Spry C J, Davies J, Tai P C, Olsen E G, Oakley C M, Goodwin J F
Q J Med. 1983 Winter;52(205):1-22.
Fifteen patients with the hypereosinophilic syndrome were studied during a period of 6.5 years. The mean age at onset was 36 years. Two were female. The commonest presenting symptoms were nocturnal sweating with or without severe coughing attacks, symptoms of cardiovascular disease, anorexia and weight loss, neurological and gastrointestinal symptoms and itching with or without skin lesions. The mean blood eosinophil counts at presentation were 20.1 X 10(9)/l. Eight patients had previous allergic or parasitic disease which could have predisposed them to the development of hypereosinophilia. Eight patients had raised serum immunoglobulin levels: IgM in five, IgE in four and IgG in one. Five of nine patients had raised serum eosinophil cationic protein levels. Episodes of clinical relapse occurred with increased white blood counts and were treated with prednisolone and cytotoxic drugs. Thrombotic and embolic complications developed in 10 patients, despite treatment with anticoagulants and inhibitors of platelet function, and were the cause of death in three. Two patients with severe endomyocardial fibrosis responded well to cardiac surgery, and a third required emergency mitral valve replacement. The 12 surviving patients have lived 0.8-11.5 years (mean 4.4), since the onset of their illness. It is concluded that the hypereosinophilic syndrome has distinctive features with an episodic course. The principal complications affect the cardiovascular system, especially endomyocardial fibrosis and thromboembolic occlusion of large and small blood vessels in many organs. Although treatment is usually effective in overcoming relapses, the underlying disease process appears to be unaffected. Despite this, patients can have prolonged periods of remission and may survive for many years.
在6.5年的时间里，对15例高嗜酸性粒细胞综合征患者进行了研究。发病时的平均年龄为36岁。其中2例为女性。最常见的症状是盗汗，伴有或不伴有严重咳嗽发作、心血管疾病症状、厌食和体重减轻、神经和胃肠道症状以及伴有或不伴有皮肤病变的瘙痒。就诊时的平均血液嗜酸性粒细胞计数为20.1×10⁹/L。8例患者既往有过敏性或寄生虫病，可能使他们易患高嗜酸性粒细胞增多症。8例患者血清免疫球蛋白水平升高：5例IgM升高，4例IgE升高，1例IgG升高。9例患者中有5例血清嗜酸性粒细胞阳离子蛋白水平升高。临床复发时白细胞计数增加，用泼尼松龙和细胞毒性药物治疗。尽管使用了抗凝剂和血小板功能抑制剂，但仍有10例患者发生血栓形成和栓塞并发症，3例患者因此死亡。2例严重心内膜纤维化患者对心脏手术反应良好，第3例患者需要紧急二尖瓣置换术。自发病以来，12例存活患者已存活0.8 - 11.5年（平均4.4年）。结论是，高嗜酸性粒细胞综合征具有独特的特征，病程呈发作性。主要并发症累及心血管系统，尤其是心内膜纤维化以及许多器官的大小血管血栓栓塞性阻塞。尽管治疗通常能有效克服复发，但潜在的疾病进程似乎未受影响。尽管如此，患者仍可获得较长时间的缓解，并且可能存活多年。
