LAUSR

Myelodysplastic syndromes (MDS) often develop pulmonary complications such as organising pneumonia (OP) during the clinical course. OP is defined histopathologically by intra-alveolar buds of granulation tissue. OP can be either idiopathic or secondary which is commonly associated with autoimmune disorders infections drugs and malignancy. Although corticosteroids are effective, relapses are common after treatment cessation. MDS accompanied by OP has been reported as case reports to date. However, little is known about its frequency, pathogenesis, and impacts on clinical features of MDS. To address these questions, we retrospectively investigated 159 patients with MDS in our institute. We included in the samples our patients with MDS whom we diagnosed from 1 January 2010 to 30 September 2017. The detailed methods, such as study design, patients, and statistical analysis are described in Data S1. Five patients (3 1%) developed pathologically diagnosed OP. Their chest imaging examinations are shown in Fig 1. Subpleural distributed consolidation with air bronchogram and ground-glass opacities were observed in all cases. Bilateral or multiple recurrent or migratory lung lesions were observed over time. The specimens of all patients obtained by transbronchial lung biopsy showed organised granulation tissue consisting of myofibroblasts and inflammatory cells within the alveolar space (Figure S1). Their clinical findings are shown in Table I. The mean age at the time of diagnosis of OP was 73 years. Fever was observed in all cases at the onset of OP. The median (range) time to onset of OP after MDS diagnosis was 8 5 (0 5–17) months. All patients were categorised as high-risk MDS. Interestingly cytogenetic studies revealed that three out of the five patients had a der(1;7)(q10; p10) abnormality. Subsequently we investigated the association between OP and karyotype der (1;7)(q10;p10). Among the 159 patients with MDS analysed, 11 (6 9%) harboured der(1;7)(q10;p10). Within this group of patients who harboured der(1;7)(q10;p10), three developed OP. The frequency of OP development in patients with MDS with der(1;7)(q10;p10) was 27 3% (three out of 11 patients). In contrast, only two of the 148 patients (1 4%) without der (1;7)(q10;p10) developed OP. The frequency of OP among the patients with der(1;7)(q10;p10) was higher than that among patients without der(1;7)(q10;p10) (odds ratio of 25 70: 95% confidence interval 2 582–347 4). These findings suggest that there is a relationship between MDS with der (1;7)(q10;p10) and OP. Regarding the treatments of MDS, two patients received azacitidine prior to the development of OP. The effect of azacitidine on MDS was that it stabilised the disease condition. After the onset of OP, both patients received only supportive care for MDS. The other three patients received only supportive care for MDS during the course. Treatment with prednisolone (PSL) was initiated against OP in all patients. Although PSL improved the symptoms transiently, there was lethal OP relapse in all cases after the reduction of PSL. All the patients with OP in the present study died from the progression of lung disease. The overall survival (OS) of patients with MDS after diagnosis with OP