Castleman disease

Castleman disease is a group of rare lymph-node disorders involving abnormal growth of lymphoid tissue. It ranges from disease confined to one lymph-node region to systemic illness affecting multiple regions and organs.

Unicentric Castleman disease: Castleman disease confined to one lymph-node region, often presenting as a localized mass. This is the localized form, usually distinguished by a single affected nodal region.
Interleukin 6: A cytokine that regulates immune responses, inflammation, and blood-cell production. Excess IL-6 signaling contributes to inflammation and many systemic symptoms.
Lymph node biopsy: A procedure that removes lymph-node tissue for microscopic and laboratory examination. An excisional biopsy is central to confirming Castleman disease and assessing its tissue pattern.
Lymphoma: A cancer arising from lymphocytes or other cells of the lymphatic system. Lymphoma can resemble Castleman disease in enlarged nodes and requires different treatment.
Multicentric Castleman disease: Castleman disease involving multiple lymph-node regions and often producing systemic inflammation. This is the widespread form, with manifestations beyond one lymph-node region.
Human herpesvirus 8: A herpesvirus associated with Kaposi sarcoma and several lymphoproliferative disorders. Infected cells can drive HHV-8-associated multicentric Castleman disease.
Computed tomography: An imaging method that uses X-rays and computer processing to create cross-sectional images. Imaging maps involved lymph-node regions and helps distinguish localized from multicentric disease.
IgG4-related disease: A systemic immune-mediated disorder characterized by tissue infiltration with IgG4-positive plasma cells and fibrosis. It can cause lymph-node enlargement and overlapping tissue findings.
HHV-8-associated multicentric Castleman disease: A multicentric form of Castleman disease driven by human herpesvirus 8 infection. HHV-8 identifies a major virus-associated subtype of multicentric disease.
Viral interleukin 6: A viral cytokine encoded by human herpesvirus 8 that can mimic human interleukin 6. This viral signaling molecule helps explain inflammation in HHV-8-associated disease.

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TocilizumabRelated: Tocilizumab is used for certain forms, particularly where interleukin-6 drives symptoms.