a Unidad Médico-Quirúrgica de Enfermedades Respiratorias. Área de Neumopatías Intersticiales y Ocupacionales. Hospital Universitario Virgen del Rocío. OSSES A, RODRIGO; GONZALEZ B, SERGIO; AGUIRRE Z, MARCIA SALDIAS P, FERNANDO. Histiocitosis de células de Langerhans pulmonar: Caso clínico. La histiocitosis de células de Langerhans es una enfermedad . Tras plantearse el caso como histiocitosis hepático y/o pulmonar).4 Su presentación.

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It is now considered a form of smoking-related interstitial lung disease. LCH is clinically divided into three groups: Recurrent cytogenetic or genomic abnormalities would also be required to demonstrate convincingly that LCH is a malignancy.

The name, however, originates back to its discoverer, Paul Langerhans. Assessment of endocrine function and bonemarrow biopsy are also performed when indicated. Ann Thoracic Surg, 30pp. Langerhans cell histiocytosis is occasionally misspelled as “Langerhan” or “Langerhan’s” cell histiocytosis, even in authoritative textbooks.

Continuing navigation will be considered as acceptance of this use. The Journal is published both in Spanish and English. When found in the lungs, it should be distinguished from Pulmonary Langerhans cell hystiocytosis—a special category of disease most commonly seen in adult smokers. Retrieved from ” https: Excellent for single-focus disease. After six months of follow up, the patient is in good conditions.

MRI and CT may show infiltration in sella turcica. In the 10th episode of season 3 of House entitled “Merry Little Christmas”the primary patient histiocitoeis a girl with dwarfism who has a variety of symptoms, who is ultimately diagnosed with Langerhans cell histiocytosis.


Histiocitosis de células de Langerhans pulmonar: Caso clínico

Peak onset is 2—10 years of age. International Journal of Pediatric Otorhinolaryngology. Nelson Textbook of Pediatrics 19th ed.

Journal of Clinical Pathology. Multifocal multisystem LCH, also called Letterer-Siwe diseaseis a rapidly progressing disease in which Langerhans Cell cells proliferate in many tissues. Robbins and Cotran pathologic basis of disease.

This page was last edited on 1 Decemberat Chest,pp. In other projects Wikimedia Commons. The Journal of Clinical Endocrinology and Metabolism. Micrograph showing a Langerhans cell histiocytosis with the characteristic reniform Langerhans pulmohar accompanied by abundant eosinophils. Chemotherapeutic agents such as alkylating agentsantimetabolitesvinca alkaloids either singly or in combination can lead to complete remission in diffuse disease.

A surgical biopsy confirmed the diagnosis of pulmonary Langerhans cell histiocytosis.

Int J Clin Exp Pathol. CS1 German-language sources de Infobox medical condition new All articles with unsourced statements Articles with unsourced statements from April Commons category link is locally defined. CD1 positivity are more specific. Archivos de Bronconeumologia http: The British Journal of Dermatology.

Langerhans cell histiocytosis – Wikipedia

In adults, pulmonary involvement with Langerhans cell histiocytosis usually occurs as a single-organ disease and is characterized by focal Langerhans cell granulomas infiltrating and destroying distal bronchioles.

Report from the International Registry of the Histiocyte Society”. Puulmonar is usually a sporadic and non- hereditary condition but familial clustering has been noted in limited number of cases. Previous article Next article.


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British Journal of Haematology. Are you a health professional able to prescribe or dispense drugs? Radiology will show osteolytic bone lesions and damage to the lung. Archived from the original on New England Journal of Medicine. It typically has no extraskeletal involvement, but rarely an identical lesion can be found in the skin, lungs, or stomach. Initially routine blood tests e.

Dendritic cells ; Histiocytosis, Langehaus-cell ; Tobacco smoking. Langerhans cell histiocytosis Micrograph showing a Langerhans cell histiocytosis with the characteristic reniform Langerhans cells accompanied by abundant eosinophils.

Views Read Edit View history. Langerhans cell histiocytosis LCH is a rare disease involving clonal proliferation of Langerhans cellsabnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Hematoxylin-eosin stain of biopsy slide will show features of Langerhans Cell e. This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases.

Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma. All manuscripts are sent to peer-review and handled by the Editor or an Associate Editor from the team.

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