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塞扎里综合征:一例病例报告及当前治疗方法综述

Sezary Syndrome: A Case Report and Review of Current Therapeutics.

作者信息

Krishnan Jayasree, Thanikachalam Kannan

机构信息

Internal Medicine, Mobile Infirmary Medical Center, Mobile, USA.

Hematology/Oncology, Infirmary Health System, Mobile, USA.

出版信息

Cureus. 2024 Apr 18;16(4):e58570. doi: 10.7759/cureus.58570. eCollection 2024 Apr.

DOI:10.7759/cureus.58570
PMID:38765439
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11102576/
Abstract

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.

摘要

塞扎里综合征(SS)是一种罕见但侵袭性的皮肤T细胞淋巴瘤(CTCL)。SS患者有特征性皮肤病变(红皮病)和白血病期。与CTCL相关的皮疹常常可模仿常见的良性皮肤疾病,如银屑病、特应性皮炎等,因此在疾病后期之前可能一直未被诊断出来。我们报告了一例SS患者,该患者在接受皮肤活检前使用外用类固醇治疗湿疹长达一年多。检查证实有白血病累及,患者开始接受贝沙罗汀全身治疗。该患者对全身治疗仍有良好反应。在治疗病因不明和/或治疗无反应的持续性皮疹患者时,初级保健医生和内科医生需要考虑将SS/蕈样肉芽肿作为一种可能的鉴别诊断,并且对于早期转诊至皮肤科进行明确诊断应保持较低的阈值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/dfaa4e834723/cureus-0016-00000058570-i09.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/257f63978afd/cureus-0016-00000058570-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/b3b28502a73b/cureus-0016-00000058570-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/96164b01ecf8/cureus-0016-00000058570-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/73cf1714c2d7/cureus-0016-00000058570-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/f13e67902c96/cureus-0016-00000058570-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/679d453d3e05/cureus-0016-00000058570-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/8d5ae351636d/cureus-0016-00000058570-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/bc74b78de29b/cureus-0016-00000058570-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/dfaa4e834723/cureus-0016-00000058570-i09.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/257f63978afd/cureus-0016-00000058570-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/b3b28502a73b/cureus-0016-00000058570-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/96164b01ecf8/cureus-0016-00000058570-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/73cf1714c2d7/cureus-0016-00000058570-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/f13e67902c96/cureus-0016-00000058570-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/679d453d3e05/cureus-0016-00000058570-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/8d5ae351636d/cureus-0016-00000058570-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/bc74b78de29b/cureus-0016-00000058570-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbcf/11102576/dfaa4e834723/cureus-0016-00000058570-i09.jpg

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本文引用的文献

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EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2023.EORTC 共识推荐的蕈样肉芽肿/赛泽里综合征治疗方案 - 2023 年更新版。
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