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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Four new cases are reported, and there is a review of the 38 cases in the literature. This disease may involve the vulva, vagina, cervix, endometrium, and ovary. Four distinct patient groups, segregated on the basis of initial presentation and subsequent anatomic extent of disease, were categorized as follows: (1) "pure" genital LCH, (2) genital LCH with subsequent multi-organ involvement, (3) oral or cutaneous LCH with subsequent genital and multi-organ involvement, and (4) diabetes insipidus with subsequent genital and multi-organ disease. Although involvement of the genital tract can occur at any age, it is most common in young adulthood. Clinically, LCH may mimic either primary neoplasia or various inflammatory lesions; the major pathologic differential diagnosis is venereal and other inflammatory diseases. The pure genital form may have a distinct nosologic position in the spectrum of LCH similar to the "pure," self-limited cutaneous histiocytosis seen in infants. There is no correlation between histologic findings and the outcome of the genital lesions. There is also no correlation between clinical presentation and/or the extent of involvement and outcome of genital lesions; complete regression, partial improvement, persistent lesions, and recurrences were seen in all four groups of patients. The treatment of genital LCH is not well defined and is highly individualized. Therapy has included surgery, radiation, topical corticosteroids, topical nitrogen mustard, systemic chemotherapy, and combination therapy; mixed results were obtained with all treatment modalities. Although no modality has been shown to yield a superior outcome, complete surgical excision is advocated as initial therapy.
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PMID:Langerhans cell histiocytosis of the female genital tract. 200 54

In a patient aged 29 a recurrent histiocytic granuloma developed in vulva, vagina and cervix. The patient was treated by cervical resection followed by irradiation. Langerhans cells with Birbeck granules in plasma of the cells, besides multiple eosinophilic leucocytes were evidenced by electron microscope. The eosinophilic histiocytic granuloma is suggested to be a benign variety of histiocytosis X, whose basic cellular component is the Langerhans cell. Eosinophil leucocytes represent a local immune response in the benign variety of histiocytosis X.
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PMID:[Eosinophilic histiocytic granuloma of the vulva and cervix]. 278 3

Langerhans cells represent a minor epidermal cell population in mammals. They are also observed in squamous epithelia of the oesophagus, vagina and cervix, as well as in oral epithelia. They occur in higher density in the non keratinized epithelium. These cells are characterized by a dendritic pattern, a clear cytoplasm and ultrastructurally by the presence of Birbeck granules. They are usually located in a suprabasal position. Their bone marrow origin is now well established. Surface markers and functional properties identify them as belonging to the macrophage/monocyte lineage. Langerhans cells can be identified in tissue sections by immunofluorescence or immunoperoxidase techniques using monoclonal antibodies directed against surface antigens such as class II histocompatibility antigens, T6 marker, or possibly T4 marker. There is also a cytoplasmic marker, the S-100 protein. A renewed interest in Langerhans cells comes from evidence of their role in the cutaneous immune response. At present these cells are considered as dendritic cells expressing a high density of class II histocompatibility antigens and behave as very potent antigen presenting cells that activate mainly helper T lymphocytes. However, experimental data on antigen processing and interleukin 1 secretion are still lacking. This review also examines the oral pathology literature with respect to modifications in the number or localization of Langerhans cells and their proximity to T lymphocytes, for example in lichen planus, Behcet's syndrome, erythema multiforme, gingivitis and oral carcinoma. Histiocytosis X represents a particular case in which the Langerhans cell itself is affected.
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PMID:[Physiology of Langerhans cells and their potential role in oral pathology]. 351 86

An unusual case of a patient diagnosed as having the histiocytic proliferative disorder characterized by the lesion eosinophilic granuloma (histiocytosis X) is presented. Fifteen-year follow-up revealed a progression from a solitary eosinophilic granuloma of the bone to multifocal involvement of many body structures including the vulva, vagina, and cervix. This progression occurred after nine asymptomatic years and was temporally related to the patient's first pregnancy. After successful treatment and remission, rapid appearance and growth of observable genital lesions were noted during a second pregnancy. Pregnancy termination in the first trimester was advised due to signs of dissemination and local symptoms.
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PMID:Adverse effects of pregnancy on multifocal eosinophilic granuloma. 394 63