A group of health conditions that involve enlarged lymph nodes (it means lymph nodes that get bigger) and different symptoms is called Castleman disease. Most commonly, people who experience this condition have only one enlarged lymph node. It usually occurs in the neck or chest but may happen anywhere in the body too. In such cases, doctors call this condition unicentric Castleman disease (UCD).
However, people may experience multicentric Castleman disease (MCD) that involves different body regions. These enlarged lymph nodes in the body often lead to inflammatory symptoms and organ function problems.
Multicentric Castleman Disease Types
There are three types of MCD. For example:
- HHV-8-associated MCD – People with this MCD type should know that it is linked with herpes virus type 8 (HHV-8) and HIV (human immunodeficiency virus).
- Idiopathic MCD – The causes of this MCD type cannot be identified and it is also known as HHV-8-negative MCD. One of the most dangerous forms of MCD is called iMCD-TAFRO. The name comes from the symptoms that cause it.
- POEMS-associated MCD – In such cases, MCD is linked with another health condition known as POEMS syndrome. This is a quite rare blood disorder that causes damage to the nerves and other body parts.
Furthermore, some people may experience a few enlarged lymph nodes along with mild symptoms that do not indicate MCD. However, they may describe a rare subtype of Castleman disease known as oligocentric Castleman disease.
In addition, the treatment depends on the type of Castleman disease you experience. For instance, people who experience unicentric and oligocentric Castleman disease usually get rid of the condition by surgery that removes enlarged lymph nodes. However, people with MCD do not respond usually to the first treatment but fortunately, there are different medicines that can treat HHV-8-associated MCD and idiopathic MCD.
Symptoms
Most people with an unicentric type of Castleman disease often do not experience any symptoms and the enlarged lymph node is found during a physical examination or imaging test done for another reason. In any case, symptoms appear depending on the affected body part and the type of Castleman disease you experience. Check some symptoms below:
Body Symptoms
- Fatigue (extreme tiredness)
- Swelling
- Enlarged spleen or liver
- Night sweats
- Unusual weight loss
Blood Symptoms
- Reduced blood protein (albumin) or red blood cell count (anemia)
- Low or high platelet counts
- Increased levels of Creatinine (caused by poor kidney function usually) or antibodies (immunoglobulins)
Check also more serious symptoms caused by idiopathic MCD (also known as iMCD-TAFRO) below:
- Thrombocytopenia (reduced platelet count)
- Anasarca (swelling caused by buildup of fluids in the body)
- Fever
- Elevated levels of C-reactive protein, which is a marker of inflammation
- Reticulin fibrosis
- Organomegaly (swelling of certain organs)
It is advised to see a doctor immediately if you notice an enlarged lymph node on your neck, collarbone, underarm, or in the groin area. Unusual weight loss, extreme tiredness, and high fever also are signs you should contact your healthcare provider right away.
Causes
Experts do not fully understand the exact cause of unicentric or idiopathic multicentric Castleman disease (MCD). However, people with weakened immune systems or HIV infection may also experience an HHV-8-positive MCD). For more details, talk with your healthcare professional.
Risk Factors
Anyone can develop this condition no matter of gender or age. While most people are diagnosed with this condition during midlife, it may appear at any age including during childhood. Moreover, there are no specific risk factors for idiopathic multicentric and unicentric Castleman disease. However, people with HIV infection or other diseases that reduce the immune system’s function are at higher risk of getting HHV-8-positive multicentric Castleman disease.
What Are The Possible Complications of Castleman Disease?
In most cases, people with unicentric Castleman disease do not experience problems after the enlarged lymph node is removed and their life expectancy is unaffected. However, in some cases, people may develop a rare autoimmune disorder (Paraneoplastic pemphigus) that is life-threatening. This condition causes blisters on the skin and in the mouth that are misdiagnosed in most cases due to reduced risk of getting the condition. That’s why it is recommended for all people with UCD to be checked for paraneoplastic pemphigus.
However, idiopathic multicentric Castleman disease usually worsens suddenly causing life-threatening problems with the function of different organs. People with this MCD type frequently require a ventilator (a machine that helps with breathing), dialysis, and transfusions.
People with an HHV-8-positive MCD often experience life-threatening infections and organ failure. Generally, people with HIV/AIDS have reduced chances to recover.
Diagnosis
Physicians often perform a physical examination to check for enlarged lymph nodes and ask questions about symptoms and medical history. However, they can also perform the following tests to confirm the condition and rule out other infections or conditions that cause similar symptoms. For example:
- Urine and blood tests – These tests help doctors to exclude other conditions and infections with similar symptoms. Moreover, blood tests also can identify blood protein changes (which is a symptom of Castleman disease) and find anemia.
- Imaging tests – These are special tests that produce detailed images of different body parts. Imaging tests can determine enlarged lymph nodes, liver, or spleen. To diagnose Castleman disease, doctors usually perform a positron emission tomography scan (also called PET scan).
- Lymph node biopsy – This is the primary test used to diagnose Castleman disease. It involves the removal of a small sample of an enlarged lymph node, which is sent to a laboratory for testing.
Treatment
The treatment doctors will recommend depends on the type and severity of the condition you experience. For example:
Unicentric Castleman Disease
For people with UCD surgery is the only treatment needed usually. Sometimes, major surgery is needed because an enlarged lymph node occurs in the chest or abdomen.
Surgery that removes the enlarged lymph node usually cures UCD but sometimes, it is not possible to perform surgery. In such cases, doctors will prescribe you medications used to treat multicentric Castleman disease. In case medicines do not work, radiation therapy may help you.
Treatment for oligocentric Castleman disease is similar to UCD but experts continue studying new treatment options for people with this subtype of Castleman disease.
HHV-8-positive Multicentric Castleman Disease
The primary treatment for people with this MCD type is Rituximab. It helps to lessen the symptoms and treat the condition. In some cases, people need antiviral medicines (this group of medicines helps to block HHV-8 or HIV) and chemotherapy (used to destroy extra immune cells).
Idiopathic Multicentric Castleman Disease
The only medication approved by the FDA (Food and Drug Administration) to treat idiopathic MCD is Siltuximab. Those who notice improvements after taking this medicine tend to have a treatment for long periods. Siltuximab works by blocking the action of a protein (called interleukin-6). People with idiopathic multicentric Castleman disease make an excess of this protein.
Those who experience a severe form of idiopathic MCD may also need corticosteroids (such as Prednisone) to reduce inflammation and chemotherapy that helps to destroy immune cells that cause problems. In case you do not notice any improvements after using Siltuximab, physicians may recommend Rituximab and Sirolimus.
Frequently Asked Questions
What are the primary causes of Castleman disease?
Nowadays, experts do not fully understand the exact causes of this condition. However, more research is needed.
What is the main treatment for people with Castleman disease?
For people with UCD, surgery that removes the enlarged lymph node can be the only treatment needed. Sometimes, doctors may prescribe medicines, chemotherapy drugs, radiation therapy, corticosteroids, and others. For more details, discuss it with your healthcare professional.
What happens if Castleman disease is not treated?
Those who ignore the symptoms and do not get treatment for this condition may experience certain life-threatening complications including organ failure and even cancer. If you have additional questions, ask your healthcare professional.
