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一名骨髓纤维化患者出现Sweet综合征的非典型表现。

An atypical presentation of Sweet's syndrome in a myelofibrosis patient.

作者信息

Thebo Umera, Tummala Sirisha, Nassereddine Samah, Haroun Faysal

机构信息

Hematology and Oncology, George Washington University, Washington, DC, USA.

George Washington University School of Medicine and Health Sciences, Washington, DC, USA.

出版信息

BMJ Case Rep. 2019 Mar 9;12(3):e228076. doi: 10.1136/bcr-2018-228076.

Abstract

A 46-year-old man with no significant medical history presented to haematology with symptoms of fatigue, dyspnoea on exertion and weight loss. Physical examination revealed a lesion on the right shin and splenomegaly. Labs were significant for leucocytosis with immature components, thrombocytosis and 3% peripheral blasts on smear. A bone marrow biopsy confirmed a diagnosis of myelofibrosis (MF). Dynamic International Prognosis Scoring system was 2. He was started on ruxolitnib, with decitabine added subsequently prior to definitive therapy with an allogenic haematopoietic stem cell transplant. His course with decitabine was complicated with febrile neutropaenia with multiple tender erythematous plaques unresponsive to antibacterial and antifungal coverage. A skin biopsy showed neutrophilic dermatitis, consistent with a diagnosis of Sweet's syndrome (SS) and empirical treatment with glucocorticoids was initiated resulting in resolution of symptoms. This report reviews the literature for cases of SS in the setting of MF.

摘要

一名46岁无显著病史的男性因疲劳、劳力性呼吸困难和体重减轻症状就诊于血液科。体格检查发现右小腿有一处病变及脾肿大。实验室检查显示白细胞增多伴未成熟成分、血小板增多,涂片可见3%外周血原始细胞。骨髓活检确诊为骨髓纤维化(MF)。动态国际预后评分系统评分为2分。他开始使用芦可替尼治疗,随后在进行异基因造血干细胞移植的确定性治疗之前加用了地西他滨。他使用地西他滨的过程中出现了发热性中性粒细胞减少症,并伴有多处压痛性红斑性斑块,对抗菌和抗真菌治疗无效。皮肤活检显示为嗜中性皮病,符合Sweet综合征(SS)的诊断,遂开始使用糖皮质激素进行经验性治疗,症状得以缓解。本报告回顾了MF背景下SS病例的文献。

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本文引用的文献

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Sweet syndrome: long-term follow-up of 138 patients.Sweet综合征:138例患者的长期随访
Clin Exp Dermatol. 2016 Oct;41(7):741-6. doi: 10.1111/ced.12899.
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Sweet syndrome as terminal event in ruxolitinib-treated myelofibrosis.
Br J Haematol. 2015 May;169(3):307. doi: 10.1111/bjh.13334. Epub 2015 Feb 23.
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Splenic irradiation-induced Sweet's syndrome associated with preceding myelofibrosis.
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