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Castleman's Disease

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Copyright 1988, 1989, 1996, 2003, 2004

NORD is grateful to Corey Casper, MD, MPH, of the University of Washington Virology Research Clinic, Fred Hutchinson Cancer Research Center, for assistance in the preparation of this report.

Synonyms of Castleman's Disease
  • Angiofollicular Lymph Node Hyperplasia
  • Angiomatous Lymphoid
  • Castleman Tumor
  • Giant Benign Lymphoma
  • Giant Lymph Node Hyperplasia
  • Hamartoma of the Lymphatics

Disorder Subdivisions



General Discussion
Castleman's disease is a rare disorder characterized by non-cancerous (benign) growths (tumors) that may develop in the lymph node tissue throughout the body (i.e., systemic disease [plasma cell type]). Most often, they occur in the chest, stomach, and/or neck (i.e., localized disease [hyaline-vascular type]). Less common sites include the armpit (axilla), pelvis, and pancreas. Usually the growths represent abnormal enlargement of the lymph nodes normally found in these areas (lymphoid hamartoma). There are two main types of Castleman's disease: hyaline-vascular type and plasma cell type. The hyaline vascular type accounts for approximately 90 percent of the cases. Most individuals exhibit no symptoms of this form of the disorder (asymptomatic) or they may develop non-cancerous growths in the lymph nodes. The plasma cell type of Castleman's disease may be associated with fever, weight loss, skin rash, early destruction of red blood cells, leading to unusually low levels of circulating red blood cells (hemolytic anemia), and/or abnormally increased amounts of certain immune factors in the blood (hypergammaglobulinemia).

A third type of Castleman's disease has been reported in the medical literature. This type may affect more than one area of the body (multicentric or generalized Castleman's disease). Many individuals with Multicentric Castleman's disease may exhibit an abnormally large liver and spleen (hepatosplenomegaly). Researchers' opinions in the medical literature differ as to whether Multicentric Castleman's disease is a distinct entity or a multicentric form of the plasma cell type of Castleman's disease.
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Organizations related to Castleman's Disease
  • American Autoimmune Related Diseases Association, Inc.
    22100 Gratiot Avenue
    Eastpointe MI 48021
    Phone #: 586-776-3900
    800 #: 800-598-4668
    e-mail: aarda@aarda.org
    Home page: http://www.aarda.org/
  • American Cancer Society, Inc.
    1599 Clifton Road NE
    Atlanta GA 30329
    Phone #: 404-320-3333
    800 #: 800-227-2345
    e-mail: N/A
    Home page: http://www.cancer.org
  • AutoImmunity Community
    None
    None None None
    Phone #: (91-9) -552-9057
    800 #: N/A
    e-mail: bandrews@autoimmunitycommunity.org
    Home page: http://autoimmunitycommunity.org
  • Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg MD 20898-8126
    Phone #: 301-251-4925
    800 #: 888-205-2311
    e-mail: ordr@od.nih.gov
    Home page: http://rarediseases.info.nih.gov/Default.aspx
  • International Castleman's Disease Organization
    4 Brazos Court
    Santa Fe NM 87508
    Phone #: 505-424-7947
    800 #: N/A
    e-mail: HMConsult@msn.com
    Home page: http://www.castlemans.org
  • Madisons Foundation
    PO Box 241956
    Los Angeles CA 90024
    Phone #: 310-264-0826
    800 #: N/A
    e-mail: getinfo@madisonsfoundation.org
    Home page: http://www.madisonsfoundation.org
  • NIH/National Heart, Lung and Blood Institute Information Center
    P.O. Box 30105
    Bethesda MD 20824-0105
    Phone #: 301-592-8573
    800 #: --
    e-mail: nhlbiinfo@rover.nhlbi.nih.gov
    Home page: N/A
  • Rare Cancer Alliance
    1649 North Pacana Way
    Green Valley AZ 85614
    Phone #: 520-625-5495
    800 #: --
    e-mail: sharon.lane@rare-cancer.org
    Home page: http://www.rare-cancer.org
  • UCSF Hemophilia Treatment Center
    400 Parnassus Ave.
    First Floor
    San Francisco CA 94143
    Phone #: 415-353-2986
    800 #: N/A
    e-mail: N/A
    Home page: http://www.ucsfhealth.org/adult/medical_services/blood/hemophilia/index.html

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Last modified Wednesday, November 26, 2008