CASTLEMAN'S DISEASE

Castleman's Disease (CD) is a rare and complex group of lymphoproliferative disorders that

affect the lymph nodes and related tissues in the body. It is also known as Castleman's

syndrome or angiofollicular lymph node hyperplasia. Castleman's Disease can manifest in

two main forms: unicentric Castleman's Disease (UCD) and multicentric Castleman's Disease

(MCD). These two forms have different clinical presentations, causes, and treatment

approaches.

Unicentric Castleman's Disease (UCD):

Definition: UCD is the more common form of Castleman's Disease, and it typically involves

a single lymph node or group of adjacent lymph nodes in one area of the body.

Causes: The exact cause of UCD is not well understood, but it appears to be related to the

overgrowth of lymphoid tissue in a localized region. It is not associated with the human

herpesvirus-8 (HHV-8) or HIV.

Signs and Symptoms:

 Enlarged lymph nodes, usually in the chest or abdomen.

 Most patients with UCD do not experience symptoms, but some may have discomfort

or pain related to the enlarged lymph nodes.

Treatment: The primary treatment for UCD is surgical removal of the affected lymph node

or lymph nodes. This can often lead to a complete cure, as long as the entire affected lymph

node is removed.

Multicentric Castleman's Disease (MCD):

Definition: MCD is a more aggressive and systemic form of Castleman's Disease that

involves multiple lymph nodes and affects various organs throughout the body.

Causes: MCD is often associated with infection by the human herpesvirus-8 (HHV-8) or can

be seen in the context of HIV infection. It is considered an immune system disorder.

Signs and Symptoms:

 Enlarged lymph nodes in multiple areas of the body.

 Fever, night sweats, and weight loss.

 Enlarged liver and spleen.

 Anemia and low platelet counts.

 Symptoms related to organ dysfunction, such as difficulty breathing due to lung

involvement or neurological symptoms.

Treatment: The treatment of MCD is more challenging than UCD and may involve a

combination of therapies, including:

 Antiviral medications in cases associated with HHV-8.

 Immunotherapy, including monoclonal antibodies like rituximab.

 Chemotherapy to suppress the overactive immune response.

 Anti-inflammatory medications like corticosteroids.

Nursing Care: Nursing care for Castleman's Disease patients includes:

1. Assessment of symptoms and vital signs.

2. Monitoring for complications related to organ involvement.

3. Administering prescribed medications and managing side effects.

4. Providing emotional support and education to patients and their families.

 5. Collaborating with the healthcare team to ensure comprehensive care.

Complications: Complications of Castleman's Disease can include severe organ dysfunction,

immune system dysfunction, and an increased risk of developing other lymphoproliferative

disorders or malignancies. In severe cases of MCD, it can be life-threatening.

The management and prognosis of Castleman's Disease depend on the type (UCD or MCD),

associated conditions, and the patient's overall health. Patients with UCD typically have a

better prognosis, while MCD is more challenging to treat and can be more aggressive. Long-

term follow-up and monitoring are essential for all patients with Castleman's Disease.