You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.
NORD is very grateful to Corey Casper, MD, MPH, of Fred Hutchinson Cancer Research Center and the University of Washington, 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
- No subdivisions found.
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). Most often, they occur in the chest, abdomen, and/or neck, but may also be found in the armpit (axilla), pelvis, and pancreas. Usually the growths represent abnormal enlargement of the lymph nodes normally found in these areas.
Castleman's disease may be divided into four types. There are two main types of Castleman's disease that are determined by the microscopic appearance of the lymph node: 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). Additionally, Castleman's disease can be divided into categories which are defined by number of anatomic body regions affected by the disease. Unicentric Castleman's disease affects lymph nodes in only one part of the body, while multicentric Castleman's disease affects multiple parts of the body. A person can have any combination of the microscopic and anatomic variants, thus there are four possibilities: unicentric hyaline vascular, unicentric plasma cell variant, multicentric hyaline vascular variant, and multicentric plasma cell variant. Many individuals with multicentric Castleman's disease may exhibit an abnormally large liver and spleen (hepatosplenomegaly).
Castleman's Disease Resources
NORD Member Organizations:
(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors. If you're interested in becoming a member, please contact Susan Olivo, Membership Manager, at email@example.com.)
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
Copyright 1988, 1989, 1996, 2003, 2004, 2010
NORD's Rare Disease Information Database is copyrighted and may not be published without the written consent of NORD.