以胃肠道表现为主的特发性嗜酸粒细胞增多综合征9例报告及文献复习

doi:10.13481/j.1671-587x.20160434

摘要/Abstract

摘要：

目的：探讨以胃肠道表现为主的特发性嗜酸粒细胞增多综合征（IHES）的临床特点，提高该病的诊疗水平。方法：回顾性分析符合IHES诊断标准的9例以胃肠道表现为主的IHES患者临床资料、诊治经过及预后。结果：9例患者平均年龄（22.66±12.86）岁，男女比例1.25∶1，以腹痛、腹泻和腹胀为主要临床表现。9例患者外周血和骨髓中嗜酸粒细胞百分比分别为（42.66±19.88）%和（39.33±15.99）%。腹水为渗出液，脱落细胞学检查可见嗜酸粒细胞。胃镜或结肠镜下主要表现为黏膜充血水肿、散在出血斑和暗红色颗粒增生，组织活检可证实有大量嗜酸粒细胞浸润，且结肠最易受累。6例患者腹部CT检查示胃壁或肠壁增厚。9例患者经糖皮质激素治疗后腹部症状消失，腹水吸收。随访2年，2例复发，余7例未见复发。结论：临床医生对胃肠道疾病进行常规诊疗时应考虑IHES的可能，以避免误诊、漏诊及延误治疗。该病确诊后首选类固醇治疗。

关键词: 特发性嗜酸粒细胞增多综合征, 骨髓, 腹腔积液, 类固醇

Abstract:

Objective: To investigate the clinical characteristics of idiopathic hypereosinophilic syndrome (IHES)with gastrointestinal manifestations,and to improve the level of diagnosis and treatment of IHES. Methods: The clinical materials, process of diagnosis and treatment and prognosis of 9 patients diagnosed as IHES with gastrointestinal manifestations were retrospectively analyzed. Results: The average age of 9 patients was (22.66±12.86) years old,and the ratio of male and female was about 1.25∶1. The main clinical manifestations included abdominal pain,diarrhea and abdominal distension.The eosinophil percentages in peripheral blood and bone marrow of the patients were (42.66±19.88)% and (39.33 + 15.99)%,respectively.The ascites exudate cytology examination showed eosinophil infiltrated.The results of gastroscope or colonoscope showed mucosal hyperemia and edema, scattered bleeding spots, and dark red granular hyperplasia;the colon was affected frecuently.The histological biopsy confirmed that the mucosal was infiltrated by eosinophils.The abdominal CT of 6 patients showed that the walls of stomach or bowel were thickened. The abdominal symptoms disappeared,and the ascites was absorpted in 9 patients after the treatment of glucocorticoid.After 2 years of follow up,2 patients had relapse, others had no recurrence. Conclusion: Performing the routine diagnosis and treatment of gastrointestinal diseases, the clinicians should consider the possibility of IHES in order to avoid the misdiagnosis and delayed treatment. When IHES is diagnosed, steroid treatment should be performed in preference.

Key words: idiopathic hypereosinophilic syndrome, bone marrow, ascites, steroids

中图分类号:

R557.5

周长丽, 程洪晶, 白焕焕, 白蔷薇, 孙逊, 孟祥伟. 以胃肠道表现为主的特发性嗜酸粒细胞增多综合征9例报告及文献复习[J]. 吉林大学学报(医学版), 2016, 42(04): 813-816.

ZHOU Changli, CHENG Hongjing, BAI Huanhuan, BAI Qiangwei, SUN Xun, XU Baiguo, MENG Xiangwei. Idiopathic hypereosinophilic syndrome with gastrointestinal manifestations:A report of 9 casesand literature review[J]. Journal of Jilin University Medicine Edition, 2016, 42(04): 813-816.

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