类固醇耐药性嗜酸性粒细胞增多综合征为霍奇金淋巴瘤

Christine J. Licata, Sowmya Arja, S. Teuber
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摘要

嗜酸性粒细胞增多综合征(HES)是罕见的,临床医生可能没有认识到它与恶性肿瘤的潜在联系。HES的危险信号包括类固醇抵抗、年龄较大和可能预示恶性肿瘤的显著淋巴结病。在这种情况下,一名老年男性出现右胸壁红斑和腋窝淋巴结病,最初被诊断为蜂窝组织炎并接受了治疗。实验室对嗜酸性粒细胞增多症具有重要意义。末端器官损伤的证据引起了HES的关注。在三次住院的过程中,尽管接受了高剂量皮质类固醇治疗,但他发现嗜酸性粒细胞计数仍在上升。进一步的调查最终揭示了霍奇金淋巴瘤的诊断。该病例强调了类固醇抵抗性HES是恶性肿瘤的表现,并允许讨论HES治疗的潜在研究方法。尽管皮质类固醇是治疗嗜酸性粒细胞增多症和HES的一线药物,但众所周知,它们有许多不良反应。生物制品,如美宝珠单抗和本拉利珠单抗,具有更可接受的副作用,对治疗非髓系HES有效。使用生物制剂作为HES的一线治疗尚待研究。
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Steroid resistant hypereosinophilic syndrome found to be Hodgkin’s Lymphoma
Hyperoeosinophilic syndrome (HES) is rare, and clinicians may not recognize its potential association with malignancy. Red flag signs of HES include steroid resistance, older age, and significant lymphadenopathy that can be indicative of malignancy. In this case, an elderly male presenting with right chest wall erythema and axillary lymphadenopathy was initially diagnosed with and treated for cellulitis. Labs were significant for hypereosinophilia. Evidence of end organ damage raised concern for HES. Over the course of three hospitalizations, he was found to have a rising eosinophil count despite high-dose corticosteroid treatment. Further investigation eventually revealed a diagnosis of Hodgkin’s Lymphoma. This case highlights steroid-resistant HES as a presenting sign of malignancy and allows for discussion of potential investigative approaches for HES therapy. Though corticosteroids are first-line treatment for hypereosinophilia and HES, they are well known to have many adverse effects. Biologics, such as mepolizumab and benralizumab, have more acceptable side effect profiles and are effective in treating non-myeloid HES.  The use of biologics as first-line treatment for HES has yet to be investigated.
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