Fischer T J, Daugherty C, Gushurst C, Kephart G M, Gleich G J
Department of Pediatrics, Children's Hospital Medical Center, University of Cincinnati College of Medicine, OH 45229-2889.
Pediatrics. 1988 Jul;82(1):69-75.
A 9-year-old girl had fever, life-threatening interstitial pneumonia, a vesicular skin rash, parotid and submandibular swelling, and marked blood eosinophilia (WBC count 47,000/microL, 39% eosinophils). Results of lung and skin biopsies showed vasculitis with intense eosinophil infiltration. Immunofluorescence analyses of these biopsies, as well as analysis of tissue from the parotid, lip, and trachea, showed striking deposition of eosinophil granule major basic protein associated with areas of tissue damage. Treatment with glucocorticoids and hydroxyurea produced clinical improvement. The association between tissue damage and deposition of the cytotoxic major basic protein suggests that the eosinophil contributed to the pathophysiology of this disease. Recognition of the capacity of the eosinophil to produce tissue damage can be helpful in the selection of therapy.
一名9岁女孩出现发热、危及生命的间质性肺炎、水疱性皮疹、腮腺和颌下肿胀，以及明显的血液嗜酸性粒细胞增多（白细胞计数47,000/微升，嗜酸性粒细胞占39%）。肺和皮肤活检结果显示血管炎伴强烈嗜酸性粒细胞浸润。对这些活检组织以及腮腺、唇部和气管组织的免疫荧光分析显示，与组织损伤区域相关的嗜酸性粒细胞颗粒主要碱性蛋白有显著沉积。糖皮质激素和羟基脲治疗使临床症状改善。组织损伤与细胞毒性主要碱性蛋白沉积之间的关联表明，嗜酸性粒细胞促成了该疾病的病理生理学过程。认识到嗜酸性粒细胞产生组织损伤的能力有助于治疗方案的选择。
