نتایج جستجو برای: familial histiocytosis
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A 45-year old male presented latero-cervical lymphoadenopathy. Biopsy revealed a malignant proliferation of immature "lymphoid" cells bearing T6 antigen and HLA-DR but negative for other lymphoid markers, suggesting a phenotype similar to Langerhans cells. The patient did not receive any therapy and six months later developed a histologically typical malignant histiocytosis, involving spleen an...
Smoking-induced diffuse interstitial lung processes include respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease (RBILD), desquamative interstitial pneumonia (DIP) and Langerhans' cell histiocytosis. The histological, radiological and clinical features of respiratory bronchiolitis, RBILD and DIP are reviewed, with particular reference to management issues; L...
Langerhan's cell histiocytosis (LCH) results from the proliferation of immunophenotypically and functionally immature, morphologically rounded Langerhan's cells along with eosinophils, macrophages, lymphocytes, and, commonly, multinucleated giant cells. Here we report a case in a 6-year-old boy of differential diagnoses including dermatopathic lymphadenitis (DL), parasitic infection, Kimura's d...
Indeterminate cell histiocytosis is a very rare disorder of histiocytes proliferation. It has both Langerhans and non-Langerhans cell histiocytosis immunophenotypic features. We described a 45-year-old man with a 2 years history of multiple yellow-brown papules and a few red nodules on his trunk and extremities. No internal involvement was detected first and after 8 months. As his lesions were ...
BACKGROUND Isolated mediastinal involvement in Langerhans cell histiocytosis (LCH) has been rarely reported. CASE CHARACTERISTICS A 3-month-old boy presented with history of low grade intermittent fever, cough and noisy breathing for 2 weeks. OBSERVATION A chest X-ray showed massive mediastinal widening. Biopsy of the mass confirmed LCH. OUTCOME Patient is doing well after one year of tre...
Sinus histiocytosis is a rare inflammatory disease mainly affecting the cervical lymph nodes, presenting with skin lesions in 10% of cases. Our patient had a solitary nodule on the trunk without any other clinical signs. The histology reported a dermal neoplasm composed mainly of macrophages and lymphocytes. Macrophages were aggregated in clusters resembling lymph node sinuses. Lymphophagocytos...
Histiocytoses are a heterogeneous group of diseases, characterized by the accumulation of reactive or neoplastic histiocytes in various tissues. Generalized eruptive histiocytosis belongs to cutaneous non-Langerhans' cell histiocytoses and is a rare, generalized, self-healing disorder that usually follows a benign clinical course. Herein, we report a case of generalized eruptive histiocytosis i...
PURPOSE To describe the MR findings in the hypothalamic pituitary area in children with Langerhans cell histiocytosis and to define those MR alterations especially associated with the risk of developing diabetes insipidus. METHODS The hypothalamic-neurohypophyseal axis was studied by sagittal and coronal 1.5 T1-weighted MR imaging in 14 children with Langerhans cell histiocytosis (five with d...
BACKGROUND Pulmonary Langerhans'-cell histiocytosis is an uncommon interstitial lung disease in adults. It has an unpredictable course and may be associated with an increased susceptibility to the development of malignant neoplasms. METHODS We reviewed the records of 102 adults with histopathologically confirmed pulmonary Langerhans'-cell histiocytosis to ascertain their vital status and whet...
INTRODUCTION We report the case of a 24-year-old Greek woman with histologically proven osseous and pulmonary Langerhans cell histiocytosis whose lesions had progressively regressed with a "switch on and off" mode. This is the first report in the literature of this mode of presentation of Langerhans cell histiocytosis. CASE PRESENTATION The patient had first presented at the age of 20 years w...
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