Langerhans cell histiocytosis


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histiocytosis

 [his″te-o-si-to´sis]
a condition marked by the abnormal appearance of histiocytes in the blood.
Langerhans cell histiocytosis a generic term that encompasses a group of disorders characterized by proliferation of Langerhans cells, which are specialized cells found in the epidermis that function as part of the immune system. These disorders are believed to arise from disturbances in regulation of the immune system. Children are more often affected than adults, and the bone marrow, endocrine system, and lungs may be involved (the lungs are affected more commonly in adults than in children). Langerhans cell histiocytosis is divided into unifocal and multifocal variants; there is also an acute, disseminated form (letterer-siwe disease). This group of disorders was formerly called histiocytosis X and was classified in three forms: Letterer-Siwe disease, Hand-Schüller-Christian disease, and eosinophilic granuloma. Called also eosinophilic granuloma, eosinophilic granulomatosis, and Langerhans cell granulomatosis.
Langerhans cell histiocytosis, acute disseminated Letterer-Siwe disease.
Langerhans cell histiocytosis, multifocal Langerhans cell histiocytosis occurring as erosive accumulations of proliferating Langerhans cells. It occurs most commonly in the marrow cavities of bones, but may also affect the skin, gingiva, lungs, and stomach. When the triad of involvement of the bones of the skull, exophthalmos, and diabetes insipidus is present, it is referred to as Hand-Schüller-Christian disease.
Langerhans cell histiocytosis, unifocal Langerhans cell histiocytosis occurring as a single osteolytic lesion, usually in a long or flat bone; it may be asymptomatic or may produce bone pain, tenderness, and swelling and, sometimes, pathologic fracture.
histiocytosis X former name for Langerhans cell histiocytosis.

Lang·er·hans cell histiocytosis

(lahng'ĕr-hahnz), [MIM*604856]
a set of closely related disorders unified by a common proliferating element, the Langerhans cell. Three overlapping clinical syndromes are recognized: a single site disease (eosinophilic granuloma), a multifocal unisystem process (Hand-Schuller-Christian syndrome), and a multifocal, multisystem histiocytosis (Letter-Siwe syndrome.) Formerly this process was known as histiocytosis X.
Synonym(s): histiocytosis X

Langerhans cell histiocytosis

n.
Any of several disorders of varying severity in which immature dendritic cells having the appearance of Langerhans cells proliferate, causing lesions or tissue damage in one or more organ systems. Symptoms include bone lesions, swollen gums, rashes, anemia, dysfunction of the liver, spleen, or lungs, and diabetes insipidus.

Lang·er·hans cell his·ti·o·cy·to·sis

(lahng'er-hahnz sel his'tē-ō-sī-tō'sis)
A set of closely related disorders unified by a common proliferating element, the Langerhans cell. Three overlapping clinical syndromes are recognized: a single site disease (eosinophilic granuloma), a multifocal unisystem process (Hand-Schüller-Christian syndrome), and a multifocal, multisystem histiocytosis (Letterer-Siwe syndrome.) Formerly this process was known as histiocytosis X.

Lang·er·hans cell his·ti·o·cy·to·sis

(lahng'er-hahnz sel his'tē-ō-sī-tō'sis) [MIM*604856]
Set of closely related disorders unified by a common proliferating element, the Langerhans cell. Three overlapping clinical syndromes are recognized: single site disease, multifocal unisystem process, and a multifocal, multisystem histiocytosis.
References in periodicals archive ?
Langerhans cell histiocytosis: bilateral temporal involvement in an adult with diabetes insipidus.
Imamura et al., "Intensified and prolonged therapy comprising cytarabine, vincristine and prednisolone improves outcome in patients with multisystem Langerhans cell histiocytosis: results of the Japan Langerhans Cell Histiocytosis Study Group-02 Protocol Study," International Journal of Hematology, vol.
Daniel, "Isolated langerhans cell histiocytosis bone lesion in pediatric patients: systematic review and treatment algorithm," Otolaryngology --Head and Neck Surgery, vol.
The differential diagnosis includes Langerhans cell histiocytosis, lymphoma, immunoglobulin G4-related interstitial lung disease, and Erdheim-Chester disease.
While our case and other reports demonstrate success with vinblastine, there remains need for a consensus on the treatment of aggressive Langerhans cell histiocytosis in adults.
Brown, "Langerhans cell histiocytosis a clinicopathologic review and molecular pathogenetic update," Archives of Pathology & Laboratory Medicine, vol.
Pulmonary Langerhans cell histiocytosis: Emerging concepts in pathobiology, radiology, and clinical evolution of disease.
Langerhans cell histiocytosis (LCH) is the most common form of histiocytosis, with an annual incidence of 4-5 per million.
Langerhans cell Histiocytosis and diagnostic images
Langerhans cell histiocytosis (formerly called histiocytosis X by Lichtenstein in 1953) refers to a group of disorders that are characterized by proliferation of cytologically benign histiocytes.
Presence of Erdheim-Chester disease and Langerhans cell histiocytosis in the same patient: a report of 2 cases.
Langerhans cell histiocytosis (LCH) is a rare neoplasm defined as the proliferation of bone marrow langerhans cells, which is a kind of dendritic cells.

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