$Unique_ID{BRK03807} $Pretitle{} $Title{Hemoglobinuria, Paroxysmal Cold} $Subject{Hemoglobinuria Paroxysmal Cold Donath-Landsteiner Hemolytic Anemia Donath-Landsteiner Syndrome Immune Hemolytic Anemia Dressler Syndrome Harley Syndrome PCH Paroxysmal Nocturnal Hemoglobinuria Cold Antibody Hemolytic Anemia} $Volume{} $Log{} Copyright (C) 1990 National Organization for Rare Disorders, Inc. 776: Hemoglobinuria, Paroxysmal Cold ** IMPORTANT ** It is possible that the main title of the article (Paroxysmal Cold Hemoglobinuria) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Donath-Landsteiner Hemolytic Anemia Donath-Landsteiner Syndrome Immune Hemolytic Anemia Dressler Syndrome Harley Syndrome PCH Information on the following diseases can be found in the Related Disorders section of this report: Paroxysmal Nocturnal Hemoglobinuria Cold Antibody Hemolytic Anemia 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. Paroxysmal Cold Hemoglobinuria is a very rare autoimmune hemolytic disorder characterized by the premature destruction of healthy red blood cells minutes to hours after exposure to cold. Autoimmune diseases occur when the body's natural defenses against invading organisms (e.g., lymphocytes, antibodies) destroy healthy tissue for unknown reasons. Normally, red blood cells have a life span of approximately 120 days before they are removed by the spleen. In an individual affected with Paroxysmal Cold Hemoglobinuria, the red blood cells are destroyed prematurely and suddenly (paroxysmally) upon exposure to temperatures of 10 to 15 degrees Centigrade and below. Symptoms Attacks of Paroxysmal Cold Hemoglobinuria can occur within minutes or up to eight hours after exposure to cold. Symptoms may include fever, malaise, anorexia, flank pain, pain in the back and legs, headache, vomiting, diarrhea, mild anemia, and the passage of dark brown urine (hemoglobinuria). This urine contains the iron-containing protein pigment of blood called hemoglobin. The presence of hemoglobin causes the dark brown color of the urine. Attacks of Paroxysmal Cold Hemoglobinuria may be followed by yellowing of the skin (jaundice). There may also be enlargement of the liver and spleen. Causes Most cases of Paroxysmal Cold Hemoglobinuria occur after a viral infection such as chickenpox or mumps, or in conjunction with congenital or acquired syphilis. Paroxysmal Cold Hemoglobinuria may also affect individuals with no history of another disorder. In these cases the cause is unknown. Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc.), against invading organisms suddenly begin to attack healthy tissue. In individuals affected with Paroxysmal Cold Hemoglobinuria, these attacks occur when red blood cells are attacked as if they were a virus or bacteria. Localized exposure to cold such as washing one's hand in cold water or drinking cold water may also trigger an attack in some severe cases. Affected Population Anyone may acquire Paroxysmal Cold Hemoglobinuria. An individual with a viral infection such as chickenpox or mumps, or anyone affected with syphilis, is of higher risk of contracting the disorder. Related Disorders Symptoms of the following disorders can be similar to those of Paroxysmal Cold Hemoglobinuria. Comparisons may be useful for a differential diagnosis: Paroxysmal Nocturnal Hemoglobinuria is a form of anemia resulting from defects in the membranes of red blood cells. It is characterized by the presence of hemoglobin in the urine (hemoglobinuria) chiefly occurring at night. Symptoms of Paroxysmal Nocturnal Hemoglobinuria include severe abdominal or back pain occurring during the phase where the oxygen carrying portion of the red blood cell (hemoglobin) is released from the red blood cells (hemolysis). Other symptoms may include blood in the urine, yellowing of the skin (jaundice) and enlargement of the liver and spleen. (For more information on this disorder, choose "Paroxysmal Nocturnal Hemoglobinuria" as your search term in the Rare Disease Database). Cold Antibody Hemolytic Anemia is a rare autoimmune disorder in which red blood cells are attacked and destroyed by the body's natural defenses in temperatures of 15 degrees Centigrade or below. Symptoms of Cold Antibody Hemolytic Anemia may include weakness, dizziness, headache, ringing in the ears (tinnitus), spots before the eyes, fatigue, drowsiness, irritability or bizarre behavior. Absent menstruation, gastrointestinal complaints, yellowing of the skin, and enlargement of the spleen may also occur. Heart failure or shock may result. More rarely, there may be a passing of dark urine (hemoglobinuria). (For more information on this disorder, choose "Cold Antibody Hemolytic Anemia" as your search term in the Rare Disease Database). Therapies: Standard Paroxysmal Cold Hemoglobinuria is usually corrected when the accompanying viral infection is treated, requiring only supportive therapy, bed rest and protection of the affected individual from cold temperatures. If the disorder is chronic, it may respond to treatment with glucocorticoids or immunosuppressive drugs such as cyclophosphamide. In cases where blood transfusions are necessary, certain guidelines must be followed. Crossmatching 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 and destroyed. Therapies: Investigational This disease entry is based upon medical information available through April 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 Paroxysmal Cold Hemoglobinuria, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Heart, Lung and Blood Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, M.D. et al., eds; W.B. Saunders Company, 1988. Pp. 921. INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 1059. THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief; Merck Sharp & Dohme Laboratories, 1982. Pp. 1115. DONATH-LANDSTEINER HEMOLYTIC ANEMIA DUE TO AN ANTI-Pr-LIKE BIPHASIC HEMOLYSIN. W.J. Judd et al.; TRANSFUSION (September-October, 1986: issue 26 (5)). Pp. 423-425. AN UNUSUAL DONATH-LANDSTEINER ANTIBODY DETECTABLE AT 37 DEGREES C BY THE ANTIGLOBULIN TEST. S. Lindgren et al.; (March-April, 1985: issue 25 (2)). Pp. 142-144.