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.

Author: Brarisar Arashigore
Country: Kuwait
Language: English (Spanish)
Genre: Relationship
Published (Last): 8 February 2011
Pages: 359
PDF File Size: 12.81 Mb
ePub File Size: 2.49 Mb
ISBN: 221-9-35044-597-9
Downloads: 33750
Price: Free* [*Free Regsitration Required]
Uploader: Tushakar

Views Read Edit View history. These diseases are related to other forms of abnormal proliferation of white blood cellssuch as leukemias and lymphomas. Robbins and Cotran Pathologic Basis of Disease 9th ed. CiteScore measures average citations received per document published. The Journal of Pathology.

September Pages Clinically, its manifestations range from isolated bone lesions to multisystem disease. CD1 positivity are more specific. The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cellssometimes called dendritic cell histiocytosis. 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.

Chest,pp. Organ involvement can also cause more specific symptoms.

There was a problem providing the content you requested

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. However systemic diseases often require chemotherapy.

All manuscripts are sent to peer-review and handled by the Editor or an Associate Editor from the team. SNIP measures contextual citation pulmknar by wighting citations based on the total histiocitosiw of citations in a subject field.


Recurrent cytogenetic or genomic abnormalities would also be required to demonstrate convincingly that LCH is a malignancy. Archived from the original on Gary 21 July Multifocal multisystem LCH, also called Letterer-Siwe diseaseis a rapidly progressing disease in which Langerhans Cell cells proliferate in many tissues.

S protein, peanut agglutinin, and transmission electron microscopy study”. It is mostly seen in children under age 2, and the prognosis is poor: Report from the International Registry of the Histiocyte Society”.

All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License. Endocrine deficiency often require lifelong supplement e. Solitary bone lesion may be amenable through excision or limited radiation, dosage of Gy for children, Gy for adults.

Presence of Birbeck granules on electron microscopy and immuno-cytochemical features e. Retrieved from ” https: Adult pulmonary Langerhans cell histiocytosis PLCH is a rare disorder of unknown etiology that occurs predominantly in young smokers, with an incidence peak at years of age. Micrograph showing a Langerhans cell histiocytosis with the characteristic reniform Langerhans cells accompanied by abundant eosinophils. Two independent studies have confirmed this finding.

Langerhans cell histiocytosis – Wikipedia

Dendritic cells ; Histiocytosis, Langehaus-cell ; Tobacco smoking. Local steroid cream is applied to skin lesions.

In the 10th episode of season 3 of House entitled “Merry Little Christmas”the primary patient is a girl with dwarfism who has a variety of symptoms, who is ultimately diagnosed with Langerhans cell histiocytosis. The latter may be evident in hostiocitosis X-rays with micronodular and interstitial infiltrate in the mid and lower zone of lung, with sparing of the Costophrenic angle or honeycomb appearance in older lesions.


LCH usually affects children between 1 histocitosis 15 years old, with a peak incidence between 5 and 10 years of age. Archivos de Bronconeumologia http: Orphanet Journal of Rare Diseases. Juvenile xanthogranuloma Hemophagocytic lymphohistiocytosis Erdheim-Chester disease Niemann—Pick disease Sea-blue histiocyte Benign cephalic histiocytosis Generalized eruptive histiocytoma Xanthoma disseminatum Progressive nodular histiocytosis Papular xanthoma Hereditary progressive mucinous histiocytosis Reticulohistiocytosis Multicentric reticulohistiocytosisReticulohistiocytoma Indeterminate cell histiocytosis.

LCH provokes a non-specific inflammatory responsewhich includes fever, lethargyand weight loss. After six months of follow up, the patient is in good conditions. The Journal is published both in Spanish and English.

The Journal of Clinical Endocrinology and Metabolism.

Murphy tried to diagnose Langerhans cell histiocytosis in a boy with a previously diagnosed osteosarcoma. 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. Translators working for the Journal are in charge histjocitosis the corresponding translations. Guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years”.