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Since the first description of cryoglobulinemia in association with the clinical triad of skin purpura, joint pain, and weakness by Meltzer et al. in 1966, [4] [5] the percentage of cryoglobulinemic diseases described as essential cryoglobulinemia or idiopathic cryoglobulinemia (that is, cryoglobulinemic disease that is unassociated with an underlying disorder) has fallen.
Cryoglobulinemic vasculitis is a form of inflammation affecting the blood vessels caused by the deposition of abnormal proteins called cryoglobulins.These immunoglobulin proteins are soluble at normal body temperatures, but become insoluble below 37 °C (98.6 °F) and subsequently may aggregate within smaller blood vessels.
Cold agglutinins are antibodies, typically immunoglobulin M (), that are acquainted with and then binding the antigens on red blood cells, typically antigens "I" or "i" on the RBC surface, [1] in the environment in which the temperatures are lower than normal core body temperature and, thus, ends up leading to agglutinations of the red blood cells and hemolysis reaction occurring outside the ...
The diagnosis of secondary cryofibrinogenemia also requires evidence for the cited infectious, malignant, premalignant vasculitis, and autoimmune disorders while the diagnosis of primary cryofibrinogenemia requires a lack of evidence for 1) the cited associated disorders, 2) other vascular occlusive diseases, and 3) cryoglobulinemia.
This article provides a list of autoimmune diseases. These conditions, where the body's immune system mistakenly attacks its own cells, affect a range of organs and systems within the body. Each disorder is listed with the primary organ or body part that it affects and the associated autoantibodies that are typically found in people diagnosed ...
Secondary cold agglutinin syndrome occurs when autoantibodies bind to red blood cells, rendering them subject to attack by the complement system. [17] It is a result of an underlying condition potentially associated with either monoclonal cold-reacting autoantibodies or polyclonal cold-reacting autoantibodies [16] predominantly caused by infection or lymphoproliferative disorders. [16]
Paroxysmal cold hemoglobinuria (PCH) or Donath–Landsteiner hemolytic anemia (DLHA) is an autoimmune hemolytic anemia featured by complement-mediated intravascular hemolysis after cold exposure. [1] It can present as an acute non-recurrent postinfectious event in children, or chronic relapsing episodes in adults with hematological malignancies ...
Autoimmune hemolytic anemia: D59.0-D59.1: Autoimmune hemolytic anemia (AIHA) is a type of hemolytic anemia where the body's immune system attacks its own red blood cells (RBCs), leading to their destruction . [28] [29] Types of AIHA include warm autoimmune hemolytic anemia, cold agglutinin disease, and paroxysmal cold hemoglobinuria.