复杂性未定类嗜酸粒细胞增多综合征一例

Complex undefined hypereosinophilic syndrome： a case report

患者男，41岁，全身泛发红斑、丘疹，偶颜面水肿伴痒1年半，家族中无类似患者。实验室检查：外周血嗜酸粒细胞增多，骨髓活检：嗜酸粒细胞比例增高，多次粪检未见寄生虫卵，血多种寄生虫抗体阴性，心肌酶升高，抗中性粒细胞胞质抗体阴性，FIP1L1／PDGFRα融合基因阴性；心脏彩色超声见轻度三尖瓣和肺动脉瓣关闭不全，右房轻度增大。诊断为复杂性未定类嗜酸粒细胞增多综合征，予泼尼松联合环磷酰胺治疗后，症状控制理想。

A 41-year-old male patient presented with generalized pruritic erythema and papules with occasional facial edema for one and a half years. The condition was absent in other members in his family. Peripheral blood film demonstrated an elevated count of morphologically normal eosinophils. Bone marrow biopsy revealed an increase in the proportion of eosinophils. Parasite eggs were undetected in stool samples by multiple examinations, and the blood sample was negative for antibodies against many parasites, antineutrophil cytoplasmic antibodies （ANCA）, and FIP1L1/PDGFRα fusion gene. The level of blood myocardial enzyme was elevated. Colour Doppler ultrasound examination of the heart showed mild tricuspid incompetence, pulmonary valve incompetence and slight dilatation of the right atrium. The patient was diagnosed with complex undefined hypereosinophilic syndrome, which responded well to oral prednisone and intravenous cyclophosphamide.