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Castleman’s Disease: A Study of A Rare Lymphoproliferative Disorder In A University Hospital

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Author(s): Ibrahiem Saeed Abdul-Rahman | Ali M. Al-Amri | Khalid Qassim Ghallab

Journal: Clinical Medicine : Blood Disorders
ISSN 1178-2269

Volume: 2;
Start page: 5;
Date: 2009;
Original page

Keywords: Castleman’s disease | hyaline vascular | plasma cell | multicentric | polyclonal gammopathy | IL-6 | Kaposi’s sarcoma | HHV-8

ABSTRACT
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. Three histological patterns of lymph nodes were described: the hyaline-vascular, the plasma-cell and the mixed 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.
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