Signaling pathways, microenvironment, and targeted treatments in Langerhans cell histiocytosis

Gao, Xuemin; Li, Jian; Cao, Xinxin

doi:10.1186/s12964-022-00917-0

Cited by 7 publications

(4 citation statements)

References 121 publications

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“…LCH comprises qualities of both neoplasia and immunogenic components, and in ammation also plays a vital role in the pathophysiology of LCH [33][34][35]. LCH lesions are accompanied by a diverse in ammatory in ltrate, enriching dysfunctional T cells [36][37][38].…”

Section: Discussionmentioning

confidence: 99%

Serum soluble CD25 levels were associated with prognosis of children with Langerhans cell histiocytosis

Zhao,

Lian,

et al. 2024

Preprint

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Background Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm with inflammatory characteristics. This study aims to investigate the correlation between sCD25 levels and clinical characteristics and prognosis in pediatric LCH. Methods Serum sCD25 levels were measured in 370 LCH patients under 18 years old using ELISA assays. The patients were divided into two cohorts based on different treatment regiments. We further assessed the predictive value for prognosis impact of sCD25 in a test cohort, which was validated in the independent validation cohort. Results The median serum sCD25 level at diagnosis was 3908 pg/ml (range: 231 − 44 000). sCD25 level was significantly higher in MS RO + LCH patients compared to SS LCH patients (P < 0.001). Patients with increased sCD25 were more likely have involvement of risk organs, skin, lung, lymph node, or pituitary (all P < 0.05). sCD25 level could predict LCH progression and relapse with an area under the ROC curve of 60.6%. The best cutoff value was determined at 2921 pg/ml. High-sCD25 group had a significantly worse progression-free survival than those in the low-sCD25 group (P < 0.05). Conclusion Elevated serum sCD25 levels at initial diagnosis was associated with high-risk clinical features and worse prognosis. sCD25 levels can predict the progression/recurrence of LCH after treatment with first-line chemotherapy.

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Section: Discussionmentioning

confidence: 99%

Serum soluble CD25 levels were associated with prognosis of children with Langerhans cell histiocytosis

Zhao,

Lian,

et al. 2024

Preprint

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show abstract

“…LCH comprises qualities of both neoplasia and immunogenic components, and inflammation also plays a vital role in the pathophysiology of LCH [33][34][35] . LCH lesions are accompanied by a diverse inflammatory infiltrate, enriching dysfunctional T cells [36][37][38] .…”

Section: Discussionmentioning

confidence: 99%

Serum soluble CD25 levels were associated with prognosis of children with Langerhans cell histiocytosis

Zhao,

Lian,

et al. 2024

Preprint

View full text Add to dashboard Cite

Background: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm with inflammatory characteristics. This study aims to investigate the correlation between sCD25 levels and clinical characteristics and prognosis in pediatric LCH. Procedure: Serum sCD25 levels were measured in 370 LCH patients under 18 years old using ELISA assays. The patients were divided into two cohorts based on different treatment regiments. We further assessed the predictive value for prognosis impact of sCD25 in a test cohort, which was validated in the independent validation cohort. Results: The median serum sCD25 level at diagnosis was 3908 pg/ml (range: 231-44 000). sCD25 level was significantly higher in MS RO+ LCH patients compared to SS LCH patients ( P<0.001). Patients with increased sCD25 were more likely have involvement of risk organs, skin, lung, lymph node, or pituitary (all P < 0.05). sCD25 level could predict LCH progression and relapse with an area under the ROC curve of 60.6%. The best cutoff value was determined at 2921 pg/ml. High-sCD25 group had a significantly worse progression-free survival than those in the low-sCD25 group ( P < 0.05). Conclusion: Elevated serum sCD25 levels at initial diagnosis was associated with high-risk clinical features and worse prognosis. sCD25 levels can predict the progression/recurrence of LCH after treatment with first-line chemotherapy.

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“…It has been suggested that BRAF V600E mutation leads disruption of endogenous host immune surveillance, immune escape of tumors, and anti-apoptotic activity (7,10).…”

Section: Discussionmentioning

confidence: 99%

“…LCH can be seen in one or more than one organs and can be unifocal or multifocal (2,5,6). Skull involvement is also considered as a risky localization since it comprises risk for diabetes insipidus and degenerative disease (7,8). LCH manifests as an inflammatory lesion in any tissue.…”

Section: Introductionmentioning

confidence: 99%

The Relationship of Prognostic Factors with Regulatory T Cells in Langerhans Cell Histiocytosis

ÇOBAN,

COŞANAY TEKDEN,

GÜLER

et al. 2023

Journal of Anatolian Medical Research

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Aim: Langerhans cell histiocytosis (LCH), is a clonal disorder characterized by abnormal proliferation of dendritic cells, which has an intense inflammatory microenvironment. There is limited information about contribution of microenvironment to this rare disease. We aimed to compare regulatory T cells in microenvironment and BRAFV600E mutation with prognostic data. Material and Methods: Overall, 26 cases were included to the study. The number FOXP3+ regulatory T cell (Treg) and presence of BRAFV600E mutation were assessed according to age, gender, localization, unifocal or multifocal, involvement of organ at risk, and recurrence status in a histochemical manner. Results: The number of adult cases was higher than pediatric cases. Bone was the most common localization, and 81% of cases were unifocal. Risk organ involvement was observed in 3 cases, 2 of which showed recurrence. It was found that the number of FOXP3+ Tregs was higher in adults, those with unifocal localization, and those with bone involvement. In addition, the number of FOXP3+ Tregs was higher in the group with recurrence than those without recurrence. BRAFV600E mutation was higher in children when compared to adults (p=0.003), but, no significant correlation was found when compared with remaining prognostic parameters (p>0.05). Conclusion: Although BRAFV600E mutation is more common in pediatric patients, it can also be seen in adult patients. The number of FOXP3+ Tregs is proportional to CD3+ and CD4+ cells. T cells, which present at varying rates in microenvironment, play an essential role in the pathogenesis of LCH.

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Signaling pathways, microenvironment, and targeted treatments in Langerhans cell histiocytosis

Cited by 7 publications

References 121 publications

Serum soluble CD25 levels were associated with prognosis of children with Langerhans cell histiocytosis

Serum soluble CD25 levels were associated with prognosis of children with Langerhans cell histiocytosis

Serum soluble CD25 levels were associated with prognosis of children with Langerhans cell histiocytosis

The Relationship of Prognostic Factors with Regulatory T Cells in Langerhans Cell Histiocytosis

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