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$Unique_ID{BRK03460}
$Pretitle{}
$Title{Anemia, Hemolytic, Acquired Autoimmune}
$Subject{Anemia Hemolytic Acquired Autoimmune Immune Hemolytic Anemia Warm
Antibody Hemolytic Anemia Cold Antibody Hemolytic Anemia Paroxysmal Cold
Hemoglobinuria Paroxysmal Nocturnal Hemolytic Anemia Chronic Lymphatic
Leukemia Lymphoma Lupus Erythematosus}
$Volume{}
$Log{}
Copyright (C) 1990 National Organization for Rare Disorders, Inc.
771:
Anemia, Hemolytic, Acquired Autoimmune
** IMPORTANT **
It is possible that the main title of the article (Acquired Autoimmune
Hemolytic Anemia) is not the name you expected. Please check the SYNONYM
listing to find the alternate names and disorder subdivisions covered by this
article.
Synonyms
Immune Hemolytic Anemia
Disorder Subdivisions:
Warm Antibody Hemolytic Anemia
Cold Antibody Hemolytic Anemia
Paroxysmal Cold Hemoglobinuria
Information on the following diseases can be found in the Related
Disorders section of this report:
Paroxysmal Nocturnal Hemolytic Anemia
Chronic Lymphatic Leukemia
Lymphoma
Lupus Erythematosus
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.
Acquired Autoimmune Hemolytic Anemia is an autoimmune disorder
characterized by the premature destruction of red blood cells. Autoimmune
diseases occur when the body's natural defenses against invading organisms
(e.g., lymphocytes, antibodies) destroy healthy tissue for unknown reasons.
Normally, the red blood cells have a life span of approximately 120 days
before they are removed by the spleen. In an individual affected with
Acquired Autoimmune Hemolytic Anemia, the red blood cells are destroyed
prematurely and bone marrow production of new cells can no longer compensate
for their loss. The severity of this type of anemia is determined by the
time the red blood cell is allowed to survive in an affected person, and by
the capacity of the bone marrow to continue red cell production.
Acquired Autoimmune Hemolytic Anemia is a disorder that occurs in
individuals who previously had a normal red blood cell system. The disorder
commonly occurs as the result of, or in conjunction with some other medical
condition. It less commonly occurs alone without a precipitating factor.
Symptoms
General symptoms of Acquired Autoimmune Hemolytic Anemia may include fatigue,
chills, backache, difficulty in breathing upon exertion and heart
palpitations.
There are several forms of Acquired Autoimmune Hemolytic Anemia including
Warm Antibody Hemolytic Anemia, Cold Antibody Hemolytic Anemia and Paroxysmal
Cold Hemoglobinuria.
Warm Antibody Hemolytic Anemia is 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. Warm Antibody Hemolytic Anemia usually has a
sudden onset, and symptoms may include severe anemia, pallor, fatigue,
difficulty in breathing, dizziness and heart palpitations. When the onset is
gradual, symptoms are usually less severe. Mild jaundice and an enlarged
spleen are usually present. (For more information on this disorder, choose
"Warm Antibody Hemolytic Anemia" as your search term in the Rare Disease
Database.)
Cold Antibody Hemolytic Anemia is characterized by the destruction of red
blood cells by certain antibodies particularly at lower than normal
temperatures. Symptoms of Cold Antibody Hemolytic Anemia may include
weakness, dizziness, headache, ringing in the ears (tinnitus), spots before
the eyes, fatigue, drowsiness, and irritability. Absent menstruation
(amenorrhea), gastrointestinal complaints, yellowing of the skin (jaundice)
and enlargement of the spleen may also occur. Heart failure or shock may
result. (For more information on this disorder, choose "Cold Antibody
Hemolytic Anemia" as your search term in the Rare Disease Database.)
Paroxysmal Cold Hemoglobinuria (PCH) is characterized by a disintegration
of red blood cells occurring minutes to hours after exposure to cold. It is
frequently associated with viral infections such as chickenpox and mumps.
Symptoms of Paroxysmal Cold Hemoglobinuria may include severe pain in the
back and legs, headache, vomiting, diarrhea and passage of blood in the urine
(hemoglobinuria). There may also be a temporary enlargement of the liver.
(For more information on this disorder, choose "Paroxysmal Cold
Hemoglobinuria" as your search term in the Rare Disease Database.)
Causes
Most cases of Acquired Autoimmune Hemolytic Anemia are the result of some
associated disorder. Injury to the immune system by such disorders as Chronic
Lymphatic Leukemia, Lymphoma, Lupus Erythematosus or viral infections such as
Chickenpox or Mumps may be responsible. Other cases of Acquired Autoimmune
Hemolytic Anemia have no known cause. Scientists do not understand why
lymphocytes and antibodies in people with autoimmune disorders attack the
body's own healthy blood cells, apparently mistakenly identifying them as
foreign.
Other types of hemolytic anemia may be caused by certain medications such
as sulfonamides, phenothiazines, quinine, quinidine and methyldopa.
Affected Population
When Acquired Autoimmune Hemolytic Anemia occurs from unknown causes, it
affects twice as many women as men, specifically women under 50 years old.
Cold Antibody Hemolytic Anemia most commonly affects elderly persons, and
Warm Antibody Hemolytic Anemia can affect anyone at any age.
Related Disorders
Symptoms of the following disorder can be similar to those of Acquired
Autoimmune Hemolytic Anemia. Comparison may be useful for a differential
diagnosis:
Paroxysmal Nocturnal Hemoglobinuria is a disorder characterized by a
decrease of red blood cells (anemia) caused by a defect in the membrane of
the red blood cells. The major symptom is the presence of blood in the urine
(hemoglobinuria) which occurs chiefly at night due to the breakdown of red
blood cells. Other symptoms may include paleness, severe abdominal or back
pain, yellowing of the skin (jaundice) and enlargement of the spleen and
liver. (For more information on this disorder, choose "Paroxysmal Nocturnal
Hemoglobinuria" as your search term in the Rare Disease Database.)
The following disorders may precede the development of Acquired
Autoimmune Hemolytic Anemia. They can be useful in identifying an underlying
cause of some forms of this disorder:
Chronic Lymphatic Leukemia is a disorder characterized by an excessive
amount of white blood cells in the bone marrow, spleen, liver and blood. As
the disease progresses, the leukemic cells invade other areas of the body
including the intestinal tract, kidneys, lungs, gonads and lymph nodes.
Symptoms of Chronic Lymphatic Leukemia may include fatigue, weakness,
itchiness, night sweats, abdominal discomfort or weight loss. An enlarged
spleen is usually discovered upon physical examination. People with this
type of leukemia can get Acquired Autoimmune Hemolytic Anemia. (For more
information on this disorder, choose "Chronic Myelogenous Leukemia" as your
search term in the Rare Disease Database.)
Lymphoma is a malignant growth which most commonly occurs in the lymph
nodes, spleen and other areas involved in the body's immune system
(lymphoreticular system). The major types are Hodgkin's Disease and Non-
Hodgkin's Lymphoma. People with Lymphoma can get Acquired Autoimmune
Hemolytic Anemia. (For more information on this disorder, choose "Hodgkin"
or "Lymphoma" as your search term in the Rare Disease Database.)
Lupus Erythematosus is an inflammatory connective tissue disease that can
affect many parts of the body including the joints, skin and internal organs.
Lupus is an autoimmune disease most often striking young women between the
ages of 15 and 35 years. The symptom for which Lupus was originally named, a
butterfly-shaped red rash across the bridge of the nose and cheeks, is found
in only five percent of newly diagnosed patients. Far more common are
arthritic symptoms such as painfully inflamed joints. People with Lupus can
get Acquired Autoimmune Hemolytic Anemia. (For more information on this
disorder, choose "Lupus" as your search term in the Rare Disease Database).
Therapies: Standard
When Acquired Autoimmune Hemolytic Anemia is caused by other diseases,
diagnosis and treatment of the underlying disorder usually brings marked
improvement of the anemia. Mild cases may require no treatment. Individuals
with more severe cases of Warm Antibody Hemolytic Anemia may be treated with
oral steroids or intravenous hydrocortisone followed by divided daily oral
doses of prednisone. Improvement usually occurs within five to ten days
after treatment. If the response to the steroid therapy is unsatisfactory,
other therapeutic approaches must be considered. In some resistant cases,
total removal of the spleen may be required. Immunosuppressive drugs such as
oral azathioprine or cyclophosphamide may be administered. Blood
transfusions may be required in severe cases.
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. (For more information on this disorder, choose
"Cold Antibody Hemolytic Anemia" as your search term in the Rare Disease
Database.)
Paroxysmal Cold Hemoglobinuria is usually corrected when the accompanying
viral infection is treated, requiring only supportive therapy and protection
of the affected individual from cold. If the disorder is chronic, it may
respond to treatment with glucocorticoids or immunosuppressive drugs such as
cyclophosphamide.
Other types of hemolytic anemia caused by certain medications usually
subside after discontinuance of the medication. No additional therapy is
usually required.
Therapies: Investigational
Studies are being conducted in the use of Sandoglobulin as a treatment
for Acquired Autoimmune 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 Acquired Autoimmune Hemolytic Anemia, 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
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 918-923.
THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief;
Merck Sharp & Dohme Laboratories, 1982. Pp. 1114-1115.
RAPID TRANSIENT REVERSAL OF ANEMIA AND LONG-TERM EFFECTS OF MAINTENANCE
INTRAVENOUS IMMUNOGLOBULIN FOR AUTOIMMUNE HEMOLYTIC ANEMIA IN PATIENTS WITH
LYMPHOPROLIFERATIVE DISORDERS. E.C. Besa; AM J MED (April, 1988: issue 84
(4)). Pp. 691-698.
ISOLATION OF PEPTIDE ASSOCIATED WITH AUTOIMMUNE HAEMOLYTIC ANEMIA FROM RED
CELL MEMBRANES. E. Kajii et al.; CLIN EXP IMMUNOL (September, 1988: issue 73
(3)). Pp. 406-409.
ELUCIDATION OF ALLOANTIBODIES IN AUTOIMMUNE HAEMOLYTIC ANEMIA. P. James
et al; VOX SANG (1988; issue 54 (3)). Pp. 167-171.