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Respiratory investigation · Jan 2014
Review Case ReportsClinical features of three cases with pulmonary alveolar proteinosis secondary to myelodysplastic syndrome developed during the course of Behçet's disease.
- Tomohiro Handa, Takeshi Nakatsue, Motoo Baba, Toshinori Takada, Koh Nakata, and Haruyuki Ishii.
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, 54 Kawaharacho Shogoin, Sakyo-ku, Kyoto 606-8507, Japan. Electronic address: hanta@kuhp.kyoto-u.ac.jp.
- Respir Investig. 2014 Jan 1; 52 (1): 75-9.
AbstractWe have previously reported that myelodysplastic syndrome (MDS) is the most common underlying disease in cases of secondary pulmonary alveolar proteinosis (PAP). Here, we present 3 MDS cases in which PAP developed during the course of Behçet's disease (BD). All patients carried trisomy 8 in the bone marrow. Chest HRCT scans showed variable distribution of ground glass opacities, but none of the scans showed so called "crazy paving appearance". Two patients with intestinal BD who underwent potent immunosuppressive therapy died of sepsis. These findings demonstrate that PAP secondary to MDS may be occasionally associated with BD. Copyright © 2013 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.
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