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Langerhans cell histiocytosis LCH is a rare multisystem disease with a wide and heterogeneous clinical spectrum and variable extent of involvement. Langerhans cell histiocytosis was previously known as histiocytosis X.
The newer term is preferred as it's more descriptive of its cellular background, and removes the ambiguity of the connotation "X". The disease is more common in the pediatric population, with a peak incidence between one and three years of age 5. The course of the disease ranges from those that spontaneously regress to those that have a rapidly progressive course the latter is especially common in young children with multisystem disease.
Historically, three two eponymous forms have been recognized, although there is some confusion as to their definition :. A more useful and less controversial classification, which roughly correlates to the eponymous diseases above, is as follows:. As well as systemic disease, individual organ systems may be involved, which will be discussed separately:. Langerhans cell histiocytosis is due to uncontrolled monoclonal proliferation of Langerhans cells distinctive cells of monocyte-macrophage lineage and should be considered a malignancy although its biological behavior is very variable 1,3.
An immune-mediated mechanism has been postulated. This proliferation is accompanied by inflammation and granuloma formation. Electron microscopy may reveal characteristic Birbeck granules. Immunohistochemistry reveals expression of the following antigens:. Imaging features are often not pathognomonic and tissue diagnosis is usually required for definitive diagnosis.
As LCH can affect most organ systems, radiographic appearances are discussed separately see above. The prognosis can be extremely variable with eosinophilic granuloma carrying the best and Letterer-Siwe disease carrying the worst prognosis.
The prognosis is more closely related to the disease burden rather than histological features, although frankly malignant features Langerhans cell sarcoma do also have an impact on survival 3,4 :.
The Langerhans cell was discovered within the epidermis by German physician Paul Langerhans in when he was a medical student and working under famed Professor Rudolf Virchow 9.
The differential diagnosis for bone tumors is dependent on the age of the patient, with a very different set of differentials for the pediatric patient. Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys.
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On this page:. Quiz questions. Radiology review manual. Read it at Google Books - Find it at Amazon. Related Radiopaedia articles Bone tumours The differential diagnosis for bone tumors is dependent on the age of the patient, with a very different set of differentials for the pediatric patient. Edit article Share article View revision history Report problem with Article. URL of Article. Article information. Support Radiopaedia and see fewer ads. Cases and figures. Case 1: eosinophilic granuloma of bone Case 1: eosinophilic granuloma of bone.
Case 4 Case 4. Case 5 Case 5. Case 6 Case 6. Case 7: lytic lesion on left iliac bone Case 7: lytic lesion on left iliac bone. Case 8: with a vertebra plana Case 8: with a vertebra plana.
Case enhancing lesion in right iliac blade Case enhancing lesion in right iliac blade. Case 11 Case Case punched out lytic lesions Case punched out lytic lesions. Case in an adult Case in an adult. Case 14 Case Loading more images Close Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Loading Stack - 0 images remaining. By System:.
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Langerhans cell histiocytosis
Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Langerhans cell histiocytosis LCH is a systemic disease associated with the proliferation and accumulation usually in granulomas of Langerhans cells in various tissues.
Langerhans cell histiocytosis LCH is a rare multisystem disease with a wide and heterogeneous clinical spectrum and variable extent of involvement. Langerhans cell histiocytosis was previously known as histiocytosis X. The newer term is preferred as it's more descriptive of its cellular background, and removes the ambiguity of the connotation "X". The disease is more common in the pediatric population, with a peak incidence between one and three years of age 5. The course of the disease ranges from those that spontaneously regress to those that have a rapidly progressive course the latter is especially common in young children with multisystem disease.
Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. 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 granulomas. Many researchers now consider Langerhans cell histiocytosis to be a form of cancer, but this classification remains controversial. In approximately 80 percent of affected individuals, one or more granulomas develop in the bones, causing pain and swelling. The granulomas, which usually occur in the skull or the long bones of the arms or legs, may cause the bone to fracture.
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 archaic term for activated dendritic cells and macrophages. These diseases are related to other forms of abnormal proliferation of white blood cells , such as leukemias and lymphomas. The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cells , sometimes called dendritic cell histiocytosis. These cells in combination with lymphocytes , eosinophils , and normal histiocytes form typical LCH lesions that can be found in almost any organ.