Langerhans Cell Histiocytosis

Green, Adam L.; Rodríguez‐Galindo, Carlos

doi:10.1007/978-1-4614-8755-5_26

Cited by 1 publication

(5 citation statements)

References 47 publications

(46 reference statements)

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“…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). Bones were most involved which is consistent with other reports [2,4,6]. The course of LCH can be diverse and often is unpredictable.…”

Section: Discussionsupporting

confidence: 91%

“…The younger the child, the greater the risk of multisystem disease, and the greater the risk of the unfavorable course of LCH. In addition, the involvement of the risk organs (hematopoietic system, liver, spleen) is associated with a worse prognosis [1][2][3][4]6]. In our material, both deceased patients had MS disease.…”

Section: Discussionmentioning

confidence: 62%

“…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%

“…Langerhans cells histiocytosis (LCH) is a rare disease with unclear etiology. It may affect different age groups, but is most often observed in the first decade of life [1][2][3][4]. It can affect different organs and systems i.e.…”

Section: Discussionmentioning

confidence: 99%

“…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%

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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

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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.

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Section: Discussionsupporting

confidence: 91%