Castleman's Disease

Castleman's Disease

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.