Castleman Disease

Castleman disease, also known as angiofollicular lymph node hyperplasia, is a rare disorder characterized by the overgrowth of cells in the body's lymph nodes.

Types of Castleman Disease

There are two main types of Castleman disease: unicentric and multicentric. Unicentric Castleman disease typically affects only one group of lymph nodes, usually in the chest or abdomen, while multicentric Castleman disease affects multiple groups of lymph nodes throughout the body.

Unicentric Castleman Disease

Unicentric Castleman disease is often benign and may not cause any symptoms. It is typically diagnosed incidentally during imaging tests for other conditions. Treatment usually involves surgical removal of the affected lymph node.

Multicentric Castleman Disease

Multicentric Castleman disease, on the other hand, can be more aggressive and may cause a range of symptoms, including fever, weight loss, fatigue, and swelling in the lymph nodes. It is often associated with underlying conditions such as human herpesvirus 8 (HHV-8) infection or autoimmune disorders.

Symptoms

The symptoms of Castleman disease can vary depending on the type and severity of the condition. Common symptoms include:

• Fever
• Weight loss
• Fatigue
• Swelling in the lymph nodes, particularly in the neck, armpits, or groin
• Night sweats
• Loss of appetite

Causes and Risk Factors

The exact cause of Castleman disease is not fully understood. However, it is believed to be related to an overactive immune response, which can be triggered by underlying conditions such as:

• Human herpesvirus 8 (HHV-8) infection
• Autoimmune disorders, such as rheumatoid arthritis or lupus
• Cancer, particularly lymphoma
• Infections, such as HIV or tuberculosis

Diagnosis

Castleman disease is typically diagnosed through a combination of imaging tests, blood tests, and biopsy. Imaging tests may include:

• Computed tomography (CT) scans
• Magnetic resonance imaging (MRI) scans
• Positron emission tomography (PET) scans

Blood tests may be used to check for underlying conditions, such as HHV-8 infection or autoimmune disorders. A biopsy involves removing a sample of tissue from the affected lymph node and examining it under a microscope.

Treatment

Treatment for Castleman disease depends on the type and severity of the condition. Unicentric Castleman disease is often treated with surgical removal of the affected lymph node, while multicentric Castleman disease may require more aggressive treatment, including:

• Chemotherapy
• Radiation therapy
• Immunotherapy
• Corticosteroids to reduce inflammation

Prognosis

The prognosis for Castleman disease varies depending on the type and severity of the condition. Unicentric Castleman disease typically has a good prognosis, with most people experiencing complete recovery after surgical removal of the affected lymph node. Multicentric Castleman disease can be more challenging to treat and may require ongoing management to control symptoms and prevent complications.

Frequently Asked Questions (FAQs)

What is Castleman disease?
Castleman disease is a rare disorder characterized by overgrowth of cells in the body's lymph nodes.

What are the types of Castleman disease?
There are two main types: unicentric and multicentric Castleman disease.

What causes Castleman disease?
The exact cause is unknown, but it may be related to immune system dysfunction and possibly viral infections.

What are the symptoms of unicentric Castleman disease?
Symptoms may include a lump or mass, fever, weight loss, and fatigue.

How is Castleman disease diagnosed?
Diagnosis typically involves imaging tests, blood tests, and biopsy of affected lymph nodes.

What are the treatment options for unicentric Castleman disease?
Treatment usually involves surgical removal of the affected lymph node or nodes.

Can multicentric Castleman disease be treated?
Treatment for multicentric Castleman disease may involve medications to manage symptoms and slow disease progression.

Is Castleman disease contagious?
There is no evidence to suggest that Castleman disease is contagious.

What is the prognosis for Castleman disease?
The prognosis varies depending on the type of Castleman disease and the effectiveness of treatment, with some cases having a relatively good outcome.

Are there any ongoing research efforts for Castleman disease?
Yes, researchers are studying Castleman disease to better understand its causes and develop more effective treatments.