Angiofollicular lymph hyperplasia

Angiofollicular Lymph Hyperplasia Angiofollicular lymph hyperplasia, also known as Castleman disease, is a rare disorder characterized by non-cancerous growths that may develop in the lymph node tissue throughout the body. This condition can affect individuals of any age, but it is most commonly diagnosed in adults.

Classification

Angiofollicular lymph hyperplasia is classified into two main types based on the number of lymph nodes involved:

• Unicentric Castleman Disease (UCD): This type involves a single lymph node or a single region of lymph nodes. It is the more common form and is usually localized.
• Multicentric Castleman Disease (MCD): This type involves multiple lymph nodes and can affect lymphoid tissue in multiple regions of the body. MCD is often associated with systemic symptoms and can be more challenging to treat.

Symptoms

The symptoms of angiofollicular lymph hyperplasia can vary depending on the type:

• Unicentric Castleman Disease:
• Enlarged lymph node or mass
• Pressure-related symptoms depending on the location of the mass
• Multicentric Castleman Disease:
• Fever
• Night sweats
• Unintended weight loss
• Fatigue
• Enlarged liver or spleen
• Peripheral neuropathy

Causes

The exact cause of angiofollicular lymph hyperplasia is not well understood. However, some cases of multicentric Castleman disease have been associated with human herpesvirus 8 (HHV-8) infection, particularly in individuals who are immunocompromised.

Diagnosis

Diagnosis of angiofollicular lymph hyperplasia typically involves:

• Physical Examination: Checking for enlarged lymph nodes or other symptoms.
• Imaging Studies: Such as CT scans or MRI to identify enlarged lymph nodes.
• Biopsy: A lymph node biopsy is often necessary to confirm the diagnosis and to distinguish between unicentric and multicentric forms.

Treatment

Treatment options vary depending on the type of Castleman disease:

• Unicentric Castleman Disease:
• Surgical removal of the affected lymph node is often curative.
• Multicentric Castleman Disease:
• Medications such as corticosteroids, immunotherapy, or chemotherapy may be used.
• Antiviral therapy may be considered if HHV-8 is involved.

Prognosis

The prognosis for individuals with angiofollicular lymph hyperplasia depends on the type and severity of the disease. Unicentric Castleman disease generally has an excellent prognosis following surgical treatment. Multicentric Castleman disease can be more challenging to manage and may require ongoing treatment.

See Also

• Lymphadenopathy
• Human Herpesvirus 8
• Immunotherapy

External Links

• [Castleman Disease Collaborative Network](https://www.cdcn.org/)

NIH genetic and rare disease info

• NIH info on Angiofollicular lymph hyperplasia

Angiofollicular lymph hyperplasia is a rare disease.