L’histiocytose X de l’adulte est rare. Il faut savoir l’évoquer devant des lacunes osseuses cerclées d’un liseré d’autant qu’il existe des extensions dans les. Request PDF on ResearchGate | On Dec 31, , J. Corouge and others published Histiocytose X. Download Citation on ResearchGate | Histiocytose X: à propos d’un cas | Langherans’ cell histiocytosis is rare in adults. It should be considered in patients with.

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On the other hand, histiicytose infiltration of organs by monoclonal population of pathologic cells, and the successful treatment of subset of disseminated disease using chemotherapeutic regimens are all consistent with a neoplastic process.

Writing Group of the Histiocyte Society”. Two independent studies have confirmed this finding. Excellent for single-focus disease. Contact Help Who are we? Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma.

The American Journal of Surgical Pathology. Multifocal multisystem C, also called Letterer-Siwe diseaseis a rapidly progressing disease in which Langerhans Cell cells proliferate in many tissues.

Three syndromes are actually the same pathogenic process: You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted. 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.


Local steroid cream is applied to skin lesions.

Histiocytose langerhansienne – EM|consulte

Organ involvement can also cause more specific symptoms. The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties. The first patient underwent surgical resection of the tumor. The pathogenesis of Langerhans cell histiocytosis LCH is a matter of debate. Langerhans cell histiocytosis Micrograph showing a Langerhans cell histiocytosis with the characteristic reniform Langerhans cells accompanied by abundant eosinophils.

Chemotherapeutic agents such as alkylating agentsantimetabolitesvinca alkaloids either singly or in combination can lead to complete remission in diffuse disease.

The Journal of Clinical Endocrinology and Metabolism. Access to the full text of this article requires a subscription.

Initially routine blood tests e. By using this site, you agree to the Terms of Use and Privacy Policy. Hematoxylin-eosin stain of biopsy slide will show features of Langerhans Cell e.

The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties. It can be a monostotic involving only one bone or polyostotic involving more than one bone disease.

Histiocytose langerhansienne cérébrale

Robin; Hoang, Mai P. Seen mostly in children, multifocal unisystem LCH is characterized by fever, bone lesions and diffuse eruptions, usually on the scalp and in the ear canals. Report from the International Registry of the Histiocyte Society”. Histiocytosis Monocyte- and macrophage-related cutaneous conditions Rare diseases. LCH is clinically divided into three groups: Presence of Birbeck granules on electron histipcytose and immuno-cytochemical features e. These cells in combination with lymphocyteseosinophilsand histiocytosee histiocytes form typical LCH lesions that can be found in almost any organ.


Use of systemic steroid is common, singly or adjunct to chemotherapy. Orphanet Journal of Rare Diseases. American Journal of Clinical Pathology.

Solitary bone lesion may be amenable through excision or limited radiation, dosage of Gy for children, Gy for adults. It typically has no extraskeletal involvement, but rarely an identical lesion can be found in the skin, lungs, or stomach.

Micrograph showing a Langerhans cell histiocytosis histjocytose the characteristic reniform Langerhans cells accompanied by abundant eosinophils.

Histiocytose langerhansienne cérébrale

The radiological appearance is also variable; histological proof is required for diagnosis. Journal of the American Academy of Dermatology. You can move this window by clicking on the headline. Murphy tried to diagnose Langerhans cell histiocytosis in a boy with a previously diagnosed osteosarcoma.

Journal of Clinical Pathology. Assessment of endocrine function and bonemarrow biopsy are also performed when indicated.