cutaneous T-cell lymphoma

皮肤 T 细胞淋巴瘤
  • 文章类型: Journal Article
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    人类T细胞淋巴瘤(TCL)的组织病理学分类具有独特的突变基因分型,表明淋巴发生不同。假设在具有不同TCL表型的狗中观察到类似的概念。
    这项研究旨在鉴定先前报道的人类和狗在犬TCL和无效细胞淋巴瘤(NCLs)中的单核苷酸多态性(SNP),并基于多重聚合酶链反应从每个变体设计兼容的寡核苷酸。
    基因组DNA从68个肿瘤标本(62个TCL和6个NCL)和5个血沉棕黄层样本中提取。使用MassARRAY在来自21个基因的44个SNP中分析了四种TCL亚型和NCL。
    在所有TCL亚型和NCL软件SATB1c.1259A>C中观察到的SNP的最大发生率,KITc.1275A>G,SEL1Lc.2040+200C>G,TP53c.1024C>T,分别。一些SNP位置与NCL有统计学意义,包括MYCp.S75F(p=0.0003),TP53p.I149N(p=0.030),PDCD1p.F37LX(p=0.012),和POT1p.R583*(p=0.012)。
    每个TCL组织学亚型和NCL都可能包含独特的突变遗传特征,这可能在淋巴瘤基因危险因素中发挥作用,并可能有助于为每位犬科患者选择治疗性靶向药物。
    UNASSIGNED: The histopathological classification of T-cell lymphoma (TCL) in humans has distinctive mutational genotyping that suggests different lymphomagenesis. A similar concept is assumed to be observed in dogs with different TCL phenotypes.
    UNASSIGNED: This study aimed to identify the previously reported single-nucleotide polymorphisms (SNPs) in both human beings and dogs in canine TCLs and null-cell lymphomas (NCLs) and to design compatible oligonucleotides from each variant based on the multiplex polymerase chain reaction.
    UNASSIGNED: Genomic DNA was extracted from 68 tumor specimens (62 TCLs and 6 NCLs) and 5 buffy coat samples from dogs with TCL. Four TCL subtypes and NCL were analyzed in 44 SNPs from 21 genes using the MassARRAY.
    UNASSIGNED: The greatest incidences of SNPs observed in all TCL subtypes and NCL ware SATB1 c.1259A > C, KIT c.1275A > G, SEL1L c.2040 + 200C > G, and TP53 c.1024C > T, respectively. Some SNP locations were statistically significant associated with NCL, including MYC p.S75F (p = 0.0003), TP53 p.I149N (p = 0.030), PDCD1 p.F37LX (p = 0.012), and POT1 p.R583* (p = 0.012).
    UNASSIGNED: Each TCL histological subtype and NCL are likely to contain distinctive mutational genetic profiles, which might play a role in lymphoma gene-risk factors and might be useful for selecting therapeutic target drugs for each canine patient.
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  • 文章类型: Case Reports
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  • 文章类型: English Abstract
    Mycosis fungoides (MF) is defined as the most common cutaneous T‑cell lymphoma (CTCL). The bullous form is considered one of its numerous variants. Only a few cases of this rare entity have been described. We report the case of a man with an aggressive course of bullous MF, which led to lethal outcome within a few weeks due to a fulminant sepsis.
    UNASSIGNED: Die Mycosis fungoides (MF) ist das häufigste kutane T‑Zell-Lymphom (CTCL). Die bullöse Form gilt als eine ihrer zahlreichen Varianten. In der Literatur sind nur wenige Fälle dieser seltenen Entität beschrieben. Wir berichten von einem männlichen Patienten mit fulminantem Verlauf einer bullösen MF, die aufgrund der infausten septischen Gesamtkonstellation innerhalb weniger Wochen zum Exitus letalis führte.
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  • 文章类型: Journal Article
    背景:晚期真菌病(MF)和Sézary综合征(SS)的预后较差,中位生存期少于5年。尽管MF/SS患者批准了多种治疗方法,持久的完全缓解(CR)很少见。
    方法:通过对主要研究者数据库的回顾性搜索,确定了达到CR并维持CR或IA期10年以上的晚期MF或SS患者。
    结果:在2266例确诊为MF或SS的患者中,23例晚期MF/SS(6IIB,1IIIB,确定5IVA1,3IVA2,8IVB)达到CR并维持CR或IA期≥10年。作为最终/治愈性治疗,11例患者行同种异体干细胞移植(SCT)。大多数患者在年轻时出现,接受SCT与降低强度预处理方案,有匹配的相关捐赠者,和可控的移植后移植物抗宿主病。11例患者接受了TSEB作为联合模式方案的一部分,其中2例患者接受了TSEB治疗,9例患者接受了同种异体SCT之前的减积治疗。5例IIB期患者放疗获得CR。对4例血液受累患者进行了体外光脱疗法(ECP)联合长期抗生素和免疫调节剂治疗。14例患者接受长期抗生素治疗。
    结论:TSEB随后是同种异体SCT,放射治疗,ECP加长期抗生素和免疫调节剂是我们患者中最常见的治愈/最终治疗方法。我们正在报告我们的长期完整应答者的治疗过程的细节,希望在未来获得更多的治疗反应。
    BACKGROUND: Advanced-stage mycosis fungoides (MF) and Sézary syndrome (SS) have poor prognosis with median survivals of less than 5 years. Although a variety of treatments are approved for MF/SS patients, durable complete remissions (CR) are rare.
    METHODS: Advanced-stage MF or SS patients who achieved CR and maintained in CR or stage IA for more than 10 years were identified by a retrospective search of the principal investigator\'s database.
    RESULTS: Of 2266 patients diagnosed with MF or SS, 23 patients with advanced-stage MF/SS (6 IIB, 1 IIIB, 5 IVA1, 3 IVA2, 8 IVB) who achieved CR and maintained in CR or stage IA for ≥ 10 years were identified. As final/curative treatment, 11 patients underwent allogeneic stem cell transplantation (SCT). Most patients presented at young age, underwent SCT with reduced intensity conditioning regimen, had matched related donors, and controllable post-transplant graft versus host disease. Eleven patients were treated with TSEB as part of combined modality protocol in 2 patients and debulking therapy before allogeneic SCT in 9 patients. Five stage IIB patients achieved CR with radiotherapy. Four patients with blood involvement were treated with extracorporeal photopheresis (ECP) in combination with long-term antibiotics and immunomodulatory agents. Long-term antibiotics were given to 14 patients.
    CONCLUSIONS: TSEB followed by allogeneic SCT, radiotherapy, ECP plus long-term antibiotics and immunomodulatory agents were the most common curative/final treatments found in our patients. We are reporting the details of our long-term complete responders\' treatment course in the hopes of obtaining more cure responses in the future.
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  • 文章类型: Case Reports
    真菌病是原发性皮肤T细胞淋巴瘤最常见的亚型。诊断基于彻底的临床-病理相关性,它可以,特别是在早期疾病中,由于与牛皮癣和特应性皮炎等几种良性皮肤病相似,因此具有挑战性。这里,我们介绍了一例81岁的男性,他有20年的皮肤问题病史,治疗为银屑病,效果有限.自2021年12月以来,患者的皮肤症状恶化,肿瘤迅速生长,斑块和斑块广泛分布。正电子发射断层扫描/计算机断层扫描评估显示,与皮肤肿瘤有关的代谢活性显着,并且腹膜后淋巴结中的FDG摄取增加。皮肤活检的组织学评估表明,高度增殖性T细胞淋巴瘤具有γ/δ和CD8细胞毒性表型。肿瘤细胞的形态出现了异常的免疫表型CD3+,CD2-,CD5dim,CD4-,CD8+,CD56-,和CD30-。下一代测序检测到一个可能的致病性SOCS1突变,等位基因频率为72%,以及一个未知意义的STAT3变异。此病例突出了惰性皮肤淋巴瘤演变成侵袭性细胞毒性淋巴瘤的诊断复杂性。
    Mycosis fungoides is the most frequent subtype of primary cutaneous T-cell lymphomas. The diagnosis is based on a thorough clinic-pathologic correlation, which can, especially in early-stage disease, be challenging due to similarities with several benign skin disorders such as psoriasis and atopic dermatitis. Here, we present a case of an 81-year-old man with a 20-year-long medical history of skin problems treated as psoriasis with limited effect. Since December 2021, the patient experienced worsening of his skin symptoms with rapidly growing tumors and widespread patches and plaques. Positron emission tomography/computed tomography evaluation revealed markedly metabolic activity related to the skin tumors and increased FDG uptake in several retroperitoneal lymph nodes. Histological assessment of skin biopsies demonstrated a highly proliferative T-cell lymphoma with a γ/δ+ and CD8+ cytotoxic phenotype. The morphology of the tumor cells appeared blastic with an abnormal immunephenotype CD3+, CD2-, CD5dim, CD4-, CD8+, CD56-, and CD30-. Next-generation sequencing detected a likely pathogenic SOCS1 mutation with an allele frequency of 72% as well as a STAT3 variant of unknown significance. This case highlights the diagnostic complexity of an indolent skin lymphoma evolving into an aggressive cytotoxic lymphoma.
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  • 文章类型: Journal Article
    皮肤T细胞淋巴瘤是一组非霍奇金T细胞淋巴瘤,在皮肤中发展并影响皮肤,但可能扩散到其他器官。有许多亚型,其中最常见的是霉菌病,Sezary综合征,淋巴瘤样丘疹病,和原发性皮肤间变性大细胞淋巴瘤。皮肤淋巴瘤是顽固性慢性皮疹的常见原因,出了名地模仿其他皮肤病学和血液学疾病,通常导致数月至数年的诊断延迟。本文综述了皮肤T细胞淋巴瘤,主要关注临床表现,诊断,免疫发病机制,和管理的条件。
    Cutaneous T-cell lymphoma is a group of non-Hodgkin T-cell lymphomas that develop in and affect the skin but can potentially spread to other organs. There are many subtypes, the most common of which are mycosis fungoides, Sezary syndrome, lymphomatoid papulosis, and primary cutaneous anaplastic large cell lymphoma. Cutaneous lymphoma is a common cause of recalcitrant chronic skin rash and notoriously mimics other dermatologic and hematologic conditions, often resulting in diagnostic delays of months to years. This review provides an introduction to cutaneous T-cell lymphoma, with a primary focus on the clinical presentation, diagnosis, immunopathogenesis, and management of the condition.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    原发性皮肤T细胞淋巴瘤(CTCL)患者通常会出现严重且难以治疗的瘙痒,从而对其生活质量(QoL)产生负面影响。然而,CTCL瘙痒的机制,包括霉菌病(MF),基本上是未知的,CTCL相关瘙痒的详细特征尚未完全阐明。为了表征CTCL中的瘙痒,皮肤B细胞淋巴瘤(CBCL),和大斑块副银屑病(LPP),并确定CTCL患者瘙痒发病机制中潜在的瘙痒介质。从129名健康受试者和142名患者收集临床数据和血液样本。瘙痒强度,QoL受损,心理困扰,使用经过验证的问卷和工具评估睡眠质量。BDNF的血液水平,CCL24,GRP,IL-31,IL-33,sST2,P物质,TSLP,使用ELISA或ImmunoCAP测量类胰蛋白酶和总IgE。瘙痒在CTCL中普遍存在,LPP和CBCL患者,在CTCL中观察到较高的患病率和严重程度。在CTCL中,瘙痒与QoL显著受损相关,睡眠,心理困扰。与健康对照相比,IL-31,IL-33,P物质的水平升高,总IgE,胰蛋白酶,在MF患者中发现TSLP。有和没有瘙痒的MF患者的比较显示更高水平的IL-31,P物质,GRP,和CCL24在前者。瘙痒强度与IL-31、GRP、CCL24和类胰蛋白酶水平。瘙痒显著加重CTCL患者的负担,需要适当的治疗管理。我们的发现表明,可以探索各种非组胺能介质,例如类胰蛋白酶和IL-31,作为治疗MF患者瘙痒的新治疗靶标。
    Patients with primary cutaneous T-cell lymphoma (CTCL) often experience severe and difficult-to-treat pruritus that negatively affects their quality of life (QoL). However, the mechanisms of pruritus in CTCL, including mycosis fungoides (MF), remain largely unknown, and detailed characteristics of CTCL-associated pruritus is not fully elucidated. To characterize pruritus in CTCL, cutaneous B-cell lymphoma (CBCL), and large plaque parapsoriasis (LPP), and to identify potential itch mediators involved in the pathogenesis of pruritus in CTCL patients. Clinical data and blood samples were collected from 129 healthy subjects and 142 patients. Itch intensity, QoL impairment, psychological distress, and sleep quality were assessed using validated questionnaires and instruments. Blood levels of BDNF, CCL24, GRP, IL-31, IL-33, sST2, substance P, TSLP, tryptase and total IgE were measured using ELISA or ImmunoCAP. Pruritus was prevalent in CTCL, LPP and CBCL patients, with higher prevalence and severity observed in CTCL. In CTCL, pruritus correlated with significant impairment in QoL, sleep, psychological distress. Compared to healthy controls, elevated levels of IL-31, IL-33, substance P, total IgE, tryptase, and TSLP were found in MF patients. A comparison of MF patients with and without pruritus revealed higher levels of IL-31, substance P, GRP, and CCL24 in the former. Itch intensity positively correlated with IL-31, GRP, CCL24, and tryptase levels. Pruritus significantly burdens CTCL patients, necessitating appropriate therapeutic management. Our findings suggest that various non-histaminergic mediators such as tryptase and IL-31 could be explored as novel therapeutic targets for managing pruritus in MF patients.
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