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伴有骨髓增生异常综合征和卡氏肺孢子菌肺炎的急性纤维蛋白性和机化性肺炎:病例报告。

Acute fibrinous and organizing pneumonia with myelodysplastic syndrome and pneumocystis jiroveci pneumonia: a case report.

机构信息

Department of Pulmonary and Critical Care Medicine, Nanjing Drum Tower Hospital, Clinical College of Nanjing Medical University, Nanjing, China; Department of Critical Care Medicine, The Second Hospital of Nanjing, Nanjing University of Chinese Medicine, Nanjing, China.

Department of Pulmonary and Critical Care Medicine, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, China.

出版信息

Ann Palliat Med. 2021 Jul;10(7):8396-8402. doi: 10.21037/apm-20-2344. Epub 2021 Apr 15.

DOI:10.21037/apm-20-2344
PMID:33894702
Abstract

Acute fibrinous and organizing pneumonia (AFOP) is an unusual pathological pattern which is characterized by intra-alveolar deposition of fibrin (fibrin ball) and organizing pneumonia in a scattered distribution, and the pathological diagnosis plays an irreplaceable role in the diagnosis. Most Patients cannot confirm etiology, till now, known etiology included connective tissue disease, infection, environmental and occupational exposure, drugs, organ transplant, and tumor. It can be divided into acute and subacute subtype according to the extent of progress. The most common symptoms of AFOP were fever, cough, and dyspnea. Bilateral consolidations and ground-glass opacities (GGO) usually can be seen on chest CT images. At present, the treatment protocol for AFOP has not reached a consensus Glucocorticoid, immunosuppressants, stem cell transplantation or lung transplantation may contribute to improved clinical outcome. Here, we report a case of AFOP with myelodysplastic syndrome and pneumocystis jiroveci pneumonia (PJP). After treatments of glucocorticoid, immunosuppressant, chemotherapy, antibiotics and blood transfusion, the patient's clinical symptoms, peripheral blood test, and imaging findings were obviously improved. In this case, we consider the AFOP was caused by MDS and the immunodeficiency after chemotherapy lead to secondary PJP. This typical case highlights the importance of appropriate therapy for coexisted diseases of those patients with refractory AFOP.

摘要

急性纤维蛋白性和机化性肺炎(AFOP)是一种不常见的病理模式,其特征是肺泡内纤维蛋白(纤维蛋白球)沉积和机化性肺炎呈散在分布,病理诊断在诊断中起着不可替代的作用。大多数患者无法确定病因,到目前为止,已知的病因包括结缔组织疾病、感染、环境和职业暴露、药物、器官移植和肿瘤。根据进展程度,可分为急性和亚急性亚型。AFOP 最常见的症状是发热、咳嗽和呼吸困难。胸部 CT 图像通常可见双侧实变和磨玻璃影(GGO)。目前,AFOP 的治疗方案尚未达成共识,糖皮质激素、免疫抑制剂、干细胞移植或肺移植可能有助于改善临床结局。在这里,我们报告了一例伴有骨髓增生异常综合征和卡氏肺孢子虫肺炎(PJP)的 AFOP 病例。经过糖皮质激素、免疫抑制剂、化疗、抗生素和输血治疗,患者的临床症状、外周血检查和影像学表现明显改善。在这种情况下,我们认为 AFOP 是由 MDS 引起的,化疗后免疫功能低下导致继发性 PJP。这个典型病例强调了对难治性 AFOP 患者共存疾病进行适当治疗的重要性。

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