Inoue Daiki, Marumo Satoshi, Ishii Haruyuki, Fukui Motonari
Respiratory Disease Center, Tazuke Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan.
Department of Respiratory Medicine, Kyorin University School of Medicine, Tokyo, Japan.
BMJ Case Rep. 2019 Sep 18;12(9):e231055. doi: 10.1136/bcr-2019-231055.
Myelodysplastic syndrome (MDS) is frequently complicated by pulmonary disease. Here, we describe secondary pulmonary alveolar proteinosis (sPAP) that developed during corticosteroid therapy for organising pneumonia (OP) associated with MDS. A 75-year-old woman with MDS complained of cough for 2 weeks. Chest CT showed bilateral patchy consolidations with reversed halo sign. Bronchoalveolar lavage (BAL) examination showed remarkably increased cell density with an increased lymphocyte proportion. Abnormal radiological findings improved rapidly on administration of systemic corticosteroid under the diagnosis of OP; however, they relapsed a few weeks later. Transbronchial lung biopsy showed periodic acid-Schiff stain-positive amorphous materials. Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in serum and BAL fluid (BALF) were both negative, while GM-CSF level in BALF was elevated. The patient was diagnosed with sPAP. When chest radiological findings show exacerbation during corticosteroid therapy for OP in a patient with MDS, physicians should consider sPAP complication as a differential diagnosis.
骨髓增生异常综合征（MDS）常并发肺部疾病。在此，我们描述了在对与MDS相关的机化性肺炎（OP）进行皮质类固醇治疗期间发生的继发性肺泡蛋白沉积症（sPAP）。一名75岁的MDS女性患者主诉咳嗽2周。胸部CT显示双侧斑片状实变伴反晕征。支气管肺泡灌洗（BAL）检查显示细胞密度显著增加，淋巴细胞比例升高。在诊断为OP的情况下，全身应用皮质类固醇后异常影像学表现迅速改善；然而，几周后病情复发。经支气管肺活检显示高碘酸-雪夫染色阳性的无定形物质。血清和支气管肺泡灌洗液（BALF）中抗粒细胞-巨噬细胞集落刺激因子（GM-CSF）自身抗体均为阴性，而BALF中GM-CSF水平升高。该患者被诊断为sPAP。当MDS患者在接受OP皮质类固醇治疗期间胸部影像学表现加重时，医生应考虑sPAP并发症作为鉴别诊断。
