Unicentric Castleman disease

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Unicentric Castleman disease (UCD) is a rare, non-cancerous disorder characterized by the overgrowth of cells in a single lymph node or a single group of lymph nodes. It is one of the two major types of Castleman disease, the other being Multicentric Castleman disease (MCD).

Presentation

Patients with Unicentric Castleman disease often present with an enlarged lymph node, which may be discovered incidentally during imaging studies or physical examination. Unlike Multicentric Castleman disease, UCD typically does not cause systemic symptoms such as fever, night sweats, or weight loss. However, the enlarged lymph node can cause local symptoms depending on its location, such as pain or pressure on nearby structures.

Pathophysiology

The exact cause of Unicentric Castleman disease is not well understood. It is believed to involve an abnormal immune response that leads to the overproduction of certain proteins, such as interleukin-6 (IL-6), which in turn causes the lymph node to enlarge. Unlike MCD, UCD is not associated with human herpesvirus 8 (HHV-8) or HIV infection.

Diagnosis

The diagnosis of Unicentric Castleman disease is typically made through a combination of imaging studies, such as CT scan or MRI, and a biopsy of the affected lymph node. Histological examination of the biopsy sample reveals characteristic features such as abnormal lymphoid follicles and increased vascularity.

Treatment

The primary treatment for Unicentric Castleman disease is surgical removal of the affected lymph node. Surgery is usually curative, and most patients do not require additional treatment. In cases where surgery is not feasible, other treatments such as radiation therapy or corticosteroids may be considered.

Prognosis

The prognosis for patients with Unicentric Castleman disease is generally excellent, especially when the affected lymph node is completely removed. Recurrence is rare, and most patients do not experience long-term complications.

Related Pages

• Castleman disease
• Multicentric Castleman disease
• Lymph node
• Interleukin-6
• CT scan
• MRI
• Radiation therapy
• Corticosteroids

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Unicentric Castleman disease Resources
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