" /> Letterer-Siwe Disease - CISMeF





Preferred Label : Letterer-Siwe Disease;

NCIt related terms : Multifocal Multisystem Langerhans Cell Histiocytosis; Acute Disseminated Langerhans Cell Histiocytosis; Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis;

NCIt definition : A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia.;

Alternative definition : NICHD: A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems that may include the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia.;

Neoplastic status : Malignant;

ICD-O code : 9754/3;

Codes from synonyms : C96.0;

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31/07/2025


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