Biology and Genomics of LCH and Related Disorders

Rollins, Barrett J.

doi:10.1007/978-3-319-59632-7_2

Cited by 3 publications

(3 citation statements)

References 108 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The spectrum of clinical manifestations is broad, from single-focus to lifethreatening symptoms. Current classification divides LCH into two groups: single system (single organ or system is involved; found in about 55% of patients), and multisystem (involves 2 or more systems / organs; with or without risk organs involvement) [1][2][3][4][5][6]. In our study, the single system disease was found in 69% of patients, which may be associated with multisystem underestimation, since the total number of patients was not known (no data from 4 centers).…”

Section: Discussionmentioning

confidence: 65%

“…Its symptoms are the result of accumulation of the abnormal cells in tissues and organs. The disease is not associated with increased risk of treatment failure (especially among older children) However, appropriate procedures implemented preemptively can eliminate possible complications and so significantly reduce the risk of deterioration of patients' quality of life [1][2][3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Clinical characteristics and outcome of pediatric patients diagnosed with Langerhans cell histiocytosis in pediatric hematology and oncology centers in Poland

Raciborska¹,

Bilska²,

Węcławek-Tompol

et al. 2020

BMC Cancer

View full text Add to dashboard Cite

Background Langerhans cell histiocytosis (LCH) affects 1–2 in 1,000,000 people. The disease is not associated with increased risk of treatment failure (especially among older children), but appropriate procedures implemented in advance can eliminate complications which might appear and significantly worsen the patients’ quality of life. Thus, we sought to evaluate the clinical features, management, and outcome of children with LCH treated in Polish pediatric hematology-oncology centers. Materials and methods One hundred eighty two patients with LCH were treated according to the Histiocytic Society Guidelines between 2010 and 2017. The participating centers were requested to provide the following data: demographic, clinical, as well as local or systemic treatment data and patients’ outcome. Overall survival (OS) and event free survival (EFS) were estimated by Kaplan-Meier methods and compared using the log-rank test. Results Sixty nine percent of children were classified as single system (SS). The patients with SS disease were significantly older as compared to the children with multisystem disease (MS), 6 vs. 2.3 years respectively (p 0.003). Bones were involved in 76% of patients. Systemic treatment was applied to 47% of children with SS disease and 98% with MS disease. Fourteen patients relapsed while two children died. OS and EFS in entire group were 0.99 and 0.91 respectively (with median follow-up 4.3 years). Conclusion The treatment of LCH in Polish centers was effective, however, new approaches, including mutation analyses and good inter-center cooperation, are needed to identify patients who might require modification or intensification of treatment.

show abstract

Section: Discussionmentioning

confidence: 65%

Section: Introductionmentioning

confidence: 99%

Clinical characteristics and outcome of pediatric patients diagnosed with Langerhans cell histiocytosis in pediatric hematology and oncology centers in Poland

Raciborska¹,

Bilska²,

Węcławek-Tompol

et al. 2020

BMC Cancer

View full text Add to dashboard Cite

show abstract

“…Data may be preliminary. from the N-terminal negative regulatory domain of the protein which presumably derepresses the kinase domain, leading to subsequent MAPK pathway activation 12.…”

mentioning

confidence: 99%

Langerhans Cell Histiocytosis with BRAF p.N486_P490del or MAP2K1 p.K57_G61del Treated by the MEK Inhibitor Trametinib

Messinger

Bostrom

Olson

et al. 2020

Preprint

View full text Add to dashboard Cite

Langerhans Cell Histiocytosis (LCH) is characterized by activating variants of the MAPK pathway. Inhibition of the MAPK pathway with trametinib (MEK inhibitor) has been shown to induce responses in LCH patients. Two adolescent males with LCH driven by BRAF p.N486 P490del have received trametinib for >1 year with no reactivation in one and partial response in another (including stable lung disease). A third male with neonatal LCH and MAP2K1 p.K57 G61del had a complete response to trametinib with no active disease after 22 months. All patients continue on trametinib monotherapy with tolerable skin and CPK toxicity.

show abstract

Histiocytic Lesions

Chikwava

2023

Atlas of Pediatric Hematopathology

View full text Add to dashboard Cite

Biology and Genomics of LCH and Related Disorders

Cited by 3 publications

References 108 publications

Clinical characteristics and outcome of pediatric patients diagnosed with Langerhans cell histiocytosis in pediatric hematology and oncology centers in Poland

Clinical characteristics and outcome of pediatric patients diagnosed with Langerhans cell histiocytosis in pediatric hematology and oncology centers in Poland

Langerhans Cell Histiocytosis with BRAF p.N486_P490del or MAP2K1 p.K57_G61del Treated by the MEK Inhibitor Trametinib

Histiocytic Lesions

Contact Info

Product

Resources

About