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Restitutio ad integrum: Rescuing the Alveolar Macrophage Function with HSCT in Pulmonary Alveolar Proteinosis Due to CSF2Rα Deficiency.
J Clin Immunol
December 2024
Department of Blood and Marrow Transplantation, Bai Jerbai Wadia Hospital for Children, Mumbai, India, 400012.
Hereditary pulmonary alveolar proteinosis (hPAP) is a rare lung-related primary immunodeficiency. In hPAP, variants of genes encoding the heterodimeric GM-CSF receptor alpha or beta-chains (CSF2Rα, CSF2Rβ) lead to perturbations in GM-CSF signalling. These perturbations impair the scavenging function of pulmonary alveolar macrophages leading to accumulation of surfactant proteins and lipids within the alveoli.
View Article and Find Full Text PDFRemote Kidney and Liver Injury After Transplantation of Lung Allografts in an Allogeneic Mouse Model.
Transplant Proc
November 2024
Department of Anesthesiology and Intensive Care, University of Leipzig Medical Center, Leipzig, Germany. Electronic address:
Article Synopsis
- Remote organ dysfunction is common after lung transplantation and may negatively impact the patient’s outcome, prompting an investigation into protective measures.
- A study on mice showed that lung transplantation increased specific molecular markers of injury in the kidneys and liver, indicating a response to the transplant.
- Ropivacaine, while previously shown to reduce acute rejection in lung transplants, did not significantly affect inflammation markers in the remote organs during this study.
Grading Histopathology Features of Graft-versus-host Disease in Animal Models; A Systematic Review.
Iran J Allergy Asthma Immunol
April 2024
Department of Pathology and Laboratory Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Article Synopsis
- - Graft-versus-host disease (GvHD) is a serious complication after allogeneic stem cell transplants, causing significant health risks, and accurate diagnosis through histopathological examination is vital for understanding the disease and testing new treatments.
- - This systematic review evaluates different grading systems for GvHD in animal models, focusing on the tissues analyzed and the criteria used for scoring severity, with a strong emphasis on skin, liver, and gut tissues.
- - The review highlights the need for standardized grading criteria to ensure consistency across studies, which could help translate laboratory findings into effective clinical treatments for GvHD.
Pulmonary and Systemic Immune Profiles Following Lung Volume Reduction Surgery and Allogeneic Mesenchymal Stromal Cell Treatment in Emphysema.
Cells
September 2024
Department of Pulmonology, PulmoScience Lab, Leiden University Medical Center (LUMC), 2333 Leiden, The Netherlands.
Emphysema in patients with chronic obstructive pulmonary disease (COPD) is characterized by progressive inflammation. Preclinical studies suggest that lung volume reduction surgery (LVRS) and mesenchymal stromal cell (MSC) treatment dampen inflammation. We investigated the effects of bone marrow-derived MSC (BM-MSC) and LVRS on circulating and pulmonary immune cell profiles in emphysema patients using mass cytometry.
View Article and Find Full Text PDFA phase 2 multicenter trial of ruxolitinib to treat bronchiolitis obliterans syndrome after allogeneic HCT.
Blood Adv
January 2025
Hematopoietic Cell Transplant and Cellular Therapy Program, Massachusetts General Hospital, Boston, MA.
Article Synopsis
- - Bronchiolitis obliterans syndrome (BOS) is a serious lung condition that can develop after receiving a stem cell transplant, and this study tested the effectiveness of ruxolitinib in treating it.
- - The trial involved 49 adult participants divided into two groups based on how recently they were diagnosed with BOS; results showed that 27.8% of newly diagnosed patients improved, while 92.3% of those with established BOS maintained stable lung function after treatment.
- - Overall, ruxolitinib had a lung-specific response rate of 34.7% among all participants with most positive outcomes seen in patients with mild to moderate disease, indicating it could be a beneficial treatment option for BOS
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