嗜酸性白血球過多症候群在一台灣醫學中心的病例分析

Hypereosinophilic syndrome in a medical center in Taiwan – an analysis of fourteen cases

目的：嗜酸性白血球過多症候群是一種嗜酸性血球過度製造的疾病，特徵是經由嗜酸性白血球浸潤及相關介質造成多重器官的損害。本研究的目的是探討在台灣的單一醫學中心、嗜酸性白血球過多症候群的病人的臨床表現、器官侵犯的比例、治療及臨床結果。方法：本研究收集從1986年12月到2007年7月因為嗜酸性白血球過多症候群而住院的病人。回溯性地記錄病人的初發症狀、器官侵犯的程度、血液中總嗜酸性白血球的量、治療藥物及臨床結果。結果：共14個病人診斷為嗜酸性白血球過多症候群，平均年齡為44.1歲，男女比例為3.7比1，從症狀發生至疾病診斷平均時間為14.3個月。最常見的初發症狀依序為皮膚疹 （50%）、腸胃道症狀 （腹痛及腹瀉、36%）及發燒 （36%）。最常侵犯的器官依序為皮膚 （50%）、腸胃道 （43%）及周邊血管 （36%）。心臟侵犯的比例 （14%）較西方國家為低。所有的病人一開始都接受類固醇治療，大部分的病人需要長期使用口服類固醇及免疫抑制劑治療。結論：本研究提供了台灣的嗜酸性白血球過多症候群的臨床特徵，皮膚及腸胃系統是最常被侵犯的器官。高度的臨床懷疑及儘速對受侵犯器官做組織切片檢查有助診斷此少見的疾病。

Objectives: Hypereosinophilic syndromes are disorders with persistent overproduction of eosinophils. The diseases are characterized by damage to multiple organs due to eosinophilic infiltration and mediator release. This study aimed to investigate the clinical manifestation, frequency of organ involvement, treatment and outcome among patients with hypereosinophilic syndrome in a single medical center in Taiwan. Material and Methods: Patients admitted to our hospital due to hypereosinophilic syndrome from December 1986 to July 2007 were enrolled in this study. The initial symptoms, spectrum of organ involvement, total eosinophil count, therapeutic regimen and clinical outcome were retrospectively reviewed and documented. Result: A total of 14 cases were diagnosed with hypereosinophilic syndrome. The average age was 44.1 years old. The male/female ratio was 3.7:1. The average duration of symptom onset to diagnosis was 14.3 months. The most common initial symptoms were skin rashes (50%), followed by gastrointestinal symptoms (abdominal pain and diarrhea, 36%) and fever (36%). The most frequently involved organ was cutaneous involvement (50%), followed by gastrointestinal involvement (43%) and peripheral vascular involvement (36%). The frequency of cardiovascular involvement was lower (14%) than in western countries. All patients received corticosteroid treatment initially. Most patients required long-term oral prednisolone and immunosuppressive therapy. Conclusion: This study displays clinical characteristics of hypereosinophilic syndrome in Taiwan. Cutaneous and gastrointestinal systems are the most frequently involved organs. A high clinical suspicious index and early biopsy of the involved organ helps in diagnosis of this rare disease.