A case of secondary pulmonary alveolar proteinosis, but prior to myelodysplastic syndrome.
A case of secondary pulmonary alveolar proteinosis, but prior to myelodysplastic syndrome.
复制标题
继发性肺泡蛋白沉积症一例，但先于骨髓增生异常综合征
DOI：
10.1002/rcr2.29
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发表时间：
2013-12
影响因子：
0.8
通讯作者：
Wu, Xueling
中科院分区：
文献类型：
--
作者：
Zhao, Yunfeng;Xiong, Wei;Wu, Xueling
肺泡蛋白沉积症（PAP）是一种罕见的肺部疾病。我们在此报告第一例PAP发生2年前骨髓增生异常综合征（MDS）。一个34岁的中国人提出了发展反复咳嗽和呼吸急促。计算机断层扫描显示磨玻璃影伴小叶间隔增厚。组织学检查显示肺泡内充满嗜酸性致密均匀物质。该沉淀物具有细颗粒外观。嗜酸性物质为高碘酸-希夫反应阳性。患者被诊断为PAP。两年后，他因持续1个月的头晕住院。血液学检查显示白色血细胞为2700，血红蛋白为7.4 g/dL，血小板计数为21，000血小板/mm 3。在骨髓活检和组织病理学检查后，他被诊断为MDS伴难治性贫血和原始细胞过多。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。
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/mm3. 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.