San José A, Bosch J A, Candell J, Pinar J, Torrabadella M, Vallespí T, Oristrell J, Vilardell M
Servicio de Medicina Interna, Hospital General Universitario Vall d'Hebron, Barcelona.
Med Clin (Barc). 1992 Feb 8;98(5):161-5.
The idiopathic hypereosinophilic syndrome (IHS) is a rare entity of unknown etiology. Hematological and cardiac involvement is predominant. A series of 12 patients with this syndrome, initiated in 1982, is described.
Cardiological study by repeated echocardiograms and hematological study in peripheral blood and bone marrow upon initiation of the disease were performed.
Median follow up was of 48 +/- 31 months. Males predominated (75%) with mean age being 55 +/- 15 years. The principal organs or systems involved were the heart (50%) and the nervous system (41%). Of the 6 cases with cardiac involvement only 2 had clinical manifestations. The remaining 4 patients were diagnosed from echocardiographic changes with the principal alterations observed being: atypical occupation of the ventricles, endocardial thickening and mitral and tricuspid subvalvular cumulus. Echocardiographic follow-up only showed changes in one case. Hematological involvement was characterized by moderate leukocytosis with hypereosinophilia formed by mature eosinophils, conservation of other hematopoietic series, absence of blasts in peripheral blood, finding suggestive of diseosinophilopoiesis and appearance of myelofibrosis and cytogenetic alterations. Survival at four years was 58%.
In the series studied cardiac involvement is frequent, being principally diagnosed in a subclinical phase and with a very slow echocardiographic evolution. At a hematological level changes typical in myelodysplastic syndromes and myeloid leukemia were observed.
特发性嗜酸性粒细胞增多综合征（IHS）是一种病因不明的罕见疾病。血液系统和心脏受累较为常见。本文描述了自1982年起收治的12例该综合征患者。
在疾病初发时通过反复超声心动图进行心脏检查，并对外周血和骨髓进行血液学检查。
中位随访时间为48±31个月。男性占多数（75%），平均年龄为55±15岁。主要受累的器官或系统为心脏（50%）和神经系统（41%）。在6例心脏受累患者中，仅有2例有临床表现。其余4例通过超声心动图改变确诊，主要观察到的改变为：心室非典型占位、心内膜增厚以及二尖瓣和三尖瓣瓣下肿物。超声心动图随访仅1例显示有变化。血液系统受累的特征为中度白细胞增多伴成熟嗜酸性粒细胞形成的嗜酸性粒细胞增多、其他造血系列正常、外周血无原始细胞、提示嗜酸性粒细胞生成异常的表现以及骨髓纤维化和细胞遗传学改变的出现。4年生存率为58%。
在所研究的病例系列中，心脏受累很常见，主要在亚临床阶段被诊断出来，且超声心动图演变非常缓慢。在血液学层面，观察到了骨髓增生异常综合征和髓系白血病的典型变化。
