Zhao Yunfeng, Xiong Wei, Wu Xueling
Department of Respiratory Disease, Shanghai Pudong New Area Gongli Hospital Shanghai, China.
Institute of Respiratory Medicine, Xinqiao Hospital, Third Military Medical University Chongqing, China.
Respirol Case Rep. 2013 Dec;1(2):58-61. doi: 10.1002/rcr2.29. Epub 2013 Oct 31.
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder. We herein report the first case of PAP that happened 2 years before myelodysplatic syndrome (MDS). A 34-year-old Chinese presented with a developed recurrent cough and shortness of breath. Computed tomography scan disclosed ground-glass opacities with interlobular septal thickening. Histological examination showed eosinophilic dense homogenous material filling in the alveolar. This precipitate had a fine granular appearance. The eosinophilic material was periodic acid-Schiff reaction-positive. The patient was diagnosed with PAP. Two years later he was admitted to a hospital because of dizziness of 1-month duration. Hematological examination showed white blood cells was 2700, hemoglobin was 7.4 g/dL, and platelet count was 21,000 platelets/mm(3). Following bone marrow biopsy and histopathologic examination, he was diagnosed with MDS with refractory anemia and excess blasts. So for PAP patients, follow-up tests should be considered in order to find any possible underlying disease.
肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病。我们在此报告首例在骨髓增生异常综合征(MDS)出现前2年发生的PAP病例。一名34岁的中国人出现反复咳嗽和气短加重症状。计算机断层扫描显示磨玻璃影伴小叶间隔增厚。组织学检查显示嗜酸性致密均匀物质填充于肺泡内。此沉淀物呈细颗粒状外观。嗜酸性物质过碘酸-雪夫反应呈阳性。该患者被诊断为PAP。两年后,他因持续1个月的头晕入院。血液学检查显示白细胞为2700,血红蛋白为7.4 g/dL,血小板计数为21,000/mm³。经骨髓活检和组织病理学检查,他被诊断为伴有难治性贫血和过多原始细胞的MDS。因此,对于PAP患者,应考虑进行随访检查以发现任何可能的潜在疾病。