Single-system pulmonary langerhans cell histiocytosis with only tracheobronchial involvement: a case report
Pulmonary Langerhans cell histiocytosis (PLCH) only with airway involvement manifested as diffuse thickening of the tracheobronchial walls is rare. (Source: BMC Pulmonary Medicine)
Source: BMC Pulmonary Medicine - October 28, 2023 Category: Respiratory Medicine Authors: Xin Peng, Hui Liu, Xinyu Zhang, Huaibi Huo and Ting Liu Tags: Case Report Source Type: research

Is it possible to predict multisystem Langerhans cell histiocytosis with lung involvement based on radiological findings detected by computed tomography?
Conclusion:The high intensity of cigarette smoking and low severity of cysts lesions can be a predictor of isolated PLCH. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Radzikowska, E., Jeskiewicz, M., Blasinska, K., Wakulinski, J., Sobiecka, M., Wiatr, E., Załeska, M., Langfort, R., Bestry, I. Tags: Rare ILD/DPLD Source Type: research

Clinical characteristics of diffuse cystic lung diseases at a national referral centre
Conclusions: Our data indicates that despite their rarity, DCLDs share common features, yet differ in presentation and clinical course. With increased risk of malignancy and possible progressive disease, it is increasingly important to screen for these conditions. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: O'Brien, H., Gill, C., O'Callaghan, M., Lynn, E., Hickey, F., Khan, J., Franciosi, A., Keane, M. P., Mc Carthy, C. Tags: Rare ILD/DPLD Source Type: research

Lipid metabolism in interstitial lung diseases: serum amyloid A a new potential biomarker in idiopathic pulmonary fibrosis
Conclusions: SAA could be a potential biomarker of IPF that can predict clinical course, prognosis and survival of IPF patients. Monitoring SAA serum levels could help to identify rapidly progressive phenotype of IPF or at risk of acute exacerbation.References[1] Vietri, L. et al. Respiratory Investigation 2019; 57(5):430-434. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Vietri, L., D'Alessandro, M., Bergantini, L., Cameli, P., Bargagli, E. Tags: Idiopathic interstitial pneumonias Source Type: research

Mutant PIK3CA is a targetable driver alteration in histiocytic neoplasms
Blood Adv. 2023 Oct 23:bloodadvances.2022009349. doi: 10.1182/bloodadvances.2022009349. Online ahead of print.ABSTRACTLangerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm characterized by the accumulation of clonal mononuclear phagocyte system cells expressing CD1a and CD207. In the past decade, molecular profiling of LCH, as well as other histiocytic neoplasms demonstrated that these diseases are driven by MAP kinase (MAPK) activating alterations, with somatic BRAFV600E mutations in >50% of LCH patients, and clinical inhibition of MAPK signaling has demonstrated remarkable clinical efficacy. At the s...
Source: Adv Data - October 24, 2023 Category: Epidemiology Authors: Benjamin H Durham Oshrat Hershkovitz-Rokah Omar I Abdel-Wahab Mariko Yabe Young Rock Chung Gilad Itchaki Maayan Ben-Sasson Vered A Asher-Guz David Groshar Seyram A Doe-Tetteh Tina Alano David B Solit Ofer Shpilberg Eli L Diamond Roei D Mazor Source Type: research

Mutant PIK3CA is a targetable driver alteration in histiocytic neoplasms
Blood Adv. 2023 Oct 23:bloodadvances.2022009349. doi: 10.1182/bloodadvances.2022009349. Online ahead of print.ABSTRACTLangerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm characterized by the accumulation of clonal mononuclear phagocyte system cells expressing CD1a and CD207. In the past decade, molecular profiling of LCH, as well as other histiocytic neoplasms demonstrated that these diseases are driven by MAP kinase (MAPK) activating alterations, with somatic BRAFV600E mutations in >50% of LCH patients, and clinical inhibition of MAPK signaling has demonstrated remarkable clinical efficacy. At the s...
Source: Adv Data - October 24, 2023 Category: Epidemiology Authors: Benjamin H Durham Oshrat Hershkovitz-Rokah Omar I Abdel-Wahab Mariko Yabe Young Rock Chung Gilad Itchaki Maayan Ben-Sasson Vered A Asher-Guz David Groshar Seyram A Doe-Tetteh Tina Alano David B Solit Ofer Shpilberg Eli L Diamond Roei D Mazor Source Type: research

An effective treatment in Erdheim Chester disease: vemurafenib: a case report
ConclusionsBRAF V600E mutation is frequently seen in ECD. Vemurafenib plays an active role in targeted therapy. (Source: Journal of Medical Case Reports)
Source: Journal of Medical Case Reports - October 12, 2023 Category: General Medicine Source Type: research

GSE230040 BRAFV600E-induced transcriptional changes in cord blood-derived hematopoietic stem and progenitor cells
Contributors : Tommaso Sconocchia ; Andreas ReinischSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensBRAFV600E is a recurrent mutation in the histiocytoic disorders Langerhans cell histiocytosis (LCH) and Erdheim Chester Disease (ECD). The mutation can be detected in committed monocytes/macrophages, dendritic cells, and hematopoietic stem and progenitor cells (HSPCs). Although the cell of origin that gives rise to these disorders is currently unknown, it was recently suggested that LCH and ECD arise from BRAFV600E-mutant HSPCs. To understand the underlying molecular mechanisms in HS...
Source: GEO: Gene Expression Omnibus - October 9, 2023 Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research

Best Approach to a Cavitary Lung Lesion – Update
This is an update of our 2015 article on cavitary lung lesion.1 Case A 60-year-old man with alcohol use disorder presented to the hospital with fatigue, chest pain, and productive cough for two weeks. Additionally, he endorsed a 20-lb weight loss over the previous month which he attributed to a poor appetite. He lived in the southwestern U.S. and had no recent travel. His initial chest X-ray demonstrated a 3.4-cm left upper lobe cavitary lesion (Figure 1). Overview Hospitalists frequently encounter patients with cavitary lung lesions on chest imaging and are often faced with initiating their early workup and management. Ha...
Source: The Hospitalist - October 2, 2023 Category: Hospital Management Authors: Ronda Whitaker Tags: Clinical Clinical Guidelines Pulmonology Source Type: research

Pediatrics, Craniofacial and Orthognathic Surgery Track: Head and Neck Langerhans Cell Histiocytosis in Children
Langerhans Cell Histiocytosis (LCH) is a sporadic myeloid neoplasm with proliferation of dendritic cells1. In the United States, LCH has an estimated annual incidence of approximately 1 per million children2. Controversy exists regarding the management of LCH in the head and neck: debridement, chemotherapy or both. There are few reports describing LCH in the OMS literature. The purpose of this study was to present our experience with LCH of the head and neck in children. (Source: Journal of Oral and Maxillofacial Surgery)
Source: Journal of Oral and Maxillofacial Surgery - October 1, 2023 Category: ENT & OMF Authors: Joyce Xu, Julie Gilbert, Kathryn S. Sutton, Steve L. Goudy, Shelly Abramowicz Source Type: research

Cerebrospinal fluid metabolomic pattern of different pituitary stalk lesions
CONCLUSIONS: Our study is the first to systematically investigate the metabolomics of CSF in different pituitary stalk lesions. CSF metabolomics is a useful strategy for biomarker discovery.PMID:37769631 | DOI:10.1210/clinem/dgad559 (Source: The Journal of Clinical Endocrinology and Metabolism)
Source: The Journal of Clinical Endocrinology and Metabolism - September 28, 2023 Category: Endocrinology Authors: Linjie Wang Xiaoxue Chen Xiaoyan Liu Hui Miao Fengying Gong Hongbo Yang Lian Duan Huijuan Zhu Wei Sun Source Type: research

Malignus eml őtumor? Gyulladásos emlőelváltozás? Ritka benignus kórkép?
CONCLUSION: Rosai-Dorfman disease confined to the breast is extremely rare, that clinically may mimic breast cancer.PMID:37747834 | DOI:10.1556/1046.2023.30003 (Source: Magyar Sebeszet)
Source: Magyar Sebeszet - September 25, 2023 Category: Surgery Authors: Edina Zita Kiss Ákos Botos Zolt án Szöllősi Erika T óth Source Type: research

Immune microenvironment associated with the severity of Langerhans cell histiocytosis in children
Cytokine. 2023 Sep 23;171:156378. doi: 10.1016/j.cyto.2023.156378. Online ahead of print.ABSTRACTThe aim of this study is to investigate the clinical potential of immune microenvironment in peripheral blood for the severity and therapeutic efficacy of Langerhans cell histiocytosis (LCH). A total of 200 newly diagnosed children with LCH during 10 years was enrolled for analysis in this study. Peripheral blood samples were acquired from patients before treatment in our hospital and immune indicators were detected by a four-color flow cytometer. The levels of CD3 + CD8 + T cell, CD3 + CD4 + HLA-DR + T cell, CD3 + CD8 + HLA-DR...
Source: Cytokine - September 25, 2023 Category: Molecular Biology Authors: Fengqing Cai Zhaoyang Peng Hui Xu Hui Gao Chan Liao Xiaojun Xu Xiaoping Guo Weizhong Gu Kun Zhu Qiang Shu Hongqiang Shen Source Type: research

Malignus eml őtumor? Gyulladásos emlőelváltozás? Ritka benignus kórkép?
CONCLUSION: Rosai-Dorfman disease confined to the breast is extremely rare, that clinically may mimic breast cancer.PMID:37747834 | DOI:10.1556/1046.2023.30003 (Source: Magyar Sebeszet)
Source: Magyar Sebeszet - September 25, 2023 Category: Surgery Authors: Edina Zita Kiss Ákos Botos Zolt án Szöllősi Erika T óth Source Type: research

Immune microenvironment associated with the severity of Langerhans cell histiocytosis in children
Cytokine. 2023 Sep 23;171:156378. doi: 10.1016/j.cyto.2023.156378. Online ahead of print.ABSTRACTThe aim of this study is to investigate the clinical potential of immune microenvironment in peripheral blood for the severity and therapeutic efficacy of Langerhans cell histiocytosis (LCH). A total of 200 newly diagnosed children with LCH during 10 years was enrolled for analysis in this study. Peripheral blood samples were acquired from patients before treatment in our hospital and immune indicators were detected by a four-color flow cytometer. The levels of CD3 + CD8 + T cell, CD3 + CD4 + HLA-DR + T cell, CD3 + CD8 + HLA-DR...
Source: Cytokine - September 25, 2023 Category: Molecular Biology Authors: Fengqing Cai Zhaoyang Peng Hui Xu Hui Gao Chan Liao Xiaojun Xu Xiaoping Guo Weizhong Gu Kun Zhu Qiang Shu Hongqiang Shen Source Type: research