Histological Findings of ETosis in Hermansky-Pudlak Syndrome with Pulmonary Fibrosis: A Follow-Up Case Report
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 prese...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Korean Society for Thoracic & Cardiovascular Surgery
2025-01-01
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| Series: | Journal of Chest Surgery |
| Subjects: | |
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| Summary: | 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. |
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| ISSN: | 2765-1606 2765-1614 |