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Case report: Corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome

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...

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Autores principales: Jiang, Dingyuan, Chen, Xueying, Li, Jun, Zhao, Ling, Dai, Huaping
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9877430/
https://www.ncbi.nlm.nih.gov/pubmed/36714123
http://dx.doi.org/10.3389/fmed.2022.1047783
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author Jiang, Dingyuan
Chen, Xueying
Li, Jun
Zhao, Ling
Dai, Huaping
author_facet Jiang, Dingyuan
Chen, Xueying
Li, Jun
Zhao, Ling
Dai, Huaping
author_sort Jiang, Dingyuan
collection PubMed
description 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.
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spelling pubmed-98774302023-01-27 Case report: Corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome Jiang, Dingyuan Chen, Xueying Li, Jun Zhao, Ling Dai, Huaping Front Med (Lausanne) Medicine 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. Frontiers Media S.A. 2023-01-12 /pmc/articles/PMC9877430/ /pubmed/36714123 http://dx.doi.org/10.3389/fmed.2022.1047783 Text en Copyright © 2023 Jiang, Chen, Li, Zhao and Dai. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Jiang, Dingyuan
Chen, Xueying
Li, Jun
Zhao, Ling
Dai, Huaping
Case report: Corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome
title Case report: Corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome
title_full Case report: Corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome
title_fullStr Case report: Corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome
title_full_unstemmed Case report: Corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome
title_short Case report: Corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome
title_sort case report: corticosteroid-resistant acute fibrinous and organizing pneumonia with myelodysplastic syndrome
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9877430/
https://www.ncbi.nlm.nih.gov/pubmed/36714123
http://dx.doi.org/10.3389/fmed.2022.1047783
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