  • 文章类型: Journal Article
    Mogamulizumab(MOG)是一种靶向CCR4受体的抗体,已批准用于复发或难治性外周T细胞(PTCL)和皮肤T细胞淋巴瘤(CTCL)。它在指南中的采用以及FDA和EMA的认可将其确立为全身性治疗,特别是对于晚期疾病阶段,由于其相对较低的毒性。临床试验和现实世界的证据强调了其在先进的CTCL中的疗效,包括真菌病和Sézary综合征;PTCL;和成人T细胞白血病/淋巴瘤(ATLL),展示积极成果。值得注意的是,这种药物表现出显著的反应率,疾病稳定性,和延长的无进展生存期,表明其在具有多个治疗线的情况下的适用性。它的安全性通常是可控的,主要与皮肤相关的不良事件(AE),输液相关反应,药疹,自身免疫性疾病,和皮肤病。后者似乎表现为CCR4可以促进淋巴细胞的皮肤特异性归巢,MOG是针对这种受体的。虽然与干扰素α和白细胞介素12等免疫刺激剂的组合显示出有希望的结果,由于免疫介导的AE的风险增加,与PD1抑制剂联合使用时应谨慎。MOG作为全身性治疗的引入意味着在管理这些疾病方面取得了重大进展,其有利的安全状况和补充机制的支持。
    Mogamulizumab (MOG) is an antibody targeting the CCR4 receptor, authorized for relapsed or refractory peripheral T-cell (PTCL) and cutaneous T-cell lymphomas (CTCL). Its adoption in guidelines and endorsement by FDA and EMA established it as a systemic treatment, especially for advanced disease stages due to its comparatively lower toxicity. Clinical trials and real-world evidence have underscored its efficacy in advanced CTCLs, including mycosis fungoides and Sézary syndrome; PTCLs; and adult T-cell leukemia/lymphoma (ATLL), showcasing positive outcomes. Notably, the drug has demonstrated significant response rates, disease stability, and extended periods of progression-free survival, suggesting its applicability in cases with multiple treatment lines. Its safety profile is generally manageable, with adverse events (AEs) primarily related to the skin, infusion-related reactions, drug eruptions, autoimmune diseases, and skin disorders. The latter seem to appear as CCR4 can promote the skin-specific homing of lymphocytes, and MOG is directed against this receptor. While combination with immunostimulatory agents like interferon alpha and interleukin 12 has shown promising results, caution is urged when combining with PD1 inhibitors due to the heightened risk of immune-mediated AEs. The introduction of MOG as a systemic treatment implies a significant advancement in managing these diseases, supported by its favorable safety profile and complementary mechanisms.
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  • 文章类型: Case Reports
    移植后淋巴增生性疾病是一种罕见的疾病,年发病率为0.5%至3.7%。这种疾病的发展伴随着不良的预后。在这份报告中,我们描述了一例肾移植后患者移植后原发性皮肤T细胞淋巴瘤(PT-CTCL)真菌病IIB期的罕见病例,以及文献中报道的PT-CTCL的综述。诊断后的治疗包括贝沙罗汀,环孢菌素,和泼尼松。目前,病人没有疾病。此信息旨在增加对PT-CTCL的患病率和管理的了解。
    Post-transplant lymphoproliferative disease is a rare disorder with an annual incidence of 0.5% to 3.7%. Development of this disorder carries with it a poor prognosis. In this report, we describe a rare case of post-transplant primary cutaneous T-cell lymphoma (PT-CTCL) mycosis fungoides stage IIB in a patient following kidney transplantation, as well as a review of PT-CTCL reported in the literature. The treatment following diagnosis included bexarotene, cyclosporine, and prednisone. Currently, the patient is free from disease. This information aims to add to the knowledge of the prevalence and management of PT-CTCL.
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  • 文章类型: Case Reports
    血管内皮肤间变性大细胞淋巴瘤(ALCL)是一种极为罕见的非霍奇金淋巴瘤,在小血管腔中增殖,并倾向于在皮肤中表现出来。文献中描述的大多数血管内淋巴瘤病例是大B细胞淋巴瘤,使T细胞淋巴瘤非常罕见。因此,我们介绍了一名87岁男性原发性皮肤血管内间变性大T细胞淋巴瘤,最初表现为红斑,右中腹部皮下结节。我们报告的免疫组织化学结果显示淋巴瘤细胞对CD3和CD30呈阳性染色,并且缺乏间变性淋巴瘤激酶的表达,泛细胞角蛋白,CD10、CD20和SOX10。我们还回顾并比较了先前报道的血管内ALCL与原发性皮肤受累的病例。
    Intravascular cutaneous anaplastic large cell lymphoma (ALCL) is an extremely rare non-Hodgkin lymphoma that proliferates in the lumen of small blood vessels and has a propensity to manifest in the skin. Most reported cases of intravascular lymphoma described in the literature are of large B-cell lymphomas, making T-cell lymphomas incredibly rare. As such, we present the case of an 87-year-old male with primary cutaneous intravascular anaplastic large T-cell lymphoma that initially presented with an erythematous, subcutaneous nodule on the right mid-abdomen. We report the immunohistochemical results showing lymphoma cells staining positively for CD3 and CD30 and lacking expression of anaplastic lymphoma kinase, pan-cytokeratin, CD10, CD20, and SOX10. We also review and compare previously reported cases of intravascular ALCL with primary cutaneous involvement.
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  • 文章类型: Case Reports
    甲氨蝶呤相关的淋巴增殖性疾病(MTX-LPDs)是甲氨蝶呤(MTX)给药后的免疫缺陷疾病,主要发生在类风湿性关节炎患者。虽然不常见,MTX-LPDs已在一些牛皮癣患者中报道,皮肌炎,和接受MTX治疗的皮肤T细胞淋巴瘤(CTCL)。肉芽肿性真菌病(GMF)是皮肤T细胞淋巴瘤的一种罕见亚型,其中MTX是顽固性病例的治疗选择之一。在这里,我们报道了一例72岁女性GMF患者,该患者在MTX治疗期间额外发展为皮肤EB病毒(EBV)阳性弥漫性大B细胞淋巴瘤(DLBCL).根据世界卫生组织血液淋巴样肿瘤分类(WHO-HAEM)的第5版,这种情况目前被归类为“免疫缺陷/失调引起的淋巴瘤”。在这篇文章中,我们还回顾了已发表的关于CTCL中皮肤MTX-LPDs的文献.在新的情况下,应考虑该实体,接受长期MTX治疗的CTCL患者的非典型皮肤结节和/或斑块。
    Methotrexate-related lymphoproliferative disorders (MTX-LPDs) are immunodeficiency diseases following methotrexate (MTX) administration, mainly occurring in rheumatoid arthritis patients. Although uncommon, MTX-LPDs have been reported in some patients with psoriasis, dermatomyositis, and cutaneous T-cell lymphoma (CTCL) who received MTX. Granulomatous mycosis fungoides (GMF) is a rare subtype of cutaneous T-cell lymphoma, where MTX is one of the treatment options in recalcitrant cases. Herein, we report a case of a 72-year-old female patient with GMF who additionally developed cutaneous Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) during MTX treatment. According to the 5th edition of the WHO classification of Haematolymphoid Tumors (WHO-HAEM), this condition is currently categorized as \"lymphoma arising in immunodeficiency/dysregulation\". In this article, we also reviewed published literature on cutaneous MTX-LPDs in the setting of CTCL. This entity should be considered in cases of new, atypical skin nodules and/or plaques in CTCL patients receiving long-term MTX treatment.
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  • 文章类型: Case Reports
    口腔和喉部皮肤T细胞淋巴瘤(CTCL)很少见,通常与预后不良有关。这里,我们讨论了2例严重预处理的患者发生口服CTCL的病例,并提供了文献综述.第一例是一名46岁的非洲裔美国男性,患有快速进展的疾病,在被诊断为CD4+CD8+CTCL6个月后,他的硬腭出现病变。他的皮肤疾病广泛存在,其体表面积的80%以上都有肿瘤。不幸的是,患者在CTCL诊断后2½年和口腔CTCL病变发展后7个月死亡.第二例是一名38岁的非洲裔美国男性,患有IIb期CD3+CD4+CD30+霉菌病(MF),诊断后6个月在硬腭出现肿瘤。他接受了口腔病变的姑息性放疗和肺部的多种全身治疗,喉,食道,和胃受累。胃病变的活检显示CD30T细胞淋巴瘤具有与他的皮肤相同的克隆峰,但具有大细胞转化。Brentuximabvendoin开始了,病人现在已经完全缓解,30个月后。从以英语报告的76例口头CTCL中,六个是转化的MF。最常见的受影响的部位是舌头和腭,最常见的表现是红斑或溃疡性肿瘤,斑块,或与吞咽困难和疼痛相关的结节。口腔CTCL通常在CTCL的初始诊断后数年发生,并且预示预后差,在口腔病变发展后平均存活仅超过1年。
    Oral and laryngeal cutaneous T-cell lymphoma (CTCL) is rare and usually associated with poor prognosis. Here, we discuss 2 cases of oral CTCL that developed in heavily pretreated patients and provide a review of the literature. The first case is of a 46-year-old African American male with rapidly progressive disease, presenting with a lesion on his hard palate 6 months after being diagnosed with a CD4+CD8+ CTCL. His cutaneous disease was widespread with tumors on >80% of his body surface area. Unfortunately, the patient died 2 ½ years after his CTCL diagnosis and 7 months after developing the oral CTCL lesion. The second case is of a 38-year-old African American male with stage IIb CD3+CD4+CD30+ mycosis fungoides (MF), who developed a tumor on the hard palate 6 months after diagnosis. He received palliative radiation to the oral lesion and multiple lines of systemic therapy for pulmonary, laryngeal, esophageal, and gastric involvement. Biopsy of the gastric lesions showed a CD30+ T-cell lymphoma with the same clonal peak as in his skin but with large cell transformation. Brentuximab vendoin was started, and the patient is now in complete remission, 30 months later. From the 76 cases of oral CTCL that have been reported in the English language, six were of transformed MF. The most common sites affected were the tongue and palate, and the most common presentation were erythematous or ulcerated tumors, plaques, or nodules associated with dysphagia and pain. Oral CTCL typically occurs years after the initial diagnosis of CTCL and portend a poor prognosis with an average survival of just over 1 year after development of oral lesions.
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  • 文章类型: Journal Article
    有关摩洛哥真菌病(MF)患者生存的数据有限。评估这些患者的临床特征和长期预后。回顾性分析1993年至2022年诊断的114例MF病例,随访6个月以上。114名患者中,71.9%为男性,诊断时的中位年龄为56岁。大约64%和36%的患者有早期和晚期,分别。中位随访时间为56个月,诊断前症状的中位持续时间为31个月.观察到各种亚型,包括嗜毛囊的霉菌病(12.3%),多皮病(11.4%),和棕榈和足底MF(5.3%)。早期患者的10年总生存率为89%,晚期患者为48.8%。对治疗的完全反应发生率为45.6%,16.7%的患者病情稳定,7.9%的患者病情进展。年龄超过60岁,较高的T期(T3/T4)和晚期MF在预测MF预后较差方面有统计学意义.尽管诊断延迟,摩洛哥的大多数MF病例在早期被诊断。我们观察到经典MF的比例很高,预后良好。
    Limited data regarding survival of Moroccan patients with mycosis fungoides (MF). To evaluate the clinical profile and long-term outcomes of these patients. A retrospective review of 114 MF cases diagnosed from 1993 to 2022 who were followed up for more than 6 months of diagnosis was performed. Of 114 patients, 71.9% were male and the median age at diagnosis was 56 years. Approximately 64 and 36% of the patients had an early stage and advanced stage, respectively. Median follow-up duration was 56 months, and median duration of symptoms before diagnosis was 31 months. Various subtypes were observed, including mycosis fungoides folliculotropic (12.3%), poikilodermatous (11.4%), and palmaris et plantaris MF (5.3%). The 10-year overall survival was 89% in early-stage patients and 48.8% in advanced-stage patients. Complete response to treatment occurred in 45.6%, stable disease in 16.7% and disease progression in 7.9% of patients. Older age of > 60 years, higher T-stage (T3/T4) and advanced-stage MF were statistically significant in predicting poorer outcomes in MF. Despite delay in diagnosis, most cases of MF in Morocco were diagnosed in early stages. We observed a high proportion of classic MF and favorable prognosis.
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