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Childhood-onset systemic lupus erythematosus (i.e., cSLE), also termed juvenile-onset systemic lupus erythematosus, juvenile systemic lupus erythematosus, and pediatric systemic lupus erythematosus, is a form of the chronic inflammatory and autoimmune disease, systemic lupus erythematosus (i.e., SLE), that develops in individuals up to 18 years old. [1]
It is seen in systemic lupus erythematosus, where immune complexes aggregate with platelets, forming intravascular thrombi. Microangiopathic hemolytic anemia is also seen in cancer. [1] Microangiopathic hemolytic anemia may be suspected based on routine medical laboratory tests such as a CBC (complete blood cell count).
Lupus, formally called systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. [1] Symptoms vary among people and may be mild to severe. [ 1 ]
AIHA is a relatively rare condition, with an incidence of 5–10 cases per 1 million persons per year in the warm-antibody type and 0.45 to 1.9 cases per 1 million persons per year in the cold-antibody type. [4] Autoimmune hemolysis might be a precursor of later onset systemic lupus erythematosus. [5]
Immune-mediated causes could include transient factors as in Mycoplasma pneumoniae infection (cold agglutinin disease) [14] or permanent factors as in autoimmune diseases like autoimmune hemolytic anemia [15] (itself more common in diseases such as systemic lupus erythematosus, rheumatoid arthritis, Hodgkin's lymphoma, and chronic lymphocytic ...
It can be idiopathic, that is, without any known cause, drug-associated or secondary to another disease such as systemic lupus erythematosus, or a malignancy, such as chronic lymphocytic leukemia. [42] Cold agglutinin hemolytic anemia is primarily mediated by IgM. It can be idiopathic [43] or result from an underlying condition.
The idea behind the "mixed" disease is that this specific autoantibody is also present in other autoimmune diseases such as systemic lupus erythematosus, polymyositis, scleroderma, etc. MCTD was characterized as an individual disease in 1972 by Sharp et al., [3] [4] and the term was introduced by Leroy [5] in 1980. [6]
The direct Coombs test is used to test for autoimmune hemolytic anemia, a condition where the immune system breaks down red blood cells, leading to anemia. The direct Coombs test is used to detect antibodies or complement proteins attached to the surface of red blood cells. To perform the test, a blood sample is taken and the red blood cells ...