Langerhans' cell histiocytosis (histiocytosis-X) in a women with typical ultrastructure of Birbeck's granule: a case report. 1996

W S Wang, and J H Liu, and C F Yang, and A H Yang, and P M Chen
Department of Medicine, Veterans General Hospital-Taipei and National Yang-Ming University School of Medicine, Taiwan, R.O.C.

A 28-year-old woman was admitted to our hospital with the chief complaint of progressive gingival swelling with easy bleeding and loosening of teeth for about two years. The liver and spleen were not enlarged and there were no lymphadenopathy. The hemogram was normal. The skull X-ray showed floating teeth. CT scan of face showed destructive bony lesions over maxilla and left mandible with adjacent soft tissue swelling. Pathologic examination of the gingiva revealed that the oral mucosal tissue was heavily infiltrated with histiocyte-like Langerhans' cells. The Langerhans' cells showed positive immunostain for S-100 protein. Under electron microscope, ultrastructure of the Langerhans' cells revealed typical intracytoplasmic tennnis racquet-shaped structure with a central zipper-like striation (Birbeck's granules). Langerhans' cell histiocytosis was diagnosed. She received rdiotherapy with a total dose of 1,000 cGy in fractions and oral chemotherapeutic drugs. Painful gingival swelling subsided gradually. She was followed at our OPD for the past 10 months and there was no evidence of local recurrence. Langerhans' cell histiocytosis is a rare disease. The relevant literature about its distinct pathologic features, clinical course and treatment is reviewed.

UI MeSH Term Description Entries
D007801 Langerhans Cells Recirculating, dendritic, antigen-presenting cells containing characteristic racket-shaped granules (Birbeck granules). They are found principally in the stratum spinosum of the EPIDERMIS and are rich in Class II MAJOR HISTOCOMPATIBILITY COMPLEX molecules. Langerhans cells were the first dendritic cell to be described and have been a model of study for other dendritic cells (DCs), especially other migrating DCs such as dermal DCs and INTERSTITIAL DENDRITIC CELLS. Langerhans Cell,Dendritic Cells, Dermal,Dendritic Cells, Epidermal,Dendritic Cells, Skin,Dermal Dendritic Cells,Epidermal Dendritic Cells,Skin Dendritic Cells,Cell, Dermal Dendritic,Cell, Epidermal Dendritic,Cell, Langerhans,Cell, Skin Dendritic,Cells, Dermal Dendritic,Cells, Epidermal Dendritic,Cells, Langerhans,Cells, Skin Dendritic,Dendritic Cell, Dermal,Dendritic Cell, Epidermal,Dendritic Cell, Skin,Dermal Dendritic Cell,Epidermal Dendritic Cell,Skin Dendritic Cell
D003594 Cytoplasmic Granules Condensed areas of cellular material that may be bounded by a membrane. Cytoplasmic Granule,Granule, Cytoplasmic,Granules, Cytoplasmic
D005260 Female Females
D006646 Histiocytosis, Langerhans-Cell A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder. Histiocytosis X,Langerhans-Cell Granulomatosis,Langerhans-Cell Histiocytosis,Hand-Schueller-Christian Disease,Hand-Schueller-Christian Syndrome,Hand-Schüller-Christian Disease,Hand-Schüller-Christian Syndrome,Hashimoto-Pritzger Disease,Histiocytosis, Generalized,Histiocytosis-X,Langerhans Cell Granulomatosis,Langerhans Cell Granulomatosis, Pulmonary,Langerhans Cell Histiocytosis,Letterer-Siwe Disease,Non-Lipid Reticuloendotheliosis,Pulmonary Histiocytosis X,Pulmonary Langerhans Cell Granulomatosis,Schueller-Christian Disease,Systemic Aleukemic Reticuloendotheliosis,Type 2 Histiocytosis,Aleukemic Reticuloendothelioses, Systemic,Aleukemic Reticuloendotheliosis, Systemic,Cell Granulomatoses, Langerhans,Cell Granulomatosis, Langerhans,Cell Histiocytoses, Langerhans,Cell Histiocytosis, Langerhans,Disease, Hand-Schueller-Christian,Disease, Hand-Schüller-Christian,Disease, Hashimoto-Pritzger,Disease, Letterer-Siwe,Disease, Schueller-Christian,Diseases, Hashimoto-Pritzger,Generalized Histiocytoses,Generalized Histiocytosis,Granulomatoses, Langerhans Cell,Granulomatosis, Langerhans Cell,Granulomatosis, Langerhans-Cell,Hand Schueller Christian Disease,Hand Schueller Christian Syndrome,Hand Schüller Christian Disease,Hand Schüller Christian Syndrome,Hashimoto Pritzger Disease,Hashimoto-Pritzger Diseases,Histiocytoses, Generalized,Histiocytoses, Langerhans Cell,Histiocytoses, Type 2,Histiocytosis X, Pulmonary,Histiocytosis, Langerhans Cell,Histiocytosis, Type 2,Langerhans Cell Granulomatoses,Langerhans Cell Histiocytoses,Letterer Siwe Disease,Non Lipid Reticuloendotheliosis,Non-Lipid Reticuloendothelioses,Reticuloendothelioses, Non-Lipid,Reticuloendothelioses, Systemic Aleukemic,Reticuloendotheliosis, Non-Lipid,Reticuloendotheliosis, Systemic Aleukemic,Schueller Christian Disease,Syndrome, Hand-Schueller-Christian,Syndrome, Hand-Schüller-Christian,Systemic Aleukemic Reticuloendothelioses,Type 2 Histiocytoses
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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