New insights into the pathogenesis of Langerhans cell histiocytosis

Jh, de Graaf; Rm, Egeler

doi:10.1097/00008480-199702000-00011

Cited by 27 publications

(12 citation statements)

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“…Many investigators view the disease as a reactive process rather than as a neoplasm, 9 whereas studies examining the clonality of the lesional cells of this condition have shown it to be a monoclonal proliferation, a finding more consistent with a neoplastic process. 9,10 Genetic instability may lead to histiocytic proliferation and disease progression 11 but due to the lack of studies on this subject, no acceptable hypothesis supporting genetic abnormalities has been proposed. The exact pathogenesis remains unclear; it is likely that the aetiology is multifactorial.…”

Section: Discussionmentioning

confidence: 99%

Oral mucosal involvement in Langerhans’ cell histiocytosis: long‐term follow‐up of a rare case

Kılıç

Mavili

et al. 2011

Australian Dental Journal

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Langerhans' cell histiocytosis (LCH) is a rare disease where different organs and systems may be affected. Oral involvement generally consists of mucosal ulceration associated with lesions of the underlying bone. Many reports exist about the misdiagnosis of this disease. Various symptoms may lead the clinician to an incorrect diagnosis, especially with multiple organ involvement. Oral manifestations are common, and dentists should be aware of this disease and evaluate intraoral findings accordingly. This study presents an LCH case characterized by oral mucosal ulcerations with no involvement of the underlying bone. A definitive diagnosis was made by open biopsy from the oral mucosa.

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Section: Discussionmentioning

confidence: 99%

Oral mucosal involvement in Langerhans’ cell histiocytosis: long‐term follow‐up of a rare case

Kılıç

Mavili

et al. 2011

Australian Dental Journal

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“…LCH is characterized by accumulation of the Langerhans cells together with different types of inflammatory cells, causing infiltration of the affected tissues. The etiology of the disease is unknown, and it has been variously classified as a neoplastic process, a reactive disorder, or, most commonly, an aberrant immune response [4][5][6]. The definitive diagnosis of LCH is based on immunohistochemical expression of CD1a, S-100, or the presence of Birbeck granules seen by electron microscopy [7,8].…”

Section: Discussionmentioning

confidence: 99%

Hungarian Experience With Langerhans Cell Histiocytosis in Childhood

Müller

Garami

Hauser

et al. 2006

Pediatric Hematology and Oncology

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The Langerhans cell histiocytosis (LCH) in children is relatively rare and the long-term analysis of therapy results has not been done yet in Hungary. The aim of this study was to investigate the incidence, clinical features, prognostic risk factors, and treatment results of children's LCH in Hungary in a 20-year period. Children less than 18 years of age with newly diagnosed LCH in Hungary were entered in this study. Clinical data of all children with LCH were reported to the National Childhood Cancer Registry in Hungary from 1981 to 2000. The clinical files were collected and abstracted for information regarding age at diagnosis, gender, disease characteristics, treatment, and outcome of treatment. Median follow-up duration of surviving patients is 10.98 years. Between January 1981 and December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The annual incidence of LCH in children younger than 18 years of age was 2.24/million children. The male-female ratio was 1.36:1; the mean age was 4 years 11 months. Thirty-eight children had localized disease and in 73 cases systemic dissemination was found already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation, and 5 children got only local irradiation. In 2 cases remission was achieved with local steroid administration. Seventy-five patients received chemotherapy. In the 20 years of the study 14 children died, 9 due to the progression of the disease. Sixteen patients had relapse with a mean of 2.16 +/- 1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n = 111) was 88.3 +/- 3.1% at 5 years and 87.3 +/- 3.2% at 10 and 20 years. Childhood LCH is a well-treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood.

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“…In this series, 3 cases had a history of childhood malignancy, and 5 others had a family history of cancer. The pathology of LCH combines features of carcinogenesis and chronic inflammation and debate continues as to whether it represents primarily a neoplastic disease of dendritic cells (Weiss et al, 2001;Laman et al, 2003Egeler et al, 2003de Graaf and Egeler, 1997;Willman et al, 1994), or a reactive process of immune regulation with unbridled inflammation (Fadeel and Henter, 2003).…”

Section: Discussionmentioning

confidence: 99%

Ocular Adnexal Langerhans Cell Histiocytosis Clinical Features and Management

Maccheron

McNab

Elder

et al. 2006

Orbit

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Unifocal, unisystem LCH often presents to the ophthalmologist and can usually be diagnosed on clinical and imaging grounds. Computed tomography and magnetic resonance imaging are complementary imaging studies. Biopsy is essential to confirm diagnosis. Unifocal, unisystem disease can be treated with local excision and curettage. Incomplete excision, recurrent disease or multifocal disease may require systemic chemotherapy.

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New insights into the pathogenesis of Langerhans cell histiocytosis

Cited by 27 publications

References 0 publications

Oral mucosal involvement in Langerhans’ cell histiocytosis: long‐term follow‐up of a rare case

Oral mucosal involvement in Langerhans’ cell histiocytosis: long‐term follow‐up of a rare case

Hungarian Experience With Langerhans Cell Histiocytosis in Childhood

Ocular Adnexal Langerhans Cell Histiocytosis Clinical Features and Management

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