Hematologist
CION Cancer Clinics
Castleman disease: symptoms, types and treatment | CION Cancer Clinics
Castleman disease is a rare, non-cancerous overgrowth of immune cells in the lymph nodes. When one area is affected, there may be no symptoms, and surgery to remove the node is often enough. When several areas are affected, fevers, night sweats, weight loss, swelling and tiredness are common, and treatment uses medicines that calm the immune system. A whole-node biopsy confirms it. At CION Cancer Clinics, every leukaemia, MDS and MPN case is reviewed by our haematologist and discussed at a tumour board before a plan is agreed.
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Medically reviewed by Dr. Basudev PokhrelConsultant Haematologist · last reviewed September 2026, next review due September 2027
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On this page
- What is Castleman disease, and what are its symptoms?
- What are the different types of Castleman disease?
- How is Castleman disease diagnosed?
- How is each type treated?
- What do families often get wrong about Castleman disease?
- What happens over time, and what can this page not tell you?
- Common questions about Castleman disease
The short answer
What is Castleman disease, and what are its symptoms?
Castleman disease is a rare condition in which lymph nodes grow too large because the immune cells inside them overgrow. It is not a cancer, but it is looked after by haematologists because it can behave like one and can make a person very unwell.
What lymph nodes are
Lymph nodes, sometimes called glands, are small bean-shaped parts of the immune system found in the neck, armpits, chest, tummy and groin. They swell for a short time during an infection. In Castleman disease, the swelling does not go away, and a biopsy shows a particular pattern that the pathologist recognises.
Symptoms depend on how many areas are affected
When only one group of nodes is enlarged, there may be no symptoms at all. The lump is often found by chance on a scan done for something else. Sometimes it presses on nearby organs and causes a cough, a feeling of fullness or pain.
When several areas are affected, the whole body tends to feel it. Fevers, drenching night sweats, weight loss, deep tiredness and loss of appetite are common. The liver and spleen may enlarge. Fluid can build up in the legs, the tummy or around the lungs, and blood tests often show inflammation.
These symptoms overlap with infections such as tuberculosis and with lymphoma. Only a biopsy can tell them apart.The types
What are the different types of Castleman disease?
The type decides almost everything about treatment. Your biopsy and blood tests tell your team which one you have.
Unicentric
One lymph node or one group of nodes in a single area, often in the chest, tummy or neck. Most people feel well, and the outlook after treatment is usually good.
Usually treated with
- Surgery to remove the node
- Radiotherapy if surgery is not possible
Multicentric, linked to HHV-8
Several areas of nodes, driven by a virus called human herpesvirus 8. It is seen most often in people living with HIV, so an HIV test is part of the work-up. People with HHV-8 disease can become very unwell during flares, with high fevers and swelling of the spleen.
Idiopathic multicentric
Several areas of nodes with no virus found. "Idiopathic" simply means the cause is not known. It is driven by an overactive immune signal called IL-6. Symptoms can range from mild to life-threatening, and they may come and go.
A severe form, called TAFRO, can cause fluid build-up, fever, low platelets and kidney trouble very quickly.Not sure whether this applies to you?
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When to go to hospital the same day
If someone with multicentric Castleman disease has a high fever with shivering, rapidly worsening breathlessness, swelling that is spreading fast, very little urine, confusion or unusual bleeding, go to the nearest emergency department now, or call 108. Tell the doctors about the Castleman diagnosis and bring the latest reports. Do not wait for the next clinic appointment.
Getting a diagnosis
How is Castleman disease diagnosed?
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Scans to map the nodes
A CT or PET-CT shows how many groups of nodes are enlarged and where. This separates the single-area type from the multi-area types.
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A biopsy of a whole node
Ideally the entire node is removed, not just a needle sample, because the pattern the pathologist looks for is spread through its structure.
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Blood tests
Blood counts, kidney and liver function, CRP, which measures inflammation, albumin and protein levels show how active the disease is.
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Tests for viruses
HIV and HHV-8 tests decide which type it is. HHV-8 can also be looked for in the biopsy itself.
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Ruling out look-alikes
Tuberculosis, lymphoma, autoimmune conditions such as lupus, and other infections can all look similar. Your team checks for these before confirming Castleman disease. This step can take a few weeks, and it is worth the wait, because the treatments for each are very different.
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Treatment by type
How is each type treated?
These are the usual approaches. Your plan depends on your type, how unwell you are and your other health conditions.
Type
Usual approach
Unicentric
Surgery to remove the node; radiotherapy or close watching if it cannot be removed safely
Idiopathic multicentric
Medicines that block IL-6, such as siltuximab or tocilizumab, often with steroids at first
Linked to HHV-8
Rituximab, with HIV treatment when HIV is present, and sometimes antiviral medicines
Severe or not responding
Hospital care, stronger immune-calming medicines or chemotherapy
Commonly believed
What do families often get wrong about Castleman disease?
"Swollen glands and fever for weeks, so it must be TB. Let us start TB medicines."
Tuberculosis is common in India and must be checked for. But starting TB treatment without proof can delay the right diagnosis by months. A biopsy settles the question, and the same sample can be tested for TB at the same time.
"The haematologist is involved, so it is lymphoma."
Castleman disease is not lymphoma. Haematologists treat it because it involves immune cells in the lymph nodes, and because the same medicines are sometimes used.
"The node was removed, so there is nothing more to do."
For the single-area type, surgery is often all that is needed. A few follow-up visits are still sensible to confirm that no new nodes appear.
"The fever has settled on steroids, so we can stop the other medicine."
Steroids calm symptoms quickly but rarely control the disease alone. Never stop or change any medicine on your own; speak to your treating team first.
Being straight with you
What happens over time, and what can this page not tell you?
The single-area type usually does well once the node is removed. The multi-area types are long-term conditions for many people. Treatment is often continued for a long time, with regular blood tests to check inflammation, blood counts and kidney function.
Why regular review matters
In some people, especially with the HHV-8 type, the risk of lymphoma is higher than usual. New or growing nodes, returning fevers or weight loss should be reported early rather than at the next routine visit.
Day-to-day life on long-term treatment
Many people on long-term treatment work, travel and look after their families. Some medicines lower the body's defences, so infections deserve prompt attention. Keep a copy of your latest reports and a list of your medicines on your phone. Ask your team which vaccines are safe for you, and tell any other doctor you see about your Castleman diagnosis.
What this page cannot tell you
A list of symptoms is not a diagnosis, and this page cannot tell you which type you have or how you will respond to treatment. That depends on your biopsy, your blood tests and how unwell you are. CION's haematology team reviews each case at a tumour board and coordinates specialised tests or medicines with qualified centres where needed.
Questions we are asked
Common questions about Castleman disease
Is Castleman disease a cancer?
No. It is an overgrowth of immune cells in the lymph nodes, not a cancer. But the multi-area types can cause serious illness and are treated with some of the same medicines used for blood cancers, which is why a haematologist usually leads the care.
Can Castleman disease turn into lymphoma?
Most people never develop lymphoma. The risk is higher than average in some groups, particularly people with the HHV-8 type. This is one reason for regular follow-up. Tell your team about any new lumps, fevers or weight loss rather than waiting.
Is Castleman disease infectious?
The disease itself does not spread from person to person. HHV-8, the virus linked to one type, can pass between people, but most people who carry it never develop Castleman disease. Family members do not need special precautions.
Why do they want to remove a whole lymph node?
The changes that confirm Castleman disease are seen in the structure of the whole node. A thin needle sample often misses them, which can lead to a wrong or unclear result and a repeat procedure. Removing a node is usually a short operation.
How long will treatment go on?
For the single-area type, often only the operation. For the multi-area types, treatment may continue for a long time, sometimes indefinitely, because symptoms can return when it stops. Your haematologist will review this with you at regular visits.
Are the IL-6 medicines available in India?
Availability and access can vary, and some of these medicines are costly. Your haematologist will explain what is available, what alternatives exist and how access can be arranged. Ask directly about cost and scheme coverage before treatment starts.
Will Aarogyasri or insurance help?
Tests, surgery and some treatments may be covered under Aarogyasri, PM-JAY, CGHS, ECHS, EHS or cashless insurance. Coverage for specialised medicines varies, and scheme rules change. Call the helpline with your card details and we will help you check your current cover.
What should I bring to a first appointment?
Bring every scan, the biopsy report and slides or blocks if you have them, all blood test results, and a list of medicines taken so far, including any TB treatment. A written note of when fevers, sweats and weight loss began is very useful.
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Sources
- National Cancer Institute — Lymphoma - Patient Version
- Leukemia & Lymphoma Society — Lymphoma
- American Society of Hematology — Patients
- NHS — Swollen glands
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
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