New Insights Into the Molecular Pathogenesis of Langerhans Cell Histiocytosis

Rizzo, Francesca; Cives, Mauro; Simone, Valeria De; Silvestris, Franco

doi:10.1634/theoncologist.2013-0341

Cited by 51 publications

(49 citation statements)

References 131 publications

(160 reference statements)

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“…The BRAF-V600E mutation results in constitutive activation of the serine-threonine kinase and increased cell proliferation [16]. The discovery of the activating mutation in BRAF is consistent with the conclusion that LCH is a neoplastic process with inflammatory manifestations [17]. ERK activation appears to be a universal event in LCH, resulting from dysregulation of upstream signalling proteins.…”

mentioning

confidence: 59%

Genetic studies yield clues to the pathogenesis of Langerhans cell histiocytosis

Zhang

Moss

2016

Eur Respir J

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confidence: 59%

Genetic studies yield clues to the pathogenesis of Langerhans cell histiocytosis

Zhang

Moss

2016

Eur Respir J

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“…As a result of disease heterogeneity, no current universally accepted LCH treatment guidelines are available (Rizzo et al 2014). Treatment options mainly depend on the extent and severity of disease at diagnosis (Abla et al 2010).…”

Section: Discussionmentioning

confidence: 99%

“…Langerhans cells (LCs) is bone marrow derived dendritic cells in the skin and lymph nodes (Coleman et al 2013) and act as antigen presenting cells (Rizzo et al 2014). Langerhans cell histiocytosis (LCH) is a clonal disorder where pathologic Langerhans cells (LCs) have similar characteristics to bone marrow-derived Langerhans cells admixed with an inflammatory background of T-cells, macrophages, and eosinophils (Shea & Boos 2017).…”

Section: Introductionmentioning

confidence: 99%

Importance of Clinical and Morphological Correlations in Diagnosing Langerhans Cell Histiocytosis

Hussin¹

2018

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ABSTRAK Histiositosis sel Langerhans (LCH) adalah gangguan histiositik klonal. Pelbagai manifestasi klinikal, dari lesi tulang terisolasi kepada penyakit multisistem ABSTRACTLangerhans cell histiocytosis (LCH) is a clonal histiocytic disorder. The variable clinical manifestations from isolated bone lesion to multisystem disease can cause difficulties and delay in diagnosis. We report a 2 years and 8 months-old girl who presented with a 2 weeks history of persistent fever and weight loss associated with progressive abdominal distension. Physical examination revealed pallor, bilateral proptosis, seaborrheic dermatitis over the scalp and hepatosplenomegaly. Skull X-ray demonstrated multiple lytic lesions at the base and the skull vault. Bone marrow morphology showed numerous abnormal Langerhans cells (LCs) and foamy macrophages. The trephine immunohistochemistry (IHC) stains for CD1a, S-100 and CD68 were inconclusive. The diagnosis of multisystem Langerhans cell histiocytosis (MS-LCH) in this patient was based on the clinical presentation, radiological and morphological analysis. She subsequently received chemotherapy and currently she is on maintenance therapy with a good clinical response. LCH is a rare disease and although the IHC was inconclusive, the correlation of clinical, radiological and morphological data are essential for the diagnosis.

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“…There remains uncertainty regarding whether LCH is a neoplastic or inflammatory disorder. LCH has been described as a neoplastic process due to the monoclonal proliferation of Langerhans cells (18). However, there is also the possibility that LCH is a reactive inflammatory disorder resulting from a dysregulation of the immune system (18).…”

Section: Discussionmentioning

confidence: 99%

“…LCH has been described as a neoplastic process due to the monoclonal proliferation of Langerhans cells (18). However, there is also the possibility that LCH is a reactive inflammatory disorder resulting from a dysregulation of the immune system (18). Previously, a number of findings with regard to the molecular aspects of the disease suggested the possibility that LCH may be the result of an immune dysregulation (10,(19)(20)(21).…”

Section: Discussionmentioning

confidence: 99%

Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

Sun

et al. 2015

Experimental and Therapeutic Medicine

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Abstract. Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells, with a wide range of clinical presentations that vary from a solitary lesion to more severe multifocal or disseminated lesions. The disease can affect any age group; however, the peak incidence rate is in infants aged between 1 and 3 years-old. Diagnosis of LCH should be based on the synthetical analysis of clinical presentations, in addition to features of imaging and histopathology. Although certain cases regress spontaneously, other patients require systemic chemotherapy together with the administration of steroids. The present study reports the case of an infant with LDH with multisystem involvement, including that of the bone, skin, orbit, spleen and lungs. The patient received chemotherapy and obtained rapid improvement in the involved systems. A total of 2.5 years after completion of the therapy, the patient still remains in follow-up and no evidence of active disease has been noted.

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New Insights Into the Molecular Pathogenesis of Langerhans Cell Histiocytosis

Cited by 51 publications

References 131 publications

Genetic studies yield clues to the pathogenesis of Langerhans cell histiocytosis

Genetic studies yield clues to the pathogenesis of Langerhans cell histiocytosis

Importance of Clinical and Morphological Correlations in Diagnosing Langerhans Cell Histiocytosis

Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

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