Histiocytosis X (HX) is a rare disease. There are many questions and debates in its etiology, pathology, clinical type and therapy. To enhance the recognition of otolaryngologists in HX of the ear and temporal bone and the level of diagnosis and therapy, 30 years' results of 21 patients with HX of the ear and temporal bone proved by histiopathology were reviewed, 18 patients were cured (85%), 6...

Fibreoptic broncho-alveolar lavage was used in four patients; the diagnosis of histiocytosis X had been established by lung biopsy in three and was suggested on clinical grounds in the remaining patient. Characteristic cells with an ultrastructural cytoplasmic marker (X body) were found in the washes of all four patients. In the patient without biopsy confirmation, the findings in the broncho-a...

The authors present a rare case of C-6 vertebral involvement in a 12-year-old boy with histiocytosis X. The patient presented with limitation in movements of neck and upper extremities. Computed tomography (CT) and magnetic resonance (MRI) imaging were used in the preoperative workup. Surgery was performed via an anterior cervical approach along with stabilization using a fibula strut graft and...

This study describes the chest radiographs of 50 adult patients with histologically verified histiocytosis X, proposes a radiological classification, and examines the role of radiology in assessing the prognosis of the disease. Radiologically the lesions predominate in the middle and lower lung fields, usually sparing the costophrenic angles, and are typically micronodular, reticular, or cystic...

The immunological phenotype of the cells involved in skin and lymph node lesions from two cases of histiocytosis X (H-X) were analysed by immunofluorescence techniques using combinations of heterologous and monoclonal antisera to Ia-like antigen and human cortical thymocyte (HTA-1) determinant. These cells were also characterised by a new technique using simultaneous immunofluorescence and enzy...

SYNTHESIS Vasopressin is synthesised in hypothalamic neurones, predominantly concentrated in the supraoptic and paraventricular nuclei, which appear to be separate from those concerned in oxytocin synthesis. After synthesis, which may begin with the formation of a high molecular weight, biologically-inactive precursor molecule (Sachs and Takabatake, 1964), the vasopressin is packaged together w...

Five cases of bronchogenic carcinoma were observed among 93 patients with pulmonary histiocytosis X (Hx). Mean age at the time of diagnosis of Hx was 42 years; on the average, cancer occurred 10.5 years later. All patients were smokers and continued to smoke heavily at the time of diagnosis of cancer. Comparison of the five cases associating Hx and lung carcinoma with a group of 88 control pati...

Division and proliferation of dendritic cells (DCs) have been proposed to contribute to homeostasis and to prolonged antigen presentation. Whether abnormal proliferation of dendritic cells causes Langerhans cell histiocytosis (LCH) is a highly debated topic. Transgenic expression of simian virus 40 (SV40) T antigens in mature DCs allowed their transformation in vivo while maintaining their phen...

Langerhans cell histiocytosis (LCH) is a rare disease, of unknown pathogenesis, characterized by intense and abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). It can present both local and systemic manifestations involving bone, skin and mucosal tissue, and internal organs. Three basic clinical forms develop: Letterer-Siwe disease (subacute or acute disseminated form...

Accepted 29 March 1996 In 1953, Lichtenstein introduced the unifying term 'histiocytosis X' to embrace several previous eponyms, including eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian disease.' In 1987, a more prominent designation, Langerhans cell histiocytosis (LCH), was recommended to replace histiocytosis X.' The term is more appropriate as it reflects the fact ...