Search results for " Langerhans-Cell"
showing 5 items of 5 documents
Clinical and immunohistochemical evaluation of the vulvar Langerhans cell histiocytosis.
2008
: We present the case of a woman with diabetes insipidus with subsequent genital and multiorgan Langerhans cell histiocytosis (LCH). A monolateral and slightly infiltrated erythematous plaque of the vulva was observed. Hematoxylin and eosin and immunophenotypic studies were performed. The primary antibodies used were monoclonal antibody to S100, CD1a, CD34, HLA-DR, PCNA, CD45Ro, CD40, and langerin. The histology of the infiltrates revealed a granulomatous reaction pattern, with extensive aggregates of histiocyte proliferation. The histiocytes, morphologically characterized by a pale staining of cytoplasm surrounding a grooved reniform nucleus, sometimes contained small distinct nucleoli. L…
BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups.
2014
BRAF-V600E expression is identified in hematopoietic progenitor and precursor myeloid dendritic cells in patients with high-risk LCH, and enforced expression of BRAF-V600E in CD11c+ cells recapitulates a high-risk LCH-like phenotype in mice.
Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification
2007
Multisystem Langerhans cell histiocytosis (MS-LCH) is associated with high mortality when patients have risk organ involvement (RO+) or are younger than 2 years. In an international randomized trial, LCH-II, we intensified their treatment: arm A consisted of 6 weeks of daily prednisone and weekly vinblastine followed by 18 weeks of daily 6-mercaptopurine with vinblastine/prednisone pulses; etoposide was added in arm B. Considering all 193 randomized risk patients, there were similar outcomes: rapid (6 weeks) response (arm A vs arm B: 63%/71%), 5-year survival probability (74%/79%), disease reactivation frequency (46%/46%), and permanent consequences (43%/37%). However, (1) patients younger …
Langerhans's cell histiocytosis in old subjects: two rare case reports and review of the literature
2012
Background: Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children; LCH onset is rare in adults; immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. Objective: To describe two new cases of adult onset oral LCH. Case reports: Case 1: a 71-year-old woman, complaining of diffuse oral pain, presented with erythematous mucosal lesions; the panoramic radiograph and CT scan showed multiple mandible radiolucent areas. Immunohistochemical assay for S-100, CD1a and langerin test was essential in reaching the correct diagnosis. Case 2: a 77-year-old female patient presented with a…
Successful treatment of adult multisystemic Langerhans cell histiocytosis with psoralen-UV-A, prednisolone, mercaptopurine, and vinblastine.
2008
Background Langerhans cell histiocytosis (LCH) is a rare disease with a peak incidence in childhood. There is limited experience with treatment options for adult patients having multisystemic LCH involvement. We report successful treatment of a 70-year-old woman with adult onset of LCH and multisystem disease (diabetes insipidus centralis, bone marrow infiltration, and lung and skin involvement). Observations A 70-year-old woman with erythematous plaques and papules of the submammary and inguinal skin attended our outpatient clinic and was diagnosed as having LCH. Organ involvement was found in the infundibulum of the pituitary gland, associated with diabetes insipidus centralis, bone marro…