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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.
First described in 1961 (where they were simply termed "characteristic granules"), [2] they are solely found in Langerhans cells. [1] Although part of normal Langerhans cell histology, they also provide a mechanism to differentiate Langerhans cell histiocytoses (which are a group of rare conditions collectively known as histiocytoses ) from ...
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]
Sickle cell disease is a group of inherited blood disorders, caused by a genetic abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. [57] Under certain circumstances, this leads to the red blood cells adopting an abnormal sickle -like shape; with this shape, they are unable to deform as they pass through capillaries ...
Light microscopic image of bone marrow showing stromal macrophages containing numerous red blood cells in their cytoplasm. The blood count typically shows decreased numbers of blood cells—including a decreased number of circulating red blood cells, white blood cells, and platelets. The bone marrow may show hemophagocytosis. The liver function ...
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.
Erdheim–Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis). It was declared a histiocytic neoplasm by the World Health Organization in 2016. [1]