Castleman Disease (CD)

Overview

Castleman disease (CD) is a rare disorder characterized by an abnormal overgrowth of cells in the lymph nodes. While it can affect any age group, it most commonly occurs in adults. CD encompasses a spectrum of disorders with varying etiologies, presentations, treatments, and outcomes. The two primary types are unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD).

Types of Castleman Disease (CD)

1. Unicentric Castleman Disease (UCD):

   • Affects a single enlarged lymph node or a localized region.
   • Often asymptomatic or mild symptoms due to compression of surrounding structures.
   • Surgical removal of the affected lymph node usually leads to a cure.

2. Multicentric Castleman Disease (MCD):

   • Involves multiple lymph node regions throughout the body.
   • Three subtypes:
     • POEMS-associated MCD: Accompanies POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes).
     • HHV-8-associated MCD: Linked to human herpesvirus-8 (HHV-8) infection, especially in immunocompromised individuals.
     • Idiopathic MCD (iMCD): Most common form, with unknown causes. Subtypes include iMCD-TAFRO and iMCD-NOS.

Causes of Castleman Disease (CD)

The exact cause of Castleman disease remains unknown.

Risk Factors of Castleman Disease (CD)

However, several factors may contribute:

• HHV8 viral infections
• Genetic mutations
• Increased interleukin levels
• HIV/AIDS (associated with MCD)

Symptoms of Castleman Disease (CD)

Preventions of Castleman Disease (CD)

While there is no specific prevention for Castleman disease, protecting against HIV infection can reduce the risk.

Diagnosis of Castleman Disease (CD)

• Physical examination: Assess lymph node size and consistency.
• Blood and urine tests: Rule out infections.
• Imaging: CT scan or MRI to visualize enlarged lymph nodes.
• Biopsy: Lymph node biopsy to differentiate from other lymphatic diseases.

Treatment for Castleman Disease (CD)

Treatment options depend on the type of Castleman disease:

• Unicentric Castleman Disease (UCD):
  • Surgical removal of the affected lymph node.
• Multicentric Castleman Disease (MCD):
  • Antiviral therapy (Ganciclovir, Foscarnet) for HHV-8-associated MCD.
  • Corticosteroids (Prednisone) to control inflammation.
  • Interleukin-6 antagonist (Siltuximab) for iMCD.
  • Anti-cancer drugs (Carmustine, Cladribine) for refractory cases.
  • Radiation therapy to destroy affected tissue.

When to seek medical attention ?

If you experience symptoms like enlarged lymph nodes, fever, night sweats, or unexplained weight loss, seek medical attention promptly.