Castleman Disease is a rare disease in which the body's disease-fighting network (lymphatic system) contains more than required cells (overgrowth of cells). It is also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia. Castleman disease can occur in a unicentric (localized) or multicentric (widespread) form.

Multicentric Castleman disease can be life-threatening because sometimes it is linked with HIV infection. It is also associated with other cell-proliferation disorders, including cancer of the lymphatic system (lymphoma), Kaposi's sarcoma and POEMS syndrome.

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Symptoms

Most patients suffering from unicentric Castleman disease don't notice any signs or symptoms. The affected lymph node is usually located in the chest, neck or abdomen. Signs and symptoms include:

• A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or eating
• An enlarged lump under the skin in the neck, groin or armpit
• Unexpected weight loss
• Less commonly, fever, night sweats and weakness

Symptoms of multicentric Castleman disease

• Fever
• Night sweats
• Fatigue and weakness
• Loss of appetite
• Unexpected weight loss
• Enlarged lymph nodes, usually around the neck, collarbone, underarm and groin areas
• Enlarged liver or spleen
• Skin rash
• Nerve damage in the hands and feet that leads to numbness

Diagnosis of Castleman disease
If your doctor suspects unicentric or multicentric Castleman disease, he is likely to do a physical examination of your lymph nodes, to determine their size and consistency. Your doctor may then recommend:

• Blood and urine tests: This is to help rule out the possibility of other infections or diseases. These tests can also reveal anemia and abnormalities in blood proteins.
• Imaging tests: They are performed to detect enlarged lymph nodes, liver or spleen. Along with these, CT scan or MRI of your neck, chest, abdomen and pelvis may be used.
• Lymph node biopsy: It is done to differentiate Castleman disease from other types of lymphatic tissue disorders, such as lymphoma. A tissue sample from an enlarged lymph node is removed and taken to a lab for testing.

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Treatment

Unicentric Castleman disease: It can be cured by surgically removing the diseased lymph node. A major operation may be needed if the lymph node is in your chest or abdomen. If surgical removal isn't possible, medicines may be used to shrink the lymph node following a radiation therapy.
Multicentric Castleman disease: Normally, surgery is not the right treatment for multicentric Castleman disease because of the number of lymph nodes involved. However, surgery may be required to remove an enlarged spleen. Treatment generally involves medications and other therapies to manage cell overgrowth including:

• Monoclonal antibodies, to block the action of the IL-6 protein that contributes to cell overgrowth.
• Chemotherapy, to slow overgrowth of lymphatic cells.
• Corticosteroids, to control swelling.
• Antiviral drugs, to block the activity of HHV-8 or HIV if you have one or both of those viruses.
• Thalidomide, to block the action of the IL-6 protein.

A diagnosis of Castleman disease can be demanding because the condition is uncommon and often occurs with other serious illnesses, such as HIV/AIDS and Kaposi's sarcoma.