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- $Unique_ID{BRK03949}
- $Pretitle{}
- $Title{Lymphadenopathy, Angioimmunoblastic with Dysproteinemia}
- $Subject{Lymphadenopathy, Angioimmunoblastic with Dysproteinemia AILD
- Immunoblastic Lymphadenopathy Chronic Lymphadenopathy Syndrome Acquired Immune
- Deficiency Syndrome (AIDS) }
- $Volume{}
- $Log{}
-
- Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
-
- 439:
- Lymphadenopathy, Angioimmunoblastic with Dysproteinemia
-
- ** IMPORTANT **
- It is possible the main title of the article (Angioimmunoblastic
- Lymphadenopathy with Dysproteinemia) is not the name you expected. Please
- check the SYNONYMS listing on the next page to find alternate names, disorder
- subdivisions, and related disorders covered by this article.
-
- Synonyms
-
- AILD
- Immunoblastic Lymphadenopathy
-
- Information on the following diseases can be found in the Related
- Disorders section of this report:
-
- Chronic Lymphadenopathy Syndrome
- Acquired Immune Deficiency Syndrome (AIDS)
-
- 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.
-
- Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD) is a
- progressive immune system disorder possibly caused by viral infections,
- chronic stimulation of immune responses or drug treatments prescribed for
- other conditions. This disorder occurs mostly among persons over fifty years
- of age. Fever, chills, sweating, a general feeling of discomfort, weight
- loss, and/or skin rashes are the major symptoms. In some cases, AILD may
- evolve into a severe form of lymphoma (a type of cancer).
-
- Symptoms
-
- Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD) is
- characterized by acute onset with fever, chills, sweating, general
- discomfort, weight loss, and/or skin rashes. Enlargement of the liver,
- spleen and lymph nodes, anemia and the presence of excessive immune system
- defense antibodies (gammaglobulins) in the blood may occur. Infections are
- common. This disorder may evolve into a severe form of lymphoma (cancer of
- the lymph tissue) in some cases.
-
- Causes
-
- Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD) is thought to
- be caused by an over-reaction of the immune system. It may be an early stage
- in malignant lymphoproliferative disorders, which are cancers of the lymph
- tissues. Frequently, there is a history of recent drug exposure, insect
- bite, immunization, viral infections, or other potential immune system
- stimulations.
-
- Affected Population
-
- Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD) usually affects
- persons over fifty years of age. Males and females are affected in equal
- numbers.
-
- Related Disorders
-
- Symptoms of the following disorders can be similar to those of
- Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD). Comparisons
- may be useful for a differential diagnosis:
-
- Dermatopathic Lymphadenopathy, also known as Lipomelanic Reticulosis, is
- characterized by lymph node enlargement with over-production of cells known
- as histiocytes and macrophages containing fat and the black pigment known as
- melanin. This disorder may be caused by other skin conditions, particularly
- those characterized by itching (pruritus) or scaly shedding (exfoliation).
-
- Acquired Immune Deficiency Syndrome (AIDS) is characterized by
- progressive deterioration of the body's ability to ward off infection.
- Organisms which in a healthy person would either fail to cause disease, cause
- mild disease, or at least provoke immunity, completely overwhelm the AIDS
- patient. Patients with severe AIDS also contract various uncommon, life-
- threatening infections, particularly pneumocystis carinii pneumonia, and have
- an unusually high incidence of a rare cancer, Kaposi's Sarcoma. Individuals
- in the early stages of the disease are unusually susceptible to many milder
- infections. (For more information on this disorder, choose "AIDS" as your
- search term in the Rare Disease Database and see the AIDS Update section of
- NORD Services).
-
- Therapies: Standard
-
- Treatment of Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD)
- involves cyclophosphamides or corticosteroid drugs. These may reduce
- abnormally large lymph nodes and improve blood cell abnormalities. Infection
- must be carefully guarded against. Other treatment is symptomatic and
- supportive.
-
- Therapies: Investigational
-
- This disease entry is based upon medical information available through June
- 1988. 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 Angioimmunoblastic Lymphadenopathy with
- Dysproteinemia, please contact:
-
- National Organization for Rare Disorders (NORD)
- P.O. Box 8923
- New Fairfield, CT 06812-1783
- (203) 746-6518
-
- Immune Deficiency Foundation
- 3565 Ellicott Mill Drive, Unit B2
- Ellicott City, MD 21043
- (800) 296-4433
- (410) 461-3127
-
- NIH/National Cancer Institute
- 9000 Rockville Pike, Bldg. 31, Rm. 1A2A
- Bethesda, MD 20892
- 1-800-4-CANCER
-
- The National Cancer Institute has developed PDQ (Physician Data Query), a
- computerized database designed to give doctors quick and easy access to many
- types of information vital to treating patients with many types of cancer.
- To gain access to this service, a doctor can contact the Cancer Information
- Service offices at 1-800-4-CANCER. Information specialists at this toll-free
- number can answer questions about cancer prevention, diagnosis, and
- treatment.
-
- NIH/National Institute of Allergy and Infections Diseases (NIAID)
- 9000 Rockville Pike
- Bethesda, MD 20892
- (301) 496-5717
-
- References
-
- EFFECT OF CYCLOPHOSPHAMIDE THERAPY ON ONCOGENE EXPRESSION IN
- ANGIOIMMUNOBLASTIC LYMPHADENOPATHY: D.M. Klinman, et al.; Lancet (November
- 8, 1986, issue 2(8515)). Pp. 1055-1058.
-
- ANGIOIMMUNOBLASTIC LYMPHADENOPATHY: W.P. Su; Dermatol Clin (October
- 1985, issue 3(4)). Pp. 759-768.
-
- MODULATION OF C-MYB TRANSCRIPTION IN AUTOIMMUNE DISEASE BY
- CYCLOPHOSPHAMIDE: J.D. Mountz, et al.; J Immunol (October 1985, issue
- 135(4)). Pp. 2417-2422.
-
-