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J Chest Surg

Published online October 22, 2024

Copyright © Journal of Chest Surgery.

Histological Findings of ETosis in Hermansky-Pudlak Syndrome with Pulmonary Fibrosis: A Follow-Up Case Report

Sergio Michael Navarro , M.D., M.B.A.1, Aneel Ashrani , M.D., M.S.2,3, Myung Soo Park , M.D., M.S.1, Dong Chen , M.D., Ph.D.2

Departments of 1Surgery, 2Laboratory Medicine and Pathology, and 3Medicine, Mayo Clinic, Rochester, MN, USA

Correspondence to:Dong Chen
Tel 1-507-284-2511
Fax 1-507-284-8713
E-mail Chen.dong@mayo.edu
ORCID
https://orcid.org/0000-0001-7627-9560

Received: June 3, 2024; Revised: August 28, 2024; Accepted: September 6, 2024

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Hermansky-Pudlak syndrome (HPS), both alone and in conjunction with pulmonary fibrosis (HPS-PF), is a rare, genetically heterogeneous, autosomal recessive disorder that affects multiple organs, including the lungs. In cases of HPS-PF, pulmonary fibrosis is preceded by local inflammation. We present a case of HPS-PF that exhibited histological evidence of extracellular traps (ETs) ensnaring macrophages, leading to cell death in a process known as ETosis. To our knowledge, ETosis has not been previously reported in the HPS-PF population and may represent a mechanism by which pulmonary fibrosis develops in these patients. Further research is needed to explore the potential connection between ETosis and HPS-PF, as this understanding could offer insights into the disease mechanism and pave the way for the development of novel treatment modalities.

Keywords: Extracellular traps, Hermansky-Pudlak syndrome, Pulmonary fibrosis, Case report

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J Chest Surg

Published online October 22, 2024

Copyright © Journal of Chest Surgery.

Histological Findings of ETosis in Hermansky-Pudlak Syndrome with Pulmonary Fibrosis: A Follow-Up Case Report

Sergio Michael Navarro , M.D., M.B.A.1, Aneel Ashrani , M.D., M.S.2,3, Myung Soo Park , M.D., M.S.1, Dong Chen , M.D., Ph.D.2

Departments of 1Surgery, 2Laboratory Medicine and Pathology, and 3Medicine, Mayo Clinic, Rochester, MN, USA

Correspondence to:Dong Chen
Tel 1-507-284-2511
Fax 1-507-284-8713
E-mail Chen.dong@mayo.edu
ORCID
https://orcid.org/0000-0001-7627-9560

Received: June 3, 2024; Revised: August 28, 2024; Accepted: September 6, 2024

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Hermansky-Pudlak syndrome (HPS), both alone and in conjunction with pulmonary fibrosis (HPS-PF), is a rare, genetically heterogeneous, autosomal recessive disorder that affects multiple organs, including the lungs. In cases of HPS-PF, pulmonary fibrosis is preceded by local inflammation. We present a case of HPS-PF that exhibited histological evidence of extracellular traps (ETs) ensnaring macrophages, leading to cell death in a process known as ETosis. To our knowledge, ETosis has not been previously reported in the HPS-PF population and may represent a mechanism by which pulmonary fibrosis develops in these patients. Further research is needed to explore the potential connection between ETosis and HPS-PF, as this understanding could offer insights into the disease mechanism and pave the way for the development of novel treatment modalities.

Keywords: Extracellular traps, Hermansky-Pudlak syndrome, Pulmonary fibrosis, Case report

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