Aukstuolis Kestutis, Cooper Jocelyn J, Altman Katherine, Lang Anna, Ayars Andrew G
Division of Allergy and Infectious Diseases, Department of Medicine, University of Washington, Seattle, WA, USA.
Northwest Asthma and Allergy Center, Redmond, WA, 98052, USA.
Allergy Asthma Clin Immunol. 2022 Mar 22;18(1):25. doi: 10.1186/s13223-022-00666-2.
Hypereosinophilic syndrome (HES) is an extremely uncommon group of disorders. It rarely presents with coagulopathy without cardiac involvement.
A 33-year-old previously healthy male with no history of atopic disease presented with abdominal pain, hematochezia, peripheral eosinophilia as high as 10,000 eos/µL, right and left portal vein, mesenteric, and splenic vein thrombi with ischemic colitis resulting in hemicolectomy and small bowel resection. Despite an extensive workup for primary and secondary etiologies of hypereosinophilia by hematology/oncology, infectious disease, rheumatology and allergy/immunology, no other clear causes were identified, and the patient was diagnosed with idiopathic HES. His eosinophilia was successfully treated with high-dose oral corticosteroids (OCS) and subsequently transitioned to anti-IL-5-receptor therapy with benralizumab. He has continued this treatment for over a year with no recurrence of eosinophilia or thrombosis while on benralizumab.
In patients with an unexplained coagulopathy and eosinophilia, eosinophilic disorders such as HES should be considered. Corticosteroid-sparing agents, such as benralizumab show promise for successfully treating these patients.
高嗜酸性粒细胞综合征（HES）是一组极为罕见的疾病。它很少在无心脏受累的情况下出现凝血病。
一名33岁既往健康、无特应性疾病史的男性，出现腹痛、便血，外周嗜酸性粒细胞增多高达10,000个/微升，左右门静脉、肠系膜静脉和脾静脉血栓形成，伴有缺血性结肠炎，导致半结肠切除术和小肠切除术。尽管血液学/肿瘤学、传染病学、风湿病学以及过敏/免疫学对高嗜酸性粒细胞增多的原发性和继发性病因进行了广泛检查，但未发现其他明确病因，该患者被诊断为特发性HES。他的嗜酸性粒细胞增多症通过高剂量口服糖皮质激素（OCS）成功治疗，随后转为使用贝那利珠单抗进行抗IL-5受体治疗。他继续这种治疗超过一年，在使用贝那利珠单抗期间嗜酸性粒细胞增多症或血栓形成未复发。
对于原因不明的凝血病和嗜酸性粒细胞增多症患者，应考虑HES等嗜酸性粒细胞疾病。贝那利珠单抗等糖皮质激素节省剂有望成功治疗这些患者。
