Tsutsumi Yutaka, Tanaka Junji, Saito Shin, Tanaka Yasumasa, Kawamura Takahito, Obara Shinji, Noto Satosi, Shimoyama Norihiko, Asaka Masahiro, Imamura Masahiro, Masauzi Nobuo
Department of Internal Medicine, Hakodate Municipal Hospital 1-10-1, Minato-Cho, Hakodate 041-8680, Japan.
Leuk Lymphoma. 2003 Jun;44(6):1049-52. doi: 10.1080/1042819031000063453.
Hematological diseases are often accompanied by respiratory disorders. Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by excessive accumulation of surfactant in the alveolar space. We describe a case of PAP complicated by myelofibrosis following essential thrombocythemia. The patient developed high fever, respiratory failure, and leuko-erythroblastosis during the progressive course of PAP. These symptoms were alleviated by prednisolone. The level of serum IL-6 was elevated when PAP was progressing rapidly. This may explain why the symptoms were alleviated by the steroids.
血液系统疾病常伴有呼吸系统疾病。肺泡蛋白沉积症（PAP）是一种罕见疾病，其特征是肺泡腔内表面活性物质过度积聚。我们描述了1例原发性血小板增多症后并发骨髓纤维化的PAP病例。在PAP的进展过程中，患者出现高热、呼吸衰竭和幼稚粒-幼红细胞血症。这些症状通过泼尼松龙得到缓解。当PAP迅速进展时，血清IL-6水平升高。这可能解释了为何使用类固醇可缓解症状。
