An atypical presentation of Sweet’s syndrome in a myelofibrosis patient
An atypical presentation of Sweet’s syndrome in a myelofibrosis patient
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骨髓纤维化患者 Sweet 综合征的非典型表现
DOI：
10.1136/bcr-2018-228076
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发表时间：
2019
期刊：
影响因子：
0.9
通讯作者：
Faysal Haroun
中科院分区：
文献类型：
--
作者：
Umera Thebo;S. Tummala;Samah Nassereddine;Faysal Haroun
患者男，46岁，无明显病史，血液学表现为疲倦、劳累、呼吸困难、体重减轻。体格检查显示右胫骨和脾肿大。未成熟成分的白细胞增多、血小板增多和涂片上3%的外周血细胞增多的发生率显著高于实验室检查。骨髓活检证实诊断为骨髓纤维化(MF)。动态国际预后评分系统为2分。他开始使用鲁索利尼，随后加用地西他滨，然后用异基因造血干细胞移植进行最终治疗。他使用地西他滨的疗程复杂，伴有发热性中性粒细胞减少症，并伴有多个细小的红斑，对抗菌和抗真菌覆盖无效。皮肤活检显示中性粒细胞皮炎，与斯威特综合征(SS)的诊断一致，并开始进行糖皮质激素的经验性治疗，症状得到缓解。这篇报告回顾了有关多发性骨髓间质瘤中SS病例的文献。
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.