Nazir Lubna, Riaz Amir, Alam Hamza
Department of Rheumatology, Liaquat National Hospital, Karachi, Pakistan.
J Coll Physicians Surg Pak. 2019 Jun;29(6):S59-S61. doi: 10.29271/jcpsp.2019.06.S59.
Hyper eosinophilic syndrome (HES) is a rare condition with a potential for morbidity and mortality, if left untreated. Therefore, it is important to highlight it, as often these cases are misdiagnosed and mismanaged, specially when presenting with an atypical initial presentation. This case report describes an unusual initial clinical presentation of HES. Patient was a 75-year lady presenting to Rheumatology Clinic with short duration of fever and polyarthritis. Joint aspiration showed purulent fluid with a cell count of 61,000/mm3 with predominant neutrophils and eosinophils. Her peripheral blood also showed a high white blood cell (WBC) count (80,700/mm3 with 73% eosinophils). Her workup for eosinophilic leukemia was negative, so a diagnosis of HES was made. She was initiated on corticosteroids and hydroxycarbamide as first-line therapy. Unfortunately, the patient was unresponsive to steroids with her WBC count rising to 130,000/mm3 and her clinical course was complicated by cardiac failure and peripheral neuropathy. Improvement in arthritis and peripheral eosinophilia was noted after she was given imatinib and stabilised clinically.
高嗜酸性粒细胞综合征(HES)是一种罕见疾病,若不治疗,有发病和死亡风险。因此,强调这一点很重要,因为这些病例常被误诊和误治,尤其是在初始表现不典型时。本病例报告描述了HES一种不寻常的初始临床表现。患者为一名75岁女性,因发热和多关节炎病程较短就诊于风湿病诊所。关节穿刺抽出脓性液体,细胞计数为61,000/mm³,以中性粒细胞和嗜酸性粒细胞为主。她的外周血白细胞(WBC)计数也很高(80,700/mm³,嗜酸性粒细胞占73%)。她的嗜酸性粒细胞白血病检查结果为阴性,因此诊断为HES。她开始接受皮质类固醇和羟基脲作为一线治疗。不幸的是,患者对类固醇无反应,白细胞计数升至130,000/mm³,临床病程因心力衰竭和周围神经病变而复杂化。给予伊马替尼后,关节炎和外周嗜酸性粒细胞增多有所改善,临床病情稳定。