Request PDF on ResearchGate | Histiocitosis X: presentación de tres casos | Background: histiocytosis of Langerhans´cell´s or also known as histiocytosis X is a. Request PDF on ResearchGate | Histiocitosis X | Histiocytosis X is an uncommon pulmonary interstitial disease that affects young adults and is associated to an. Background: histiocytosis of Langerhans´cell´s or also known as histiocytosis X is a group of diseases among which are found eosinophilic granuloma.

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European Journal of Cancer. There are ongoing investigations to determine whether LCH is a reactive non-cancerous or neoplastic cancerous process. LCH usually affects children between 1 and 15 years old, with a peak incidence between 5 and 10 years of age.

The prognosis can be extremely variable with eosinophilic granuloma carrying the best and Letterer-Siwe disease-carrying the worst prognosis. The name, however, originates back to its discoverer, Paul Langerhans. As well as systemic disease, individual organ systems may be involved, which histipcitosis be discussed separately:.

Langerhans cell histiocytosis Dr Francis Deng and A. Essentially any part of the body can be affected and as such clinical presentation will depend on specific involvement.

These diseases are related to other forms of abnormal proliferation of white blood cellssuch as leukemias and lymphomas. Micrograph showing a Langerhans cell histiocytosis with the characteristic reniform Langerhans cells accompanied by abundant eosinophils.

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.

Arguments supporting hjstiocitosis reactive nature of LCH include the occurrence of spontaneous remissions, the extensive secretion of multiple cytokines by dendritic cells and bystander-cells a phenomenon known as cytokine storm in the lesional tissue, favorable prognosis and relatively good survival rate in patients without organ dysfunction or risk organ involvement.


British Journal of Haematology. It is now considered a form of smoking-related interstitial lung disease. New England Journal of Medicine. Log in Sign up. LCH is clinically divided into three groups: 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. To quiz yourself on this article, log in to see multiple choice questions. When found in the lungs, histiociosis should be distinguished from Histipcitosis Langerhans histiocitosia hystiocytosis—a special category of disease most commonly seen in adult smokers.

Histiocitosis X – ScienceDirect

Clinically, its manifestations range from isolated bone lesions to multisystem disease. As LCH can affect most organ systems, radiographic appearances are discussed separately see above. Related Radiopaedia articles Bone tumours The differential histuocitosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the paediatric patient.

HSC of base of skull. In the 10th episode of season 3 of House entitled “Merry Little Christmas”the primary patient is a girl histioocitosis dwarfism who has a variety of symptoms, who is ultimately diagnosed with Langerhans cell histiocytosis.

Unifocal LCH, also called eosinophilic granuloma an older term which is now known to be a misnomeris a slowly progressing disease characterized by an expanding proliferation of Langerhans cells in various bones. SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. Langerhans histiocitozis histiocytosis LCH is a rare multi-system disease with a wide and heterogeneous clinical spectrum and variable extent of involvement.

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.

The Journal of Clinical Endocrinology and Metabolism.

Local steroid cream is applied to skin lesions. CS1 German-language sources de Infobox medical condition new All articles with unsourced statements Articles with unsourced statements from April Commons category with local link different than on Wikidata.


Langerhans cell histiocytosis

The pathogenesis of Langerhans cell histiocytosis LCH is a matter of debate. Electron microscopy may reveal characteristic Birbeck granules. The prognosis is more closely histilcitosis to the disease burden rather than histological features, although frankly malignant features Langerhans cell sarcoma do also have an impact on survival Use of systemic steroid is common, singly or adjunct to chemotherapy. Histiocirosis item has received.

Wikimedia Commons has media related to Langerhans Cell Histiocytosis. Imaging features are often not pathognomonic and tissue diagnosis is usually required for definitive diagnosis. Case 4 Case 4. Solitary bone lesion may be amenable through excision or limited radiation, dosage of Gy for children, Gy for adults.

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Langerhans cell histiocytosis | Radiology Reference Article |

Gary 21 July Hematoxylin-eosin stain of biopsy slide will show features of Langerhans Cell e. Diagnosis is confirmed histologically histiocitsis tissue biopsy.

April Pages There is also a male predilection M: Robin; Hoang, Mai P. Immunohistochemistry reveals expression of the following antigens:. Robbins and Cotran Pathologic Basis of Disease 9th ed.

All articles are subjected to a rigorous process of revision in pairs, and careful editing for literary and scientific style. Subscribe to our Newsletter. Historically, three eponymous forms have been recognised, although there is some confusion as to their definition