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Current Opinion in Hematology Jan 1998The term histiocytosis identifies a group of disorders characterized by localized or generalized reactive or neoplastic proliferation of cells similar, if not identical,... (Review)
Review
The term histiocytosis identifies a group of disorders characterized by localized or generalized reactive or neoplastic proliferation of cells similar, if not identical, to cells of the mononuclear phagocyte and dendritic cell systems. In Langerhans cell histiocytosis (LCH), the proliferating cell is the Langerhans cell, and the morphologic and immunohistochemical criteria for the definitive diagnosis of LCH have been established. The clinically evident pathology of LCH is broadly divided into two categories: direct involvement with the disease (e.g., lytic lesions of the bone or organ involvement) and secondary consequences resulting from permanent damage by the primary disease, LCH (eg, diabetes insipidus, fractures, and tooth loss). Current knowledge suggests tailoring the therapy to the extent of disease. Single-system disease can be treated by excisional biopsy, low-dose radiotherapy, or mild chemotherapy. Multisystem disease should be treated with combination chemotherapy, and current experimental therapeutic approaches include randomized treatment protocols for multisystem disease. A pressing current controversy regarding LCH is that its etiology is unknown. Whether LCH is reactive or neoplastic is even debated, and several features provide seemingly contradictory evidence on this point (spontaneous resolution of disease on one hand and clonality of lesional LCH cells on the other), underscoring the need for further studies to elucidate the etiology and pathogenesis of LCH.
Topics: Antigen-Presenting Cells; Histiocytosis, Langerhans-Cell; Humans
PubMed: 9515204
DOI: 10.1097/00062752-199801000-00010 -
Pediatric Blood & Cancer Feb 2013These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro...
These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was available in the literature up to 2012. Data and publications have been ranked according to evidence based medicine and when there was a lack of published data, consensus between experts was sought. Guidelines for diagnosis, initial clinical work-up, and treatment and long-term follow-up of LCH patients are presented.
Topics: Adolescent; Child; Child, Preschool; Histiocytosis, Langerhans-Cell; Humans
PubMed: 23109216
DOI: 10.1002/pbc.24367 -
Advances in Anatomic Pathology Nov 1998Langerhans cell histiocytosis (LCH) is a rare clonal disorder that consists of single or multiple mass lesions composed of cells with an abnormal Langerhans cell... (Review)
Review
Langerhans cell histiocytosis (LCH) is a rare clonal disorder that consists of single or multiple mass lesions composed of cells with an abnormal Langerhans cell phenotype. Its etiology remains unknown, despite extensive searches for evidence of consistent cytogenetic abnormalities, gene rearrangements, or viral genomes. Similarly, the pathogenesis of the disease is enigmatic, although the altered expression of cytokines and cellular adhesion molecules, important for migration and homing of the activated normal Langerhans cell, may play an important role. The biologic behavior of LCH ranges from spontaneous remission to lethal dissemination, and such behavior cannot be predicted on the basis of histologic features. The presence and degree of organ dysfunction, together with the patient's age at diagnosis, remain the most reliable indicators of prognosis. Treatment of severe, refractory disease continues to be controversial and, in many cases, ineffectual. The revised classification scheme for LCH and related disorders recognizes the uncertain biological potential of LCH and its relation to other processes of dendritic and macrophage origin.
Topics: Diagnosis, Differential; Histiocytosis, Langerhans-Cell; Humans; Langerhans Cells; Phenotype; Prevalence
PubMed: 10095876
DOI: 10.1097/00125480-199811000-00001 -
Asian Journal of Surgery Jul 2022
Topics: Chest Pain; Histiocytosis, Langerhans-Cell; Humans; Sternum
PubMed: 35277320
DOI: 10.1016/j.asjsur.2022.02.038 -
Journal of Feline Medicine and Surgery Apr 2020The aim of this study was to report the clinical, radiographic and pathological features of pulmonary Langerhans cell histiocytosis in four cats, and carry out a... (Review)
Review
OBJECTIVES
The aim of this study was to report the clinical, radiographic and pathological features of pulmonary Langerhans cell histiocytosis in four cats, and carry out a literature review of feline histiocytic diseases.
METHODS
Necropsy reports archived at the Department of Veterinary Pathology of the Federal University of Rio Grande do Sul were reviewed. The clinical information was then obtained from the clinical records at the Veterinary Hospital. Routine samples had been collected during necropsy, fixed in 10% formalin, routinely processed for histology, and stained with hematoxylin and eosin. Samples of lung were submitted for bacterial and fungal culture. Tissue sections of lung underwent immunohistochemical testing for vimentin, pancytokeratin, CD18, CD3, CD79αcy, E-cadherin and Iba1.
RESULTS
This disease affected mixed breed cats aged 7-14 years. Clinical signs consisted of severe mixed inspiratory and expiratory restrictive dyspnea, lethargy and anorexia. Thoracic radiographs revealed different lesion profiles, predominantly of an interstitial and alveolar pattern. Grossly, the lungs were diffusely firm and did not collapse. The pleural surface was bright and irregular due to multifocal-to-coalescent, well-demarcated, white, firm nodules that also extended into and obliterated the pulmonary parenchyma. Histological changes were characterized by poorly demarcated infiltration with histiocytic cells arranged in cohesive groups within the alveolar, bronchiolar and bronchial spaces. Histiocytic cells had intense cytoplasmic immunolabeling for vimentin and Iba1, and robust membrane immunolabeling with CD18 and E-cadherin; these cells were negative for CD3, CD79αcy and pancytokeratin in all cases.
CONCLUSIONS AND RELEVANCE
This article confirms that pulmonary Langerhans cell histiocytosis is a rare disease that occurs in middle-aged to older cats and causes widespread involvement of the pulmonary parenchyma, inducing acute or chronic, progressive respiratory disease characterized by mixed restrictive dyspnea that eventually leads to death. While a definitive clinical diagnosis is challenging, the nodular appearance of the pulmonary changes, together with the histological and immunohistochemistry findings, suffice for diagnostic confirmation of pulmonary Langerhans cell histiocytosis.
Topics: Animals; Cat Diseases; Cats; Histiocytosis, Langerhans-Cell
PubMed: 30977699
DOI: 10.1177/1098612X19842384 -
Journal of Cutaneous Medicine and... 2012The cutaneous presentation of Langerhans cell histiocytosis (LCH) is very rare and can be highly variable among individuals, which can often lead to a delay in diagnosis. (Review)
Review
BACKGROUND
The cutaneous presentation of Langerhans cell histiocytosis (LCH) is very rare and can be highly variable among individuals, which can often lead to a delay in diagnosis.
OBJECTIVE
To discuss a case report and literature review of important clinical indicators, histology, diagnosis, evaluation, and treatment guidelines.
METHODS
Case report and literature review.
RESULTS
Skin biopsies positive for CD1a and/or langerin are diagnostic for LCH. A thorough review of systems, baseline laboratory tests, and imaging studies can determine the extent of LCH. Treatment of cutaneous disease is largely based on case report and small case studies, but baseline treatment should generally begin with oral or topical steroids. When patients have more severe disease that requires a systemic approach, the efficacy of therapy should be assessed 6 weeks into therapy, with subsequent treatment intensification in patients with limited response.
CONCLUSION
Owing to the rarity of this condition, there are no specific guidelines for treatment of LCH, but guidelines put forth by the Histiocyte Society assist in categorization and basic treatment approaches for patients with systemic disease.
Topics: Aged; Female; Guidelines as Topic; Histiocytosis, Langerhans-Cell; Humans
PubMed: 22417995
DOI: 10.1177/120347541201600109 -
Presse Medicale (Paris, France : 1983) Jan 2017Langerhans cell histiocytosis (LCH) is a rare disease affecting both genders and can occur at any age. It often evolves through successive flares, and its severity... (Review)
Review
Langerhans cell histiocytosis (LCH) is a rare disease affecting both genders and can occur at any age. It often evolves through successive flares, and its severity varies from benign forms that don't require treatment to life threatening disease. Some patients have important functional impairment with psychological and social consequences and prolonged disability. LCH may affect only one organ, with uni- or multifocal involvement or be multisystem disease involving multiple organs. The organs most frequently involved are bones, lung, skin and the endocrinal system. Pulmonary LCH is strongly related to smoking. Some patients have mixed histocytosis combining LCH and other histiocytic disorders. The diagnosis relies on the histological study of tissues samples, and shows tissue infiltration with large cell with pale cytoplasm and reniform nucleus, staining for CD1a and Langerin (CD207) on immunohistochemistry. The BRAF mutation is observed in tissue samples in approximately half of patients and the activation of the RAS-RAF-MEK-ERK pathway has been shown to be constantly activated in LCH lesions, regardless the BRAF status. These findings represent an important forward step in the understanding of the physiopathology of the disease. Treatment must be adapted to the severity of the disease and goes from conservative observation to systemic chemotherapy. Therapies targeting the RAS-RAF-MEK-ERK pathway are promising treatments for progressive disease.
Topics: Adult; Age of Onset; Disease Progression; Female; Histiocytosis, Langerhans-Cell; Humans; Male
PubMed: 27816338
DOI: 10.1016/j.lpm.2016.09.015 -
Revue de Stomatologie Et de Chirurgie... Nov 2009Langerhans cell histiocytosis (ex histiocytosis X) is usually present in children. It is a clonal proliferation of non-functional Langerhans's cells. Histological... (Review)
Review
Langerhans cell histiocytosis (ex histiocytosis X) is usually present in children. It is a clonal proliferation of non-functional Langerhans's cells. Histological aspects are variable. The diagnosis is made in immunolabeling by anti-CD1a. Clinical presentations are variable, depending on their extension. Three syndromes are actually the same pathogenic process: eosinophilic granuloma (single or multiple osseous localizations), Hand-Schüller-Christian disease (chronic form with bone and visceral dissemination) and Abt-Letterer-Siwe disease (disseminated and acute malignant presentation).
Topics: Histiocytosis, Langerhans-Cell; Humans
PubMed: 19828161
DOI: 10.1016/j.stomax.2009.07.008 -
Presse Medicale (Paris, France : 1983) Jan 2017Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can... (Review)
Review
DEFINITION
Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Natural history: the natural history of the disease is very heterogeneous, ranging from auto-regressive lesions to a disease affecting multiple organs with fatal consequences, while some lesions may be responsible for permanent sequels. A multidisciplinary approach: the perception of disease from physicians varies greatly depending on their speciality and experience, as well as the presentation of the disease or the short-term treatment outcomes. But whatever the initial view of the treating physician, a multidisciplinary approach to the LCH is recommended as well as the coordination of the necessary care of this systemic disease and its associated morbidity.
THERAPY
current treatment protocols, adapted to the situation of each patient, provide a survival of 98% in children. The sequels, such as diabetes insipidus, hormonal deficits, deafness and even more rarely respiratory failure and sclerosing cholangitis are seen in up to 30% of children.
Topics: Age of Onset; Child; Child, Preschool; Histiocytosis, Langerhans-Cell; Humans
PubMed: 28087208
DOI: 10.1016/j.lpm.2016.09.013 -
Dermatology (Basel, Switzerland) 1994