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伴有大细胞转化的红皮病型蕈样肉芽肿:一例罕见且复杂的病例。

Erythrodermic mycosis fungoides with large cell transformation: An unusual and complicated case.

作者信息

Lombardi Conner V, Glosser Logan D, Hopper Wade, Veria Spiro, Awad Mohammed T, Garg Anu

机构信息

College of Medicine and Life Sciences, The University of Toledo, Toledo, OH, USA.

Edward via College of Osteopathic Medicine, Spartanburg, SC, USA.

出版信息

SAGE Open Med Case Rep. 2022 Oct 24;10:2050313X221131163. doi: 10.1177/2050313X221131163. eCollection 2022.

DOI:10.1177/2050313X221131163
PMID:36313267
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9608024/
Abstract

Mycosis fungoides is the most common cutaneous T-cell lymphoma. It presents a diagnostic challenge due to resemblance with many other dermatologic conditions. The disease typically follows a progression from patches to plaques to skin-based tumors with potential for visceral involvement. Diagnosis is made by clinical presentation and histology. When early diagnosis is made, there is an estimated 88% five-year survival. This report details a 60-year-old Black man diagnosed with stage IIIA mycosis fungoides with a severe degree of cutaneous involvement. This case is unique due to the aggressive large cell transformation and rapid progression to death within 18 months of diagnosis. We highlight the challenge of diagnosing, treating, and monitoring the therapeutic response of mycosis fungoides. Finally, this case calls for a multi-disciplinary approach to treatment and to include mycosis fungoides on the differential diagnosis for patients presenting with a variety of vague, recurrent cutaneous symptoms, especially with patchy dyspigmentation or plaques.

摘要

蕈样肉芽肿是最常见的皮肤T细胞淋巴瘤。由于与许多其他皮肤病状况相似,它带来了诊断挑战。该疾病通常经历从斑片到斑块再到皮肤肿瘤的进展过程,并有可能累及内脏。诊断通过临床表现和组织学进行。如果能早期诊断,估计五年生存率为88%。本报告详细介绍了一名60岁的黑人男性,被诊断为IIIA期蕈样肉芽肿,皮肤受累程度严重。该病例独特之处在于发生了侵袭性大细胞转化,并在诊断后18个月内迅速进展至死亡。我们强调了蕈样肉芽肿诊断、治疗及监测治疗反应方面的挑战。最后,该病例呼吁采用多学科方法进行治疗,并将蕈样肉芽肿纳入对出现各种模糊、复发性皮肤症状(尤其是斑片状色素沉着异常或斑块)患者的鉴别诊断中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7d/9608024/97f49f31aa4c/10.1177_2050313X221131163-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7d/9608024/508661b2bc9a/10.1177_2050313X221131163-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7d/9608024/6bbdbe6f9267/10.1177_2050313X221131163-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7d/9608024/97f49f31aa4c/10.1177_2050313X221131163-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7d/9608024/508661b2bc9a/10.1177_2050313X221131163-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7d/9608024/6bbdbe6f9267/10.1177_2050313X221131163-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7d/9608024/97f49f31aa4c/10.1177_2050313X221131163-fig3.jpg

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本文引用的文献

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Early-stage Mycosis Fungoides: Epidemiology and Prognosis.早期蕈样肉芽肿:流行病学和预后。
Acta Derm Venereol. 2020 Jan 7;100(1):adv00013. doi: 10.2340/00015555-3367.
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Defining the mimics and clinico-histological diagnosis criteria for mycosis fungoides to minimize misdiagnosis.定义蕈样肉芽肿的模仿者及临床组织学诊断标准以尽量减少误诊。
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Evaluation of the Association Between Epstein-Barr Virus and Mycosis Fungoides.爱泼斯坦-巴尔病毒与蕈样肉芽肿之间关联的评估
Indian J Dermatol. 2015 May-Jun;60(3):321. doi: 10.4103/0019-5154.156423.
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The CARE guidelines: consensus-based clinical case reporting guideline development.《CARE指南:基于共识的临床病例报告指南制定》
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Long-term outcomes of 1,263 patients with mycosis fungoides and Sézary syndrome from 1982 to 2009.1982 年至 2009 年间 1263 例蕈样肉芽肿和赛泽里综合征患者的长期预后。
Clin Cancer Res. 2012 Sep 15;18(18):5051-60. doi: 10.1158/1078-0432.CCR-12-0604. Epub 2012 Jul 31.
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