Acute fibrinous and organizing pneumonia complicated with hemophagocytic lymphohistiocytosis caused by chronic active Epstein-Barr virus infection: a case report.
Acute fibrinous and organizing pneumonia complicated with hemophagocytic lymphohistiocytosis caused by chronic active Epstein-Barr virus infection: a case report.
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慢性活动性EB病毒感染致急性纤维素性机化性肺炎并发噬血细胞性淋巴组织细胞增多症1例报告
DOI：
10.1186/s12879-021-06868-0
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发表时间：
2021-12-04
影响因子：
3.7
通讯作者：
Zhan Q
中科院分区：
文献类型：
--
作者：
Wu X;Wang K;Gao Y;Cai Y;Wang W;Zhong D;Zhan Q
背景：急性纤维性和组织性肺炎（AFOP）是一种罕见的与急性肺损伤相关的肺部疾病。其病因可能是特发性或继发于一系列疾病，包括免疫相关疾病、未分类结缔组织疾病、造血干细胞移植、感染、血液系统疾病和药物引起的肺毒性。我们首次报告一例慢性活动性eb病毒（CAEBV）感染引起的AFOP合并嗜血球性淋巴组织细胞增多症（HLH）。病例介绍一名64岁男性，因发烧和呼吸困难2周入院。患者表现为血清转氨酶水平升高，脾肿大，红细胞和血小板进行性减少，高铁蛋白血症，低纤维蛋白原血症，可溶性白介素-2受体（sCD25）升高。胸部计算机断层扫描显示双肺多发斑片状实变，纵隔和肺门多发淋巴结病变。血清VCA-IgG抗体阳性，外周血EBV- dna升高，肺泡灌洗液中检测到EBV核酸。肺组织病理学显示肺泡内纤维蛋白和组织性肺炎占优势。骨髓涂片及活检均可见噬血细胞。用ebv编码RNA （EBER）探针原位杂交检测肺组织和骨髓中的EBV-DNA。住院50天后，患者肺部及血象好转。结论本研究报告1例免疫功能正常的成人由CAEBV引起的AFOP合并HLH，提示AFOP可能是CAEBV引起的罕见但严重的并发症，糖皮质激素治疗可改善短期预后。
BackgroundAcute fibrinous and organizing pneumonia (AFOP) is a rare lung condition that is associated with acute lung injury. Its etiology may be idiopathic or secondary to a series of conditions, including immune-related diseases, unclassified connective tissue diseases, hematopoietic stem cell transplantation, infections, hematological diseases and drug induced lung toxicity. We report for the first time a case of AFOP complicated with hemophagocytic lymphohistiocytosis (HLH) caused by chronic active Epstein-Barr virus (CAEBV) infection.Case presentationA 64-year-old man was admitted with a complaint of fever and dyspnea for 2 weeks. The patient presented with elevated serum aminotransferase levels, splenomegaly, progressive decrease of red blood cells and platelets, hyperferritinemia, hypofibrinogenemia, and elevated of Soluble interleukin-2 receptor (sCD25). His chest computed tomography (CT) scan revealed multiple patchy consolidation in both lungs and multiple lymphadenopathy in the mediastinum and hilum. The serology for antibodies of VCA-IgG was positive, EBV-DNA in peripheral blood was elevated, and EBV nucleic acid was detected in the alveolar lavage fluid. Histopathology of the lung tissue showed a dominant of intra-alveolar fibrin and organizing pneumonia. Hemophagocytic cells was found in the bone marrow smear and biopsy. EBV-DNA was detected in lung tissue and bone marrow using in situ hybridization with an EBV-encoded RNA (EBER) probe. After 50 days of hospitalization, he was improved in lung and hemogram.ConclusionWe report a case of AFOP with HLH caused by CAEBV in an immunocompetent adult, suggesting that AFOP may be a rare but serious complication caused by CAEBV, and glucocorticoid therapy may improve short-term prognosis.
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