HistiocytosisThe histiocytoses have been known by a variety of terms. Histiocytosis is a generic name for a group of syndromes characterized by an abnormal increase in the number of certain immune cells called histiocyte cells. These include monocytes, macrophages, and dendritic cells. There are 3 major classes of histiocytoses, but this article focuses on Langerhans cell histiocytosis, which is also called histiocytosis X ( Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. This tennis racquet shaped cytoplasmic organelle has an unknown function. Currently, the preferred term is Langerhans cell histiocytosis). The other 2 classes are non-Langerhans cell histiocytosis (also known as hemophagocytic syndrome) and malignant histiocytosis syndrome (now known as T-cell lymphoma). Treatment of HistiocytosisVinblastine is indicated for treatment of Histiocytosis X (Letterer-Siwe disease; Langerhans cell histiocytoses). This disorder is treated with corticosteroids, which suppress immune function (including the dangerous cells). Smoking may worsen the response to treatment and should be stopped. Most forms of histiocytosis are rare. They may occur at any age including during infancy and childhood. Symptoms range from none to severe. Some forms of histiocytosis resolve without treatment, but others may have a fatal outcome. |
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