$Unique_ID{BRK03461} $Pretitle{} $Title{Anemia, Hemolytic, Cold Antibody} $Subject{Anemia Hemolytic Cold Antibody Cold Agglutinin Disease Cold Antibody Disease Anemia Autoimmune Hemolytic Paroxysmal Cold Hemoglobinuria Anemia Warm-Antibody Hemolytic} $Volume{} $Log{} Copyright (C) 1990 National Organization for Rare Disorders, Inc. 723: Anemia, Hemolytic, Cold Antibody ** IMPORTANT ** It is possible that the main title of the article (Anemia, Cold Antibody Hemolytic) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Cold Agglutinin Disease Cold Antibody Disease Anemia, Autoimmune Hemolytic Information on the following diseases can be found in the Related Disorders section of this report: Paroxysmal Cold Hemoglobinuria Anemia, Warm-Antibody Hemolytic General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Cold Antibody Hemolytic Anemia is a rare autoimmune disorder in which red blood cells are attacked and destroyed by the body's natural defenses (antibodies) in temperatures of 32 degrees centigrade or below. Symptoms In Cold Antibody Hemolytic Anemia, cold temperatures cause certain proteins which normally attack bacteria (IgM antibodies) to attach themselves to red blood cells and bind them together into clumps (agglutination). The antibodies activate complement in the blood causing changes in the red cell surface membrane that results in their removal from the circulation macrophages. Scientists believe that this binding of antibodies and red blood cells occurs in areas farthest away from the center of the body such as ear lobes, fingertips, the tip of the nose and the cheeks where temperatures can be as low as 32 to 28 degrees Centigrade for periods of time, particularly in cold climates. Individuals with cold antibody hemolytic anemia may show symptoms of weakness, dizziness, headache, ringing in the ears (tinnitus), spots before the eyes, fatigue, drowsiness, irritability or bizarre behavior. Absent menstruation (amenorrhea), gastrointestinal complaints, yellowing of the skin (jaundice) and enlargement of the spleen (splenomegaly) may also occur. Heart failure or shock may result. More rarely, there may be a passing of dark urine (hemoglobinuria). Causes The exact cause of cold antibody hemolytic anemia is not known. Autoimmune disorders occur when the body's natural defenses (antibodies, lymphocytes, etc.) against invading organisms suddenly begin to attack perfectly healthy tissue for unknown reasons. Affected Population Cold Antibody Hemolytic Anemia most commonly affects older people. Those individuals with infectious mononucleosis, lymphoproliferative diseases, or mycoplasma pneumonia are more susceptible to this disorder. Related Disorders Symptoms of the following disorders can be similar to those of Cold Antibody Hemolytic Anemia. Comparisons may be useful for a differential diagnosis: Paroxysmal Cold Hemoglobinuria (PCH) is characterized by a disintegration of cells (hemolysis) occuring minutes to hours after exposure to cold. Symptoms include severe pain in the back and legs, headache, vomiting, diarrhea and passage of dark brown urine. There may be temporary enlargement of the liver and spleen. This disorder is frequently associated with viral infections such as chickenpox and mumps. (For more information on this disorder, choose "Paroxysmal Cold Hemoglobinuria" as your search term in the Rare Disease Database.) Warm-Antibody Anemia is a disorder characterized by destruction of red blood cells by certain antibodies. These antibodies may arise without cause or they may be associated with certain diseases such as lymphoma, chronic lymphocytic leukemia or Lupus (Systemic Lupus Erythematosus). (For more information on this disorders, choose "Warm Antibody Anemia" as your search term in the Rare Disease Database.) Therapies: Standard Prevention of cold antibody hemolytic anemia includes avoidance of exposure to cold. In severe cases, treatment with immunosuppressive drugs such as chlorambucil or cyclophosphamide is sometimes capable of reducing the cold agglutinin concentration. In cases where blood transfusions are necessary, certain guidelines must be followed. Cross-matching should be done at 37 degrees Centigrade to find compatible units of blood, and the blood should be warmed by an online warmer to prevent new red blood cells from being coated with antibodies. Therapies: Investigational Plasmapheresis may be of benefit in some cases of Cold Antibody Hemolytic Anemia. This procedure is a method for removing unwanted substances (toxins, metabolic substances and plasma parts) from the blood. Blood is removed from the patient and blood cells are separated from plasma. The patient's plasma is then replaced with other human plasma and the blood is retransfused into the patient. This therapy is still under investigation to analyze side effects and effectiveness. More research is needed before plasmapheresis can be recommended for use in all but the most severe cases of Cold Antibody Hemolytic Anemia. Studies are being conducted in the use of Sandoglobulin as a treatment for Cold Antibody Hemolytic Anemia. Further investigation is needed to determine it's safety and effectiveness. This disease entry is based upon medical information available through July 1990. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Cold Antibody Hemolytic Anemia, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Heart, Lung and Blood Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 1058-1059. THE MERCK MANUAL, 15th Ed.: Robert Berkow, M.D., ed.-in-chief;Merck Sharp & Dohme Laboratories, 1987. Pp. 1114-1117. BENEFIT OF A 37 DEGREE C EXTRACORPOREAL CIRCUIT IN PLASMA EXCHANGE THERAPY FOR SELECTED CASES WITH COLD AGGLUTININ DISEASE. C. Andrzejewski Jr. et al.; J CLIN APHERESIS (1988; 4(1):13-17). ISOLATION OF A PEPTIDE ASSOCIATED WITH AUTOIMMUNE HAEMOLYTIC ANAEMIA FROM RED CELL MEMBRANES. E. Kajii et al.; CLIN EXP IMMUNOL (Sep. 1988; 73(3):406- 409). PATIENTS WITH RED CELL AUTOANTIBODIES: SELECTION OF BLOOD FOR TRANSFUSION. R.J. Sokol et al.; CLIN LAB HAEMATOL (1988; 10(3):257-264.)