A rare but potentially deadly disorder with similarities to cancer, in which histiocytes start to multiply and attack the person’s own tissues or organs. The result can be tissue damage, pain, development of tumor-like lumps called granulomas, fatigue, and other symptoms. If histiocytosis affects the pituitary gland, diabetes insipidus may also develop. Treatment is by radiation and chemotherapy, although for reasons unknown, some cases go into remission without treatment.
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A generalized proliferation of histiocytes. SYN: histocytosis.
- Langerhans cell h. a set of closely related disorders unified by a common proliferating element, the Langerhans cell. Three overlapping clinical syndromes are recognized: a single site disease (eosinophilic granuloma), a multifocal unisystem process (Hand-Schuller-Christian syndrome), and a multifocal, multisystem h. (Letter-Siwe syndrome.) Formerly this process was known as h. X. SYN: h. X.
- lipid h. h. with cytoplasmic accumulation of lipid, either phospholipid (Niemann-Pick disease) or glucocerebroside (Gaucher disease).
- malignant h. a rapidly fatal form of lymphoma, characterized by fever, jaundice, pancytopenia, and enlargement of the liver, spleen, and lymph node s; the affected organs show focal necrosis and hemorrhage, with proliferation of histiocytes and phagocytosis of red blood cells.
- nodular non-X h. SYN: generalized eruptive histiocytoma.
- nonlipid h. SYN: Letterer-Siwe disease.
- sinus h. with massive lymphadenopathy a chronic disease occurring in children and characterized by massive painless cervical lymphadenopathy due to distension of the lymphatic sinuses by macrophages containing ingested lymphocytes, and by capsular and pericapsular fibrosis. SYN: Rosai-Dorfman disease.
- h. X SYN: Langerhans cell h..
- h. Y SYN: verrucous xanthoma.

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his·tio·cy·to·sis -'tō-səs n, pl -to·ses -.sēz abnormal multiplication of macrophages <Langerhans cell \histiocytosis> broadly a condition characterized by such multiplication

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any of a group of diseases in which there are abnormalities in certain large phagocytic cells (histiocyte) due to (1) abnormal storage of fats, as in Gaucher's disease; (2) inflammatory disorders, as in Langerhans cell histiocytosis, which includes disorders previously called histiocytosis X; or (3) malignant proliferation of histiocytes.

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his·tio·cy·to·sis (his″te-o-si-toґsis) any of a variety of proliferative disorders of macrophages (histiocytes), marked by the abnormal appearance of macrophages in the blood, or by abnormally elevated numbers of macrophages in lymphoid tissue.

Medical dictionary. 2011.

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  • Histiocytosis, sinus — A variant of histiocytosis in which the lymph nodes are the main site of histiocyte proliferation. The sinuses become filled with and distended by masses of histiocytes. See also histiocytosis …   Medical dictionary

  • Histiocytosis, Hand-Schuller-Christian — A disease in which histiocytes start to multiply and attack the tissues or organs of the patient. The disease usually affects children age 2 to 5, less often older children and adults. The most frequent sites of bony involvement are the flat… …   Medical dictionary

  • Histiocytosis, lipid — A form of histiocytosis that affects lipid (fat) storage. Also known as Niemann Pick disease, Erdheim Chester disease …   Medical dictionary

  • histiocytosis — his·ti·o·cy·to·sis (hĭs tē ō sī tōʹsĭs) n. pl. his·ti·o·cy·to·ses ( sēz) Any of several abnormal conditions characterized by the appearance of histiocytes in the blood or tissues. * * * …   Universalium

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