Pulmonary Langerhans Cell Histiocytosis Histology
This article deals with lch in general. In people with lch these cells multiply excessively and build up in certain areas of the body.
Langerhans Cell Histiocytosis Lch Thoracic Pathology A Volume
It is caused by a disorder of myeloid dendritic cells.
Pulmonary langerhans cell histiocytosis histology. Pulmonary langerhans cell histiocytosis has variable appearance depending on the stage of disease ranging from small peribronchiolar nodular opacities to multiple irregularly shaped cysts. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally but not invariably occurs in cigarette smokers. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body.
There is a mid and upper zone predilection 1 3 4. People with lch produce too many langerhans cells or histiocytes a form of white blood cell found in healthy people that is supposed to protect the body from infection. Langerhans cells which help regulate the immune system are normally found throughout the body especially in the skin lymph nodes spleen lungs liver and bone marrow in langerhans cell histiocytosis excess immature langerhans cells usually form tumors called.
It has been referred to by several eponyms hand schüller christian disease abt letterer siwe disease or letterer siwe disease and histiocytosis x. Proliferative disease or disorder of langerhans cells involving lung highly associated with smoking pulmonary langerhans cell histiocytosis plch is thought to be distinct from systemic langerhans cell histiocytosis lch most common pulmonary histiocytic lesion arch pathol lab med 2008 132 1171. The pathologic hallmark of plch is the accumulation of langerhans and other inflammatory cells in small airways resulting in the formation of nodular inflammatory lesions.
Langerhans cell histiocytosis lch is a rare cancer involving clonal proliferation of langerhans cells abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes symptoms range from isolated bone lesions to multisystem disease lch is part of a group of syndromes called histiocytoses which are characterized by an abnormal proliferation of histiocytes an. Pulmonary langerhans cell histiocytosis is a disease in which monoclonal cd1a positive langerhans cells a type of histiocyte infiltrate the bronchioles and alveolar interstitium accompanied by lymphocytes plasma cells neutrophils and eosinophils. Langerhans cell histiocytosis abbreviated lch is a rare disorder of tissue macrophages it broadly fits into the category of histiocytoses it used to known as eosinophilic granuloma.
Langerhans cell histiocytosis lch is a disorder that primarily affects children but is also found in adults of all ages. Pulmonary langerhans cell histiocytosis plch previously called eosinophilic granuloma of the lung pulmonary langerhans cell granulomatosis and pulmonary histiocytosis x is an uncommon cystic interstitial lung disease that primarily affects young adults.
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