Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X may be isolated or generalized in its clinical involvement. Spontaneous resolution may occur, although progression to diffuse systemic disease has also been found. The disease is believed to originate from the mononuclear phagocytic series of cells, although the reason for this activation remains unknown. A possible local reaction to an inciting agent in the nasopharynx or oral cavity has been suggested by the frequent head and neck manifestations in this disease. The prognosis and treatment of patients should be predicated on the staging or extent of disease. Local disease should be treated in a conservative manner. Low dose radiation therapy should be used for inaccessible lesions or lesions in which removal may produce serious morbidity. In the future, randomized treatment protocols need to be undertaken to clarify the status of chemotherapy and immunotherapy in patients with disseminated or aggressive disease.
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PMID:Histiocytosis X. 675 26

Langerhans cell histiocytosis is a rare disorder, with a few reports describing isolated thyroid gland involvement. We report seven cases, which included four females and three males ranging in age from 2 months to 55 years, with a median age of 37 years. Histologically, the cases demonstrated either diffuse or focal involvement of the thyroid gland by Langerhans cell histiocytes, characterized by bean-shaped, lobated, folded nuclei. In association with the histiocytic infiltrate, there was a prominent eosinophilic cellular component, as well as destruction of the thyroid follicles. All cases occurred in a background of lymphocytic thyroiditis. One case demonstrated adenomatoid nodules, whereas another had a microscopic papillary carcinoma. Immunohistochemical staining demonstrated positive reactivity with S-100 protein, lysozyme, and KP-1. Four patients with isolated thyroid disease, treated by surgical resection alone, are alive without systemic disease from 3 to 19 years after initial presentation. The three patients with systemic disease died within 1 year of the initial diagnosis with disease-related complications. Localized disease portends a favorable prognosis as compared to the thyroid involvement as part of systemic disease.
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PMID:Langerhans cell histiocytosis of the thyroid: a series of seven cases and a review of the literature. 865 21

Langerhans cell histiocytosis in the adult is rare, but it is important to recognize its occurrence, as it must be differentiated from lymphoma, myeloma, and a variety of skin conditions and endocrinopathies. It has been reported in patients up to the ninth decade of life, and occurs equally in men and women. Local disease has a good prognosis, but associated diseases--particularly malignancy--may be the cause of death in some adults. The optimal treatment is not known. Coordinated investigation of the epidemiology and therapy of this disease is needed.
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PMID:Langerhans cell histiocytosis in adults. 956 99

We describe an 18-year-old woman with several month's history of a 12 x 7 mm palpable mammary nodule, that was hypoechoic, with regular margins and vascularization areas by ultrasound. A fibroadenoma was hypothesized (American College of Radiology BI-RADS 3). A 14 G needle biopsy was performed, showing a LC proliferation suspected for LCH of a lymph node, with florid dermatopathic lymphadenopathy in differential diagnosis. The multidisciplinary team of the breast clinic decided to perform a lumpectomy and a diagnosis of LCH involving an intra-mammary lymph node was made. Langerhans cells (LC) are dendritic cells characterized by grooved nuclei, irregular nuclear contours, and abundant cytoplasm, that normally reside in the skin and mucosal surfaces. They were positive for CD1a, langerin/CD207, and S100 by immunohistochemistry. Langerhans cell histiocytosis (LCH) is a clonal proliferation of histiocytes that is thought to be neoplastic in most cases. Reactive LC can be distinguished from LCH by cyclin D1 immunostaining, which is positive only in LCH. About 50% of cases have BRAF V600E mutations. The revised classification of histiocytes divides LCH in subtypes: LCH SS (single system), LCH lung positive, LCH Multiple System/Risk Organ negative and LCH Multiple System/Risk Organ positive. Localized disease can progress to multisystem involvement. The diagnosis of LCH is based on clinical and radiological findings in combination with histopathological, immunophenotypic or ultrastructural analyses identifying tissue infiltration by LC. It is recommended that biopsy confirmation of suspected LCH be performed in all cases. Lymph nodes may be the only site of disease or a part of multisystem involvement by LCH. The histologic differential diagnosis is discussed.
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PMID:Langerhans cell histiocytosis of an intra-mammary lymph node in an 18-year-old woman. 3220 40