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Treatment with these drugs is exorbitantly expensive and may be needed for life in dogs with continuously active disease, which usually is the case in advanced SH. It is preferable not to invoke such powerful immuno-suppressive therapy in most cases of CH in which spontaneous regression of lesions or episodic disease activation is more likely ...
The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cells, sometimes called dendritic cell histiocytosis. These cells in combination with lymphocytes , eosinophils , and normal histiocytes form typical LCH lesions that can be found in almost any organ . [ 5 ]
The mutations causes white blood cells (lymphocytes, macrophages, and eosinophils) to move towards dendritic cells, resulting in damage in any organ except the heart and kidneys. [ 1 ] The disease was once thought to be a lipid storage disease as the lesions have a high cholesterol content, but the blood cholesterol is usually normal.
A similar disease in humans, Hashimoto-Pritzker disease, is also a Langerhans cell histiocytosis. [1] Dog breeds that may be more at risk for this tumor include Bulldogs, American Pit Bull Terriers, American Staffordshire Terriers, Scottish Terriers, Greyhounds, Boxers, and Boston Terriers. They also rarely occur in goats and cattle. [2]
Langerhans cell is represented by a yellow oval; blue arrows correspond to is_a relations, and orange arrows correspond to develops_from relations. Only a subset of Langerhans cell parent types are included in the figure. [1] A Langerhans cell (LC) is a tissue-resident macrophage of the skin [2] once thought to be a resident dendritic cell. [3]
Letterer–Siwe disease, (LSD) or Abt-Letterer-Siwe disease, is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH) and is the most severe form, involving multiple organ systems such as the skin, bone marrow, spleen, liver, and lung. Oral cavity and gastrointestinal involvement may also be seen.
The Histiocyte Society, a nonprofit organization, is a group of more than 200 physicians and scientists from around the world committed to improving the lives of patients with histiocytic disorders by conducting clinical and laboratory research into the causes and treatment of this disease. The Society has instituted several clinical trials and ...
Indeterminate cell histiocytosis (LCH) is an uncommon proliferative illness where the predominant cells have characteristics from both non-Langerhans cell histiocytosis (NLCH) and Langerhans cell histiocytosis (LCH) in terms of morphology and immunophenotypic characteristics. [1]