Castleman disease

Diagnosis

The diagnosis of Castleman disease often starts with discussing the signs and symptoms, performing a thorough physical exam, and assessing one’s medical history. Several tests may be performed by the healthcare professional to rule out certain illnesses. Further testing will be performed to determine the type of Castleman disease.

One or multiple of these tests may be necessary:

• Blood and urine tests: Blood cells may be examined for evidence of anemia (low red blood cell count), thrombocytosis (high platelet count), or thrombocytopenia (low platelet count) (low platelet count). Certain types of Castleman disease are associated with abnormal blood cell counts. An HIV test may be performed by your provider. HIV positivity is common in HHV-8-associated MCD.
• Imaging tests: Imaging techniques such as X-rays, CT scans, MRIs, and PET scans can assist doctors in identifying other symptoms of Castleman disease, such as an enlarged spleen or liver, as well as detecting swollen lymph nodes in the body. PET scans can help diagnosis the disease and can also determine whether a treatment is effective.
• Lymph node biopsy: During a biopsy, the doctor extracts a tissue sample or an entire lymph node for investigation under a microscope to find Castleman disease pathology characteristics. The test involves checking the tissue if it contains HHV-8. The results will if one has HHV-8-associated MCD or another type of Castleman disease. A lymph node biopsy is performed to differentiate Castleman disease from other types of lymphatic tissue illnesses, such as lymphoma.

Treatment

To ensure effective treatment, it is essential to determine the type of Castleman disease an individual has, as there are different treatment options available.

• Unicentric Castleman disease (UCD): Surgery is often the primary treatment for UCD. The diseased lymph node, commonly in the chest or abdomen, can be surgically removed to cure UCD. Patients may need radiation therapy or immunotherapy before UCD surgery, depending on where the lymph nodes are located or if they are too large. These treatments cause the growths on the lymph nodes to decrease, making them easier to remove.

If there are no symptoms and the tumor cannot be removed, the doctor may advise monitoring the tumor instead of immediate treatment. Medication and radiation therapy can effectively reduce or destroy the damaged tissue. Follow-up checkups are essential to monitor recovery.

• Multicentric Castleman disease (MCD): MCD is a harder condition to cure than UCD. Doctors rarely employ surgery or radiation to treat it. The type of treatment is determined by the severity of the disease and whether patients have HIV, HHV-8, or both. Medicines and other therapies to limit cell overgrowth are commonly used to treat multicentric Castleman disease.

Various treatments are available, including:

• • Corticosteroids: This is frequently suggested in severely unwell patients to efficiently manage the inflammation. These medications such as prednisone, helps relieve symptoms by reducing inflammation.
  • Immunotherapy: This medication works by inhibiting a protein that promotes tumor growth in MCD patients. Humanized monoclonal antibodies or proteins that aid in the fight against infections that stimulate the immune system is used on this treatment. Common immunotherapy drugs include siltuximab and rituximab.
  • Chemotherapy: This option is often recommended if immunotherapy alone fails to cure MCD or in case of organ failure. Anticancer drugs decrease cell development in the lymphatic system. Rituximab is a regularly used drug to treat HHV-8-related MCD.
  • Antiviral drugs.: These medications are used to treat HIV or HHV-8. For patients who have HHV-8-associated MCD, and another illness, antiviral medications and additional therapies may be prescribed.