Disease Information for Histiocytosis X: Definition

  • Group of disorders of histiocyte proliferation which includes LETTERER-SIWE DISEASE; HAND-SCHUELLER-CHRISTIAN SYNDROME; and EOSINOPHILIC GRANULOMA. Langerhans cells are components of the lesions;-----------------------------------------------Langerhans Cell Histiocytosis; Histiocytosis X

    LCH; Langerhans-Cell Granulomatosis; Non-Lipid Reticuloendotheliosis; Systemic Aleukemic Reticuloendotheliosis; Type II Histiocytosis; Subdivisions:

    Abt-Letterer-Siwe Disease; Eosinophilic Granuloma; Hand-Schueller-Christian Syndrome; Hashimoto-Pritzker Syndrome;

    Letterer-Siwe Disease; Pure Cutaneous Histiocytosis;

    Self-Healing Histiocytosis; Langerhans cell histiocytosis (LCH) is a rare spectrum of disorders characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the various tissues and organs of the body (lesions); The lesions may include certain distinctive granule-containing cells (Langerhans cells) involved in certain immune responses, as well as other white blood cells ( monocytes, eosinophils); Associated symptoms and findings may vary from case to case, depending upon the specific tissues and organs affected and the extent of involvement;Most affected individuals have single or multiple bone lesions characterized by degenerative changes and loss of the calcium of bone (osteolysis); Although the skull is most commonly affected, there may also be involvement of other bones, such as those of the spine (vertebrae) and the long bones of the arms and legs; Affected individuals may have no apparent symptoms (asymptomatic), and may experience associated pain and swelling, and/or develop certain complications, such as fractures or secondary compression of the spinal cord; In some cases, other tissues and organs may also be affected, including the skin, lungs, or other areas; In some individuals, LCH may be associated with involvement of the pituitary gland leading to diabetes insipidus; The exact cause of Langerhans cell histiocytosis is unknown