NORD gratefully acknowledges Corey Casper, MD, MPH, of Fred Hutchinson Cancer Research Center and the University of Washington, for assistance in the preparation of this report.
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).
1988, 1989, 1996, 2003, 2004, 2010