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Chronic granulomatous disease (CGD), also known as Bridges–Good syndrome, chronic granulomatous disorder, and Quie syndrome, [1] is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds (most importantly the superoxide radical due to defective phagocyte NADPH oxidase) used to kill certain ingested pathogens. [2]
Other common sites of involvement include the liver, spleen, skin, and eyes. The granulomas of sarcoidosis are similar to those of tuberculosis and other infectious granulomatous diseases. In most cases of sarcoidosis, though, the granulomas do not contain necrosis and are surrounded by concentric scar tissue .
Immunohistochemical staining of plasma cell granulomas is an important diagnostic method to show PCG lesions are also an entity within the IgG4-related diseases. [3] IHC staining is especially helpful because it allows for the assessment of the quantitative number of IgG4 positive plasma cells. [ 3 ]
The World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) is an organisation of physicians involved in the diagnosis and treatment of sarcoidosis and related conditions. [171] WASOG publishes the journal Sarcoidosis, Vasculitis and Diffuse Lung Diseases. [172]
Splenomegaly is an enlargement of the spleen. [1] The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen.Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the ...
These include such varied disturbances as xanthoma disseminatum, [6] ceroid granuloma of the gallbladder, Whipple's disease, [7] inflammatory pseudotumor of the lung, [8] [9] plasma cell granuloma of the lung, [10] malakoplakia, [11] verruciform xanthoma, foamy histiocytosis of the spleen in thrombocytopenic purpura, [12] [13] isolated xanthoma ...
The disease is still sometimes confused with lethal midline granuloma and lymphomatoid granulomatosis, both malignant lymphomas. [ 34 ] The full clinical picture was first presented by Friedrich Wegener (1907–1990), a German pathologist , in two reports in 1936 and 1939, leading to the eponymous name Wegener's granulomatosis or Wegener ...
Lymphomatoid granulomatosis (LYG or LG) is a very rare lymphoproliferative disorder first characterized in 1972. [1] Lymphomatoid means lymphoma-like and granulomatosis denotes the microscopic characteristic of the presence of granulomas with polymorphic lymphoid infiltrates and focal necrosis within it.