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Autoimmune hemolytic anemia associated with "cold" antibodies
Last reviewed: 19.10.2021
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Erythrocyte antibodies, which become more active at low body temperature, are called "Cold" antibodies. These antibodies belong to the IgM class, the presence of complement is necessary for the development of their activity, IgM activate complement in the limbs (hands, feet), where the temperature is lower than in other parts of the body; The cascade of complement reactions is interrupted when the red blood cells move to the warmer zones of the body. In 95% of healthy people, natural cold agglutinins are found in low titres (1: 1, 1: 8, 1:64). In the presence of an extremely high titer of "cold" antibodies, severe episodes of intravascular hemolysis with hemoglobinemia and hemoglobinuria and occlusion of the microcirculatory bed can develop when the patient undercools.
Autoimmune hemolytic anemia with complete cold agglutinins occurs in the presence of idiopathic parairothein or as a consequence of mycoplasmal infection, infection with the Epstein-Barr virus, including infectious mononucleosis, cytomegalovirus infection, mumps, or lymphoma. Cold agglutinins are generally polyclonal and directed against erythrocyte I antigens (for infections caused by Mycoplasma pneumoniae) or i (for infections caused by the Epstein-Barr virus). Polyclonal cold agglutinins, in addition, can be produced with cytomegalovirus infection, listeriosis, epidemic parotitis, syphilis, systemic connective tissue diseases. Monoclonal cold antibodies are produced with Waldenstrom's macroglobulinemia, lymphoma, chronic lymphocytic leukemia, Kaposi's sarcoma, myeloma. Hemolysis self-limited, symptomatic treatment.
Paroxysmal cold hemoglobinuria is a rare form of hemolytic anemia, in which there are "cold" antibodies of a specific type (Donat-Landsteiner hemolysins) that have anti-beta specificity. The disease can be both idiopathic and caused by viral infections (parotitis, measles) or tertiary syphilis. The main thing in treatment is to rule out the possibility of hypothermia.
Older people (50-80 years old) may have a chronic disease with Cold agglutinin syndrome, which is often associated with the formation of monoclonal antibodies IgM and lymphoproliferative disorders (chronic lymphocytic leukemia, Waldenstrom's macroglobulinemia). As a rule, mild anemia occurs, but sometimes severe intravascular hemolysis and kidney failure develop. In all cases, it is necessary to eliminate supercooling; can be prescribed corticosteroids. Conducting splenectomy is not recommended, since red blood cells are eliminated mainly in the liver, and not in the spleen.
Diagnostics
The diagnosis of autoimmune hemolytic anemia is confirmed by positive direct breakdown of Coombs (direct antiglobulin test) or sensitized Coombs breakdown (polybrene test). The use of anti-IgG, IgM and C3d antibodies in the Coombs reaction makes it possible to determine the antibody isotype and complement fixation. With a negative Coombs test, the detection of antibodies associated with erythrocytes should be taken into account only if there are other signs of autoimmune hemolysis. An indirect test of Coombs, which reveals anti-erythrocyte antibodies in the blood serum, has nothing to do with the diagnosis of autoimmune hemolytic anemia.