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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 46-49 |
| Number of pages | 4 |
| Journal | Journal of Chest Surgery |
| Volume | 58 |
| Issue number | 1 |
| DOIs | |
| State | Published - Jan 5 2025 |
Bibliographical note
Publisher Copyright:Copyright © 2025, The Korean Society for Thoracic and Cardiovascular Surgery 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.
PubMed: MeSH publication types
- Case Reports
- Journal Article