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Castleman’s disease is a very rare disorder characterized by non-cancerous growths (tumors) that may develop in the lymph node tissue at a single site or throughout the body.[1] It involves hyperproliferation of certain B cells that often produce cytokines.
It is named for Benjamin Castleman.[2][3]
There are several variants of Castleman’s disease. In all cases, Castleman’s disease is likely due to hypersecretion of the cytokine IL-6. [4]
Unicentric Castleman’s disease involves tissue growths at only a single site. It usually has few or no symptoms other than those directly associated with the physical enlargement of the lymph node. In 90% or more, removal of the enlarged node is curative, with no further complications.
Multicentric Castleman’s disease (MCD) involves growths at multiple sites.[6] About 50% is caused by KSHV, a gammaherpesvirus that is also the cause of Kaposi’s sarcoma and primary effusion lymphoma, while the remainder of MCD are of unknown cause. The form of MCD most closely associated with KSHV is the plasmacytic form of Castleman’s disease while another pathologic form, the hyaline-vascular form, is generally negative for this virus.
The most common ‘B Symptoms’ of MCD are high fevers, anemia, weight loss, loss of appetite, and low white blood cell counts, which may to be due to the overproduction of interleukin 6. Symptomatically, therefore, MCD can be difficult to diagnose and even in the case of a lymph-node biopsy a conclusive diagnosis remains problematic.
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