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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.
-
-