Jiang Dingyuan, Chen Xueying, Li Jun, Zhao Ling, Dai Huaping
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Diseases, Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Institute of Respiratory Medicine, Chinese Academy of Medicine Sciences, Beijing, China.
Laboratory Department of Pathology, China-Japan Friendship Hospital, Beijing, China.
Front Med (Lausanne). 2023 Jan 12;9:1047783. doi: 10.3389/fmed.2022.1047783. eCollection 2022.
Acute fibrinous and organizing pneumonia (AFOP) is a lung disease with an unusual pathological pattern. The definitive diagnosis of AFOP relies on pathological evidence of intra-alveolar fibrin exudate, lymphoplasmacytic infiltrate, and the absence of a hyaline membrane. Furthermore, its etiology is difficult to confirm, and corticosteroids are usually effective. Herein, we report the case of a young male who presented with high fever, hemocytopenia, and consolidation in both lungs. The initial misdiagnosis was community-acquired pneumonia. Subsequently, a lung biopsy revealed abundant fibrin and fibroblast exudates in the alveolar spaces, indicating AFOP. In addition, bone marrow biopsy and karyotype analysis demonstrated that the patient simultaneously had myelodysplastic syndrome (MDS) and hemophagocytic lymphohistiocytosis. In this case, the AFOP was considered secondary to MDS; however, the disease did not respond to glucocorticoid treatment or chemotherapy. Hence, AFOP should be considered in patients with underlying hematological diseases, and early identification and diagnosis are important. Furthermore, the management of patients with severe AFOP requires further investigation.
急性纤维素性机化性肺炎(AFOP)是一种具有特殊病理模式的肺部疾病。AFOP的确诊依赖于肺泡内纤维蛋白渗出、淋巴细胞和浆细胞浸润且无透明膜形成的病理证据。此外,其病因难以明确,而皮质类固醇通常有效。在此,我们报告一例年轻男性病例,该患者表现为高热、血细胞减少和双肺实变。最初误诊为社区获得性肺炎。随后,肺活检显示肺泡腔内有大量纤维蛋白和成纤维细胞渗出物,提示为AFOP。此外,骨髓活检和核型分析表明该患者同时患有骨髓增生异常综合征(MDS)和噬血细胞性淋巴组织细胞增生症。在本病例中,AFOP被认为是继发于MDS;然而,该疾病对糖皮质激素治疗或化疗均无反应。因此,对于患有潜在血液系统疾病的患者应考虑AFOP,早期识别和诊断很重要。此外,重症AFOP患者的管理需要进一步研究。