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    Langerhans Cell Histiocytosis Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Adult LCH



    Liver involvement in adults has been reported in 27% of a series of adult patients with LCH and multiorgan disease.[22] Hepatomegaly (48%) and liver enzyme abnormalities (61%) were present. CT and ultrasound imaging abnormalities are often found. The early histopathologic stage of liver LCH includes infiltration of CD1a+ cells and periductal fibrosis with inflammatory infiltrates with or without steatosis. The late stage is biliary tree sclerosis and treatment with ursodeoxycholic acid is suggested.

    Multisystem disease

    In a large series of patients from the Mayo Clinic, 31% had multisystem LCH compared with 69% registered on the Histiocyte Society adult registry; this likely reflects referral bias.[6,23] In the adult multisystem patients, the organs involved included the following:

    • Skin (50%).
    • Mucocutaneous (40%).
    • Diabetes insipidus (29.6%).
    • Hepatosplenomegaly (16%).
    • Hypothyroidism (6.6%).
    • Lymphadenopathy (6%).


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    This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http:// cancer .gov or call 1-800-4-CANCER.

    WebMD Public Information from the National Cancer Institute

    Last Updated: 8/, 015
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