Have you ever noticed that you get these little bumps on your neck or armpits? Sometimes they happen when you have a cold or flu. But, this isn't about something as simple as that. This is a rare but important condition to be aware of. We call it Castleman Disease .
Simply put, Castleman Disease is a condition in which our body's immune system becomes overactive . Imagine, when a germ that causes illness enters our body, our immune system fights it like a soldier going to war. When the illness is cured, that soldier goes silent again. But in a person with Castleman Disease, this immune system is constantly 'on'. This causes long-term inflammation inside the body, which can damage our organs.
At this time, our lymph nodes – those little bumps are what we call them – act like a filter. All the germs and immune cells are filtered through them. So, because the immune system is constantly working, these lymph nodes are also working overtime. Then the cells in them grow excessively, and the lymph nodes swell. Not only this swelling, but in Castleman Disease, there are even changes in the tissue of these lymph nodes.
This is a very rare disease . For example, in the United States, only 4,300 to 5,200 new cases are diagnosed each year. So doctors are still learning a lot about this. However, if you are diagnosed with this disease, you will receive specific treatments that are specific to you.
What are the main types of Castleman Disease?
There are two main types of this disease.
Unicentric Castleman Disease (UCD)
Unicentric Castleman disease (UCD) is when only one or a few lymph nodes in one area of your body become enlarged . Think just your neck, or just your armpits. About three-quarters of Castleman patients fall into this category.
Multicentric Castleman Disease (MCD)
Multicentric Castleman disease (MCD) is a condition where lymph nodes are swollen in multiple areas of the body, rather than just one. To be precise, doctors further divide this type of MCD into:
- POEMS-associated MCD: POEMS is a very rare blood disorder. Sometimes, POEMS can occur along with MCD.
- HHV-8-associated MCD: This type is caused by infection with a virus called Human Herpes Virus-8 (HHV-8) . If you have HIV infection or are immunocompromised, you are more likely to develop this type of MCD.
- Idiopathic MCD (iMCD): This is the most common type of MCD. 'Idiopathic' means that the exact cause is unknown.
Now this unknown cause iMCD also has three sub-parts:
- iMCD associated with TAFRO syndrome: Sometimes iMCD can be accompanied by a condition called TAFRO syndrome. The name TAFRO is derived from the first letters of its symptoms. These are: Thrombocytopenia (low blood platelets), Anasarca (swelling of the body with water), Fever , Renal (kidney) dysfunction, and Organomegaly (enlargement of the spleen or liver).
- iMCD with Idiopathic Plasmacytic Lymphadenopathy (iMCD-IPL): In this subtype, platelets may be elevated. Your white blood cells may also produce too many antibodies.
- iMCD not otherwise specified (iMCD-NOS): The exact cause of this type, called iMCD-NOS, is also unknown, and it is not related to TAFRO.
What are the symptoms of Castleman Disease?
The symptoms of Castleman disease vary depending on the type you have. In most cases, if you have unicentric Castleman disease (UCD), you may not have any major symptoms. Swollen lymph nodes may be the only sign you notice. However, sometimes symptoms can occur if a swollen lymph node presses on another nearby organ.
However, if you have MCD, the symptoms are more likely to be obvious. In addition to swollen lymph nodes, you may see symptoms such as:
- Fever
- Fatigue (this can be a sign of anemia)
- Sweating at night
- Nausea and vomiting
- Weight loss for no reason
- Swelling in your feet, ankles, or abdomen
- Enlarged spleen (splenomegaly) or enlarged liver (hepatomegaly)
- Numbness in the hands and feet (peripheral neuropathy)
What causes Castleman Disease?
The exact cause of unicentric Castleman disease (UCD) is not yet known, but infection with a virus called HHV-8 has been found to be a factor in the development of multicentric Castleman disease (MCD).
Scientists are still investigating other possible causes of UCD and iMCD. For example, they are looking into infections other than HHV-8, gene mutations , and autoimmune responses .
Who is most at risk of developing this disease?
Specific risk factors for UCD or iMCD have not yet been identified. However, if you have HIV infection or another condition that weakens your immune system, you are at increased risk of developing HHV-8-associated MCD.
Castleman disease can occur at any age, but it is most commonly diagnosed in people between the ages of 30 and 60 .
What are the possible complications of Castleman Disease?
Castleman disease increases your risk of developing cancer, especially lymphoma .
Also, although very rare, people with UCD are at risk of developing a serious condition called Paraneoplastic Pemphigus (PNP) . MCD also increases your risk of developing infections that can damage your organs. This damage can be life-threatening if not treated properly.
Don't worry, your doctor will constantly monitor your condition and help you prevent such complications whenever possible.
How do doctors diagnose Castleman Disease?
The symptoms of Castleman Disease can be similar to those of other common illnesses, such as the flu or a cold. So your doctor will first rule out those conditions. Then, they will do tests to diagnose Castleman Disease and determine the type.
Tests and procedures may include:
- Lab tests: Your doctor will check your blood cell count for abnormalities and other microscopic signs of Castleman disease. They may also order an HIV test . If you have HHV-8-related MCD, your HIV test may be positive.
- Imaging tests: Tests like CT scans and PET scans can help find swollen lymph nodes in your body, as well as check for other signs of Castleman disease, such as an enlarged liver or spleen.
- Lymph node biopsy: The only way to know for sure if you have Castleman disease is to have a biopsy. This involves taking a small piece of tissue from a lymph node and examining it under a microscope. This will determine if there are any signs of the disease.
How is Castleman Disease treated?
The treatment for Castleman disease varies depending on the type you have.
Treatment of Unicentric Castleman Disease (UCD)
The main treatment for UCD is to surgically remove the affected lymph nodes. Sometimes you may need radiation therapy or immunotherapy before surgery. These treatments shrink the growths in the lymph nodes, making them easier to remove.
But if surgeons can't remove the affected lymph node, and you don't have symptoms, your doctor may monitor it instead of treating it. If you have symptoms and can't have surgery, they may recommend treatments for MCD.
Treatment of multicentric Castleman disease (MCD)
MCD is a bit more difficult to treat than UCD. Because it's spread throughout the body, surgery or radiation therapy isn't usually used. Instead, treatment depends on whether you have HHV-8 infection and how severe the disease is.
Your doctor may use treatments such as:
- Corticosteroids: These medications help reduce inflammation and provide relief from symptoms.
- Chemotherapy drugs: Chemotherapy drugs control the overgrowth of cells in your lymphatic system. Rituximab is a drug commonly prescribed for HHV-8-associated MCD.
- Immunotherapy: This treatment involves using things like monoclonal antibodies . These help calm your body's immune response. Siltuximab (Sylvant®) is the only treatment approved by the U.S. Food and Drug Administration (FDA) for iMCD.
- Antiviral drugs: If you have HHV-8-related MCD, you may need antiviral drugs to treat HIV or HHV-8 infection.
When should I see a doctor?
If you notice a lump or a growth in your neck, armpit, or groin, see a doctor immediately . Also, if you have any of the other symptoms of Castleman Disease mentioned above that don't improve after a few weeks, tell your doctor about them as well.
How long can you live with this disease?
For most people with UCD, the prognosis, or outlook, is very good. Removing the affected lymph nodes can cure the disease. With treatment, UCD usually does not affect life expectancy.
But the outlook for people with MCD depends on the type of MCD they have and the severity of the disease. For some people, symptoms can develop suddenly and become life-threatening. For others, symptoms can last a long time. Between 65% and 75% of people diagnosed with MCD are still alive after five years . With new treatments, there is hope that this situation will improve.
There are no easy answers when it comes to Castleman disease. Your experience will depend on the type of Castleman disease you have. Additional conditions, such as HIV, or related conditions, such as TAFRO and POEMS, can also affect your experience. In some cases, surgery and close observation may be all that is needed. Or, you may need a variety of treatments throughout your life to prevent complications. Ask your doctor how your diagnosis will affect your treatment and outlook.
So, what are the most important things we need to remember from this?
- Castleman Disease is a rare but potentially serious immune system disorder in which our immune system becomes overactive.
- There are two main types: UCD and MCD. In UCD, lymph nodes swell in one place, while in MCD, they swell in multiple places.
- Symptoms vary. Sometimes there may be no symptoms at all, just a swollen lump. Or there may be symptoms such as fever, fatigue, and weight loss.
- The causes are not always clear, but the HHV-8 virus has been linked to MCD. People with HIV are at higher risk.
- Early detection and proper treatment can often lead to good outcomes. So if you notice an unusual lump, swelling, or other unusual symptoms on your body, seek medical advice immediately. The most important thing is to stay informed and not panic.
` Castleman Disease, Lymph Nodes, Lymphadenopathy, Immune System, UCD, MCD, HHV-8, Symptoms, Treatment
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