Multicentric Castleman Disease

Multicentric Castleman is a disease that affects more than one group of lymph nodes. It may also affect other organs that contain lymphoid tissue. This disease sometimes occurs in people infected with HIV, which also causes AIDS.

Multicentric Castleman disease is much more dangerous than the localized type, especially in people with HIV infection. People suffering from this disease often have nerve damage problems that cause severe infections, fever, weight loss, fatigue, and night sweats.

How Many Groups Is Multicentric Castleman Disease Divided Into?

Multicentric Castleman cases are generally divided into two different clinical groups.

• Ones that are associated with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder and skin changes
• Idiopathic Multicentric Castleman disease associated with Thrombocytopenia, Anasarca, Myelofibrosis, Renal dysfunction and Organomegaly.

What Are The Symptoms?

The symptoms of Castleman disease vary according to the type. There may be cases where patients in single-center Castleman encounter no symptoms.  This type usually occurs during an examination for another condition.

Common symptoms include:

• Pressure and fullness in the abdomen or chest
• Lump formation under the skin of the armpits, neck or groin
• Unexplained weight loss

Multicentric Castleman disease has different symptoms in addition to these.

• Fever
• Night sweats
• Fatigue
• Appetite loss
• Abnormal lymph nodes, typically in the neck, armpits, collarbone, and groin
• Enlarged spleen or liver
• Anemia

How Is It Diagnosed?

There are several different methods for diagnosing the disease. Doctors will do one or more of the following tests, depending on the situation.

• Biopsy: Tissue sample is taken from the lymph node and examined.
• Blood and urine tests: Samples are taken to evaluate the levels of substances that cause the  disease.
• Imaging tests: Enlarged test nodes are detected by methods such as X-ray and computed tomography.

How Is Multicentric Castleman Disease Treated?

Treatment of the disease usually consists of drugs and treatments aimed at controlling excessive cell growth. The degree of the disease and whether there is HIV or HHV-8 infection are effective in determining the appropriate treatment.

Generally applied treatments are as follows:

• Immunotherapy: By using drugs such as siltuximab or rituximab, the effect of excess protein produced by the body, can be prevented.
• Chemotherapy: It can slow the overgrowth of lymphatic cells. Chemotherapy may be recommended if the patient does not respond to immunotherapy or if there is organ failure.
• Corticosteroids: Medications like prednisone can help control inflammation.
• Antivirals: Can be used to inhibit HHV-8 or HIV activity.