嗜中性粒细胞脂膜炎伴骨髓增生异常综合征/骨髓增生性肿瘤:病例报告和文献综述。

Alexa J Cohen, Laura K Gleason, Safiyyah A Bhatti, Neda Nikbakht
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引用次数: 0

摘要

简介:中性粒细胞脂膜炎(NP)是中性粒细胞皮肤病的一种罕见亚型,这是一组富含中性粒细胞的炎症性皮肤病,可与髓系肿瘤相关。NP是指在没有感染或血管炎的情况下,皮下脂肪小叶中存在中性粒细胞浸润。我们在此描述一名最近被诊断为骨髓增生异常综合征/骨髓增生性肿瘤重叠综合征(MDS/MPN)的65岁女性,她突然出现疼痛、瘙痒的结节,符合NP。病例介绍:一名患有MDS/MPN的65岁妇女接受上下肢疼痛和瘙痒结节的评估。活检显示皮下组织有小叶中性粒细胞浸润,无微生物或血管炎迹象。患者被诊断为NP,并接受口服泼尼松治疗。在治疗的1个月内,她报告结节完全消退。讨论:与其他中性粒细胞性皮肤病类似,NP可能与骨髓源性血液系统恶性肿瘤(如MDS/MPN)有关。一项文献综述显示,大多数与MDS相关的NP病例发生在MDS发作后,对全身皮质类固醇而非抗生素有反应。在开始使用全身类固醇治疗之前,应排除感染。结论:尽管其机制尚不清楚,但临床医生必须意识到NP与MDS相关;因此,血液系统恶性肿瘤应在诊断为NP时进行研究。一旦诊断,NP很容易治疗,并且对全身皮质类固醇有良好的反应。
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Neutrophilic panniculitis associated with myelodysplastic syndrome/myeloproliferative neoplasm: a case report and literature review.

Introduction: Neutrophilic panniculitis (NP) is a rare subtype of neutrophilic dermatosis, a group of neutrophil-rich inflammatory skin disorders that can present in association with myeloid neoplasms. NP is defined by the presence of a neutrophilic infiltrate in the fat lobules of the subcutis in the absence of either infection or vasculitis. We herein describe a 65-year-old woman with a recent diagnosis of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndrome (MDS/MPN) who abruptly developed painful, pruritic nodules consistent with NP.

Case presentation: A 65-year-old woman with MDS/MPN presented for evaluation of painful and pruritic nodules on her upper and lower extremities. A biopsy revealed a lobular neutrophilic infiltrate in the subcutis without evidence of microorganisms or vasculitis. The patient was diagnosed with NP and treated with oral prednisone. Within 1 month of treatment, she reported complete resolution of the nodules.

Discussion: Similar to other neutrophilic dermatoses, NP may arise in association with hematologic malignancies of myeloid origin, such as MDS/MPN. A literature review revealed that most cases of NP associated with MDS occur after the onset of MDS and respond to systemic corticosteroids, not antibiotics. Infection should be ruled out before initiating treatment with systemic steroids.

Conclusion: Although the mechanism is still unknown, it is important for clinicians to be aware that NP is associated with MDS; thus, hematological malignancies should be investigated upon diagnosis of NP. Once diagnosed, NP is easily treated and has an excellent response to systemic corticosteroids.

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