Kawashima A, Kimura A, Katsuda S, Sumita R, Yachie A, Nonomura A, Nakanishi I
Department of Pathology, School of Medicine, Kanazawa University, Japan.
Pathol Int. 1995 Jan;45(1):66-74. doi: 10.1111/j.1440-1827.1995.tb03381.x.
Three siblings with eosinophilia who developed pulmonary hypertension are reported. They consisted of a 3 year old boy (case 1), a 9 year old boy (case 2) and a 13 year old girl (case 3), all of whom died within an 18 month period of severe episodic attacks of pulmonary hypertension and the resultant low cardiac output. Marked peripheral eosinophilia was found in cases 1 and 2, and mild eosinophilia in case 3. Open lung biopsy of case 1 revealed pulmonary arteritis with massive eosinophilic infiltration and intimal thickening of muscular arteries of 300-1500 microns in diameter. At autopsy, cases 2 and 3 showed almost similar findings, comprising widespread obliteration of the pulmonary arteries by concentric intimal thickening, medial hypertrophy and recanalized thrombi of arterioles. Rarely, there were foci of granulomas in the thickened intima surrounding birefringent foreign bodies. There were small areas of infarction in the lungs and heart due to arterial thrombi. Vascular lesions other than those in the lungs were mild and almost limited to the branches of the coronary arteries. Therefore, the present cases appear to be a single disease of pulmonary hypertension secondary to endothelial injury and the resultant intimal fibrosis probably evoked by toxic substances, although such agents were not confirmed.
报道了3例患有嗜酸性粒细胞增多症并发展为肺动脉高压的兄弟姐妹。他们包括一名3岁男孩（病例1）、一名9岁男孩（病例2）和一名13岁女孩（病例3），所有患儿均在18个月内死于严重的肺动脉高压发作及由此导致的低心输出量。病例1和病例2发现明显的外周嗜酸性粒细胞增多，病例3为轻度嗜酸性粒细胞增多。病例1的开胸肺活检显示为肺动脉炎，伴有大量嗜酸性粒细胞浸润以及直径为300 - 1500微米的肌性动脉内膜增厚。尸检时，病例2和病例3显示出几乎相似的 findings，包括肺动脉因同心性内膜增厚、中膜肥厚和小动脉再通血栓而广泛闭塞。在增厚的内膜中，围绕双折射异物很少有肉芽肿灶。由于动脉血栓，肺和心脏有小面积梗死。肺部以外的血管病变较轻，几乎仅限于冠状动脉分支。因此，尽管未证实有毒性物质，但目前这些病例似乎是一种继发于内皮损伤的单一肺动脉高压疾病，内膜纤维化可能是由有毒物质引起的。
