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.

Clinical-radiological-pathological correlation in pulmonary hypertension with unclear and/or multifactorial mechanisms
Ever since the second world symposium on pulmonary hypertension (PH) held in Evian, France, in 1998, PH has been classified into five major clinical groups. Group 5 PH includes a variety of distinct conditions with unclear and/or multifactorial underlying pathologies. Management of these patients is challenging as the number of patients within these groups is often small, not all individuals with certain underlying conditions are affected by PH and patients exhibit distinct symptoms due to different underlying diseases. Studies and clinical trials in these groups are largely lacking and mostly restricted to case series and...
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: Lichtblau, M., Mayer, L., Gopalan, D., Dorfmüller, P., Ulrich, S. Tags: Lung imaging, Pulmonary vascular disease Clinical-radiological-pathological correlation in pulmonary hypertension Source Type: research

Bronchoalveolar cytokine profile differentiates Pulmonary Langerhans cell histiocytosis patients from other smoking-related interstitial lung diseases
CONCLUSIONS: Our results show that patients with PLCH exhibit a distinct BAL immune profile to SR-ILD and IPF. PCA analysis and RF model identify a specific immune profile useful for discriminating PLCH.PMID:38111019 | DOI:10.1186/s12931-023-02622-z (Source: Cell Research)
Source: Cell Research - December 19, 2023 Category: Cytology Authors: Silvia Barril Paloma Acebo Paloma Millan-Billi Alfonso Luque Oriol Sibila Carlos Tar ín Abdellatif Tazi Diego Castillo Sonsoles Hortelano Source Type: research

Bronchoalveolar cytokine profile differentiates Pulmonary Langerhans cell histiocytosis patients from other smoking-related interstitial lung diseases
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease (ILD) associated with smoking, whose definitive diagnosis requires the exclusion of other forms of ILD and a compatible surgic... (Source: Respiratory Research)
Source: Respiratory Research - December 18, 2023 Category: Respiratory Medicine Authors: Silvia Barril, Paloma Acebo, Paloma Millan-Billi, Alfonso Luque, Oriol Sibila, Carlos Tar ín, Abdellatif Tazi, Diego Castillo and Sonsoles Hortelano Tags: Research Source Type: research

Facial Cutaneous Rosai-Dorfman Disease: Dermoscopic Findings with Successful Surgical Treatment
Ann Dermatol. 2023 Nov;35(Suppl 2):S287-S291. doi: 10.5021/ad.22.071.ABSTRACTRosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by an accumulation of activated histiocytes within the affected tissues. It is a heterogeneous disease that includes the classical (nodal) and extra-nodal variants. The cutaneous form of the disease without the characteristic lymphadenopathy is rare and is often misdiagnosed as other dermatologic diseases. Misdiagnosis as lymphoproliferative and infectious diseases such as lymphoma and tuberculosis have been reported in the literature. Herein, we report a case of...
Source: Annals of Dermatology - December 7, 2023 Category: Dermatology Authors: Keunyoung Hur Ji Yeon Hong Kyu Han Kim Jihui Jeon Cheol Lee Yoonjin Kwak Tae Min Kim Je-Ho Mun Source Type: research

Clinical features, genomic profiling, and outcomes of adult patients with unifocal Langerhans cell histiocytosis
Langerhans cell histiocytosis (LCH) is a rare highly heterogeneous histiocytosis, which can be divided into single system and multiple system disease according to site of involvement. There is a paucity of stu... (Source: Orphanet Journal of Rare Diseases)
Source: Orphanet Journal of Rare Diseases - November 30, 2023 Category: Internal Medicine Authors: Min Lang, Hua-cong Cai, He Lin, Long Chang, Jia-wen Dai, Jia Chen, Ming-hui Duan, Dao-bin Zhou, Gaurav Goyal and Xin-xin Cao Tags: Research Source Type: research

Cerebellar peduncle damage in Langerhans cell histiocytosis-associated neurodegenerative disease revealed by diffusion tensor imaging
ConclusionIn LCH-ND, we identified microstructural damage in the SCP and MCP. DTI parameters in these tracts may help monitor LCH-ND; therefore, future studies are required to validate these results in a large cohort. (Source: Neuroradiology)
Source: Neuroradiology - November 20, 2023 Category: Radiology Source Type: research

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