$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.