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$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.)