Histiocytosis, Letterer Siwe

A severe disease in which histiocytes start to multiply and attack the tissues or organs of the patient starting in infancy with a scaly, sometimes itchy rash on the scalp, ears, abdomen, and creases of the neck and face. Raw skin may provide a portal of entry for germs, leading to sepsis (bloodstream infection). There are often draining ears, swollen glands, enlargement of the liver and spleen, liver disease, anemia and neutropenia (low white blood cells). Loss of appetite, irritability, failure to thrive, and respiratory symptoms may occur. Because of their appearance, these children may be misdiagnosed as abused.

Letterer-Siwe disease is the most severe form of Langerhans cell histiocytosis.

Read Also:

  • Histiocytosis, Langerhans cell

    Histiocytosis in which the active histiocytes normally occur in the skin. See histiocytosis.

  • Histiocytosis, lipid

    A form of histiocytosis that affects lipid (fat) storage. Also known as Niemann- Pick disease, Erdheim-Chester disease.

  • Histiocytosis, malignant

    A term sometimes used to refer to a group of tumors including monocyte-related leukemias (for example, acute monocytic leukemia, acute myelomonocytic leukemia), extramedullary monocytic tumor, and histiocytic sarcoma. Treatment is by radiation and chemotherapy, and in some cases bone-marrow transplantation. See also histiocytosis.

  • Histiocytosis, sinus

    A type of histiocytosis in which the lymph nodes are the main site of histiocyte proliferation. The sinuses of the lymph nodes become filled with and distended by masses of histiocytes.

  • Histo

    1. Popular name for the fungus histoplasma. 2. Medical school slang for histology.

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