Langerhans Cell Histiocytosis (LCH) Research This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Pediatric pulmonary multisystem langerhans cell histiocytosis: does lung lesion severity affect the outcome?
The pediatric pulmonary multisystem Langerhans cell histiocytosis (PPM LCH) is associated with either low risk or high risk organ(s). The nodulo-cystic lung lesions although pathognomonic, yet are very variab... (Source: Orphanet Journal of Rare Diseases)
Source: Orphanet Journal of Rare Diseases - November 17, 2023 Category: Internal Medicine Authors: Mohamed Sedky, Seham Gohar, Sonia Ahmed, Iman Zaky, Asmaa Salama, Omayma Hassanein, Eslam Maher and Alaa ElHaddad Tags: Research Source Type: research

Neuroimaging manifestations of paediatric histiocytoses
SummaryHistiocytoses are rare multi-system disorders marked by abnormal histiocyte cell proliferation, affecting children with diverse clinical presentations. Classified into five groups in 2016, including Langerhans-related (L), cutaneous (C), malignant (M), Rosai –Dorfman disease (R) and haemophagocytic lymphohistiocytosis (H), newer entities such as ALK-positive histiocytosis have also emerged, heralding the era of molecular (sub)classification. Common entities include Langerhans cell histiocytosis (LCH), Erdheim–Chester disease (ECD), Rosai–Dorfman d isease (RDD) and haemophagocytic lymphohistiocytosis (HLH). Thi...
Source: Journal of Medical Imaging and Radiation Oncology - November 15, 2023 Category: Radiology Authors: Hsern Ern Ivan Tan, Rahul Lakshmanan, Richard Warne, Thomas Walwyn, Derek Roebuck Tags: Medical Imaging —Review Article Source Type: research

Therapy-Related Langerhans Cell Histiocytosis and AML
This case report describes a woman in her 30s who had been treated with chemotherapy and presented with Langerhans cell histiocytosis and acute myeloid leukemia (AML). (Source: JAMA Dermatology)
Source: JAMA Dermatology - November 15, 2023 Category: Dermatology Source Type: research

Successful treatment of Langerhans cell histiocytosis in an infant with vemurafenib: a case report and literature review
J Dermatolog Treat. 2023 Dec;34(1):2279901. doi: 10.1080/09546634.2023.2279901. Epub 2023 Nov 9.ABSTRACTLangerhans cell histiocytosis (LCH) is a histiocytic neoplasm characterized by a mass of CD1a + CD207 + histiocytes, exhibiting a diverse range of clinical manifestations from a self-healing rash or single bone destruction to multi-organ disease with potentially fatal consequences. The identification of MAPK signaling pathway activation, particularly BRAFV600E mutations, has propelled targeted therapy into the forefront of therapeutic research for LCH. Several studies have demonstrated that Vemurafenib, a BRAF inhibitor,...
Source: Journal of Dermatological Treatment - November 9, 2023 Category: Dermatology Authors: Qingqing Li Source Type: research

The clinical spectrum and prognostic factors of Erdheim-Chester disease and mixed Langerhans cell histiocytosis and Erdheim-Chester disease
AbstractErdheim-Chester disease (ECD) is a rare and probably fatal multisystemic non-Langerhans cell histiocytosis (LCH). To comprehensively investigate the clinical features, genomic analysis, treatments, and prognostic factors of ECD, we retrospectively analyzed the clinical data of 75 ECD patients and 10 mixed LCH and ECD patients in our center. The median age at diagnosis was 46 years (range, 5–70). ECD patients were older at diagnosis (p = 0.006) and had more cardiac involvement (p = 0.011) as well as vascular (p = 0.031) involvement compared to mixed LCH and ECD patients. 64.8% of ECD patients and 87....
Source: Annals of Hematology - November 3, 2023 Category: Hematology Source Type: research

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

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