Biomaterial Database

Castleman's disease: a study of a rare lymphoproliferative disorder in a university hospital. 2010

A M Al-Amri, and I S Abdul-Rahman, and K Q Ghallab

King Fahd University Hospital, Al-Khobar, Saudi Arabia.

Castleman's disease (CD) is a group of rare lymphoproliferative disorders sharing characteristic clinical and histological features, and usually accompanied by a marked systemic inflammatory response. Two histological patterns of lymph nodes were described: the hyaline-vascular and plasma-cell types. The former is more common (80-90%) and tends to be localized. The plasma cell type is more aggressive and usually multicentric. It is interesting that the inflammatory manifestations seem to be related to a lymph node lesion, because the systemic symptoms and inflammatory activity can return to normal after surgical excision or successful medical treatment of the disease. We report here our 15-year experience with this rare disease in King Fahd Hospital of the University, Al-Khobar, Saudi Arabia, focusing on the clinical features, therapy, and patients'outcome.

UI	MeSH Term	Description	Entries
D008198	Lymph Nodes	They are oval or bean shaped bodies (1 - 30 mm in diameter) located along the lymphatic system.	Lymph Node,Node, Lymph,Nodes, Lymph
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D010950	Plasma Cells	Specialized forms of antibody-producing B-LYMPHOCYTES. They synthesize and secrete immunoglobulin. They are found only in lymphoid organs and at sites of immune responses and normally do not circulate in the blood or lymph. (Rosen et al., Dictionary of Immunology, 1989, p169 & Abbas et al., Cellular and Molecular Immunology, 2d ed, p20)	Plasmacytes,Cell, Plasma,Cells, Plasma,Plasma Cell,Plasmacyte
D005260	Female		Females
D005871	Castleman Disease	Large benign, hyperplastic lymph nodes. The more common hyaline vascular subtype is characterized by small hyaline vascular follicles and interfollicular capillary proliferations. Plasma cells are often present and represent another subtype with the plasma cells containing IgM and IMMUNOGLOBULIN A.	Angiofollicular Lymphoid Hyperplasia,Castleman's Tumor,Giant Lymph Node Hyperplasia,Hyperplasia, Giant Lymph Node,Lymph Node Hyperplasia, Giant,Angiofollicular Lymph Hyperplasia,Angiofollicular Lymph Node Hyperplasia,Castleman's Disease,Castlemans Disease,Angiofollicular Lymph Hyperplasias,Angiofollicular Lymphoid Hyperplasias,Castleman Tumor,Castlemans Tumor,Disease, Castlemans,Hyperplasia, Angiofollicular Lymph,Hyperplasia, Angiofollicular Lymphoid,Lymph Hyperplasia, Angiofollicular,Lymphoid Hyperplasia, Angiofollicular,Tumor, Castleman's
D006785	Hospitals, University	Hospitals maintained by a university for the teaching of medical students, postgraduate training programs, and clinical research.	University Hospitals
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006818	Hyalin	A clear, homogenous, structureless, eosinophilic substance occurring in pathological degeneration of tissues.	Hyalin Substance,Hyaline,Hyaline Substance,Hyalins,Substance, Hyalin,Substance, Hyaline
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths