HISTIOCITOSIS DE LANGERHANS PDF

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.

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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. Updating… Please wait. Unable to process the form.

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Please use another browser until we can get it fixed. 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.

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Langerhans cell histiocytosis

The Spanish Association of Pediatrics has as one of its main objectives the dissemination of rigorous and updated scientific information on the different areas of pediatrics. Annals of Pediatrics is the Body of Scientific Expression of the Association and is the vehicle through which members communicate. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years. CiteScore measures average citations received per document published. Read more. SRJ is a prestige metric based on the idea that not all citations are the same. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact.

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Archivos de Bronconeumologia http: www. Other types of articles such as reviews, editorials, special articles, clinical reports, and letters to the Editor are also published in the Journal. It is a monthly Journal that publishes a total of 12 issues, which contain these types of articles to different extents. All manuscripts are sent to peer-review and handled by the Editor or an Associate Editor from the team.

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