Journal article

Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan

Haruyuki Ishii, John F Seymour, Ryushi Tazawa, Yoshikazu Inoue, Naoyuki Uchida, Aya Nishida, Yoshihito Kogure, Takeshi Saraya, Keisuke Tomii, Toshinori Takada, Yuko Itoh, Masayuki Hojo, Toshio Ichiwata, Hajime Goto, Koh Nakata

BMC Pulmonary Medicine | BIOMED CENTRAL LTD | Published : 2014


BACKGROUND: Secondary pulmonary alveolar proteinosis (sPAP) is a very rare lung disorder comprising approximately 10% of cases of acquired PAP. Hematological disorders are the most common underlying conditions of sPAP, of which 74% of cases demonstrate myelodysplastic syndrome (MDS). However, the impact of sPAP on the prognosis of underlying MDS remains unknown. The purpose of this study was to evaluate whether development of sPAP worsens the prognosis of MDS. METHODS: Thirty-one cases of sPAP and underlying MDS were retrospectively classified into mild and severe cases consisting of very low-/low-risk groups and intermediate-/high-/very high-risk groups at the time of diagnosis of MDS, acco..

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Awarded by Grants-in-Aid for Scientific Research

Funding Acknowledgements

The authors thank Dr. Sato J., Matsui T., Yoshioka Y., Shijubo N., Hara H., Amitani K., Takahashi S., Fukuno K., Handa T., Nakatsue T., Saito Y., Asai Y., Ohkochi Y., Saito Y., Yasui T., Baba M., Yanai H., Tanino T., Setoguchi Y., Yamamoto S., Ohkochi S., Morita M., Hashimoto K., Matsumoto K., and Komeno T. for providing patient samples and information. We also thank Takizawa J., Sone H., Akira M., and Kitamura N. for valuable discussions and suggestions, and Mori M. for technical help. Our work was partially supported by a grant from Category C23591160, B24390208, and B12023059 from the Japan Society for the Promotion of Science. This research was also supported by a grant from the Ministry of Health, Labour, and Welfare (H24, Nanchi-ippan-035, and H24 Rinken Sui-003). The sponsor had no role in the study design, data collection and analysis, and manuscript preparation.