Castleman disease is an uncommon ailment characterized by an increase in the number of cells in your lymph nodes. The most common form of the condition (unicentric Castleman disease) involves a single lymph node, commonly in the chest or belly.
Multiple lymph nodes across the body are affected by multicentric Castleman disease, which has been linked to human herpes virus type 8 (HHV-8) and human immunodeficiency virus (HIV).
Treatment and prognosis differ based on the type of Castleman disease you have. Surgery is typically effective in treating the kind that affects only one lymph node.
WHAT ARE THE SYMPTOMS OF CASTLEMAN DISEASE?
Many persons with unicentric Castleman disease have no symptoms or indicators. The swollen lymph node may be discovered during a routine physical exam or imaging test for another reason.
Some persons with unicentric Castleman illness may have signs and symptoms similar to those seen in multicentric Castleman disease, such as:
- Fever
- Fatigue
- Sweating at night
- Nausea
- A swollen liver or spleen
- Unintentional weight loss
The swollen lymph nodes associated with multicentric Castleman disease are usually seen in the neck, collarbone, underarm, and groin region.
WHAT ARE THE CAUSES OF CASTLEMAN DISEASE?
It is unknown what causes Castleman illness. Infection with a virus known as human herpesvirus 8 (HHV-8) is linked to multicentric Castleman illness.
The HHV-8 virus has also been related to the formation of Kaposi's sarcoma, a malignant tumor that can be an HIV/AIDS consequence. According to studies, HHV-8 is present in virtually all HIV-positive Castleman disease patients and approximately half of HIV-negative Castleman disease patients.
WHAT ARE THE RISK FACTROS FOR CASTLEMAN DISEASE?
Castleman illness may strike anyone at any age. However, the average age of persons with unicentric Castleman disease is 35. The majority of persons who have the multicentric form are in their 50s and 60s. Men are somewhat more likely than women to have the multicentric form.
People who are infected with human herpesvirus 8 have an increased chance of getting multicentric Castleman illness (HHV-8).
WHAT ARE THE COMPLICATIONS OF CASTLEMAN DISEASE?
Patients with unicentric Castleman disease typically recover well once the afflicted lymph node is removed. Multicentric Castleman disease can result in potentially fatal infections or organ failure. People who also have HIV/AIDS tend to fare the worst.
Having any type of Castleman illness increases your chances of developing lymphoma.
HOW IS CASTLEMAN DISEASE DIAGNOSED?
Following a complete physical examination, your doctor may advise:
- Blood and urine tests will be performed to help rule out other infections or disorders. These tests can also identify anemia and blood protein abnormalities that are occasionally associated with Castleman disease.
- Imaging studies may be performed to detect swollen lymph nodes, liver, or spleen. It is possible that a CT scan or MRI of your neck, chest, abdomen, and pelvis will be used. PET scans can also be used to diagnose Castleman illness and determine whether or not a therapy is effective.
- Lymph node biopsy to distinguish Castleman disease from other forms of lymphatic tissue illnesses, such as lymphoma. A tissue sample is taken from an enlarged lymph node and analyzed in the laboratory.
WHAT ARE THE TREATMENTS FOR CASTLEMAN DISEASE?
The type of Castleman illness you have determines your treatment.
Unicentric Castleman disease
The diseased lymph node can be surgically removed to cure unicentric Castleman disease. If the lymph node is located in your chest or abdomen, which is common, extensive surgery may be necessary.
If surgical removal is not possible, medicines to reduce the lymph node may be employed. Radiation treatment may potentially be an effective method of destroying the damaged tissue.
Follow-up testing, including imaging, will be required to check for recurrence.
Multicentric Castleman disease
Medication and other therapy to limit cell overgrowth are commonly used to treat multicentric Castleman disease. The type of treatment you receive is determined on the severity of your disease and whether you have HIV, HHV-8, or both.
Multicentric Castleman disease treatment options may include:
- Immunotherapy: Drugs such as siltuximab (Sylvant) and rituximab (Rituxan) can inhibit the activity of a protein that is overproduced in persons with multicentric Castleman disease.
- Chemotherapy: This sort of drug can inhibit lymphatic cell proliferation. If the illness does not respond to immunotherapy or if you suffer organ failure, your doctor may advise you to add chemotherapy.
- Corticosteroids: Prednisone and other anti-inflammatory medications can assist.
- Antiviral medications: If you have HHV-8 or HIV, these medications can inhibit the activities of those viruses.
HOW IS CASTLEMAN DISEASE DIAGNOSED?
You can lower your risk of Castleman disease by lowering your chance of HIV infection. To stop the spread of this virus, you can do the following:
- When engaging in sexual activity, use condoms.
- Limit the amount of sexual partners you have.
- Avoid using drugs.
- When using intravenous (into the vein) drugs, avoid sharing needles.
CONCLUSION
Most persons with unicentric Castleman disease (UCD) who have the afflicted lymph node removed have a very favourable prognosis. Surgery is often seen to be curative. When treated, this condition has little effect on life expectancy.
People with multicentric Castleman disease (MCD) have varying prognoses. Some patients require continued treatment since the condition never completely disappears. In such circumstances, doctors devise a treatment strategy that may involve chemotherapy and other medications in order to keep the illness from worsening for as long as feasible.