Questions & Answers

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How is Castleman disease diagnosed ? 

The disease is usually diagnosed by analysing a sample (biopsy) taken from a lymph node. 

The diagnosis may be suspected following a blood test or radiological examination but a sample must also be taken from the lymph node.

How will I know which type of Castleman disease I have ? 

The simplest way is to speak to your doctor.

There are three forms of Castleman disease which are very different from each other.

 Unicentric Castleman disease is characterized by an enlarged lymph node which you can feel or which is discovered by chance during a radiological examination. This lymph node does not generally make you feel unwell. There are no particular symptoms.

- Idiopathic Multicentric Castleman disease is diagnosed either by the discovery of several enlarged lymph nodes or by a fever and unexplained anaemia. Examinations must be carried out to rule out the possibility of other diseases.

 Castleman disease associated with the HHV8 virus presents in the same way as the idiopathic form but traces of this virus are found either in the lymph node or the blood. It generally affects HIV-positive patients but can also affect other people. You can be infected by the HHV8 virus without being HIV-positive.

Is Castleman disease herditary ? 

No, Castleman disease is not hereditary and it can occur at any age.

Is Castleman disease contagious or infectious ?

No, Castleman disease cannot be passed on to other people.

Is Castleman disease a cancer of the lymph nodes ? 

No, Castleman disease is not a form of cancer. 

It can be considered as a benign tumour of the lymph nodes. 

However, acute forms of the disease exist which require quick, effective treatment.

Can I live a normal life with the Castleman disease ? 

Yes of course, but in some cases long-term treatment may be necessary.


Do we know what causes Unicentric Castleman disease ? 

No, the cause is unknown but current research suggests that it could be due to an anomaly (mutation) in a lymph node cell which leads to the development of an enlarged lymph node (adenopathy).


I do not have any symptoms, do I still need to have an operation ?

The best way of ensuring a full recovery is to have the affected lymph node removed completely. However, if there are no symptoms, simple monitoring may be an option.


My doctor has told me that the lymph node is in a difficult position and is therefore inoperable. What can I do ?

If surgery is difficult (because the lymph node is in an awkward position) and in the absence of symptoms, simple observation may be an option. Another option is to try to reduce the size of the lymph node with drugs or use embolization to make surgery easier.


I have heard about embolization, what does it involve? 

It involves inserting a small probe, during a radiological examination, into the blood vessel which irrigates the affected lymph node so that it is blocked and can no longer develop. Embolization can be carried out before surgery to make the procedure easier and in particular to prevent bleeding.


What is the risk of relapse after the operation ? 

The risk is very low (less than 5%) but not completely absent. It is probably higher if the disease is accompanied by a fever or anaemia, which is rare with this form.


My doctor has told me that the diagnosis is likely but not certain. Why ?

Some diseases which are also characterized by enlarged lymph nodes can “mimic” Castleman disease. In these cases, an analysis of the lymph node may suggest this diagnosis. This is why it is so important to carry out other examinations (blood tests, radiological examinations) and sometimes even to take a second sample on another lymph node to rule out other diseases


Is idiopathic Multicentric Castleman disease serious ?

The disease can present in an acute form, but we now have effective treatments which can control it.


How is idiopathic Multicentric Castleman disease treated ?

The treatments with the fastest effects involve antibodies which block the inflammation. Siltuximab (Sylvant®) is already used in many countries. Tocilizumab (RoActemra®) is used to treat other inflammatory diseases and obtains similar results. Rituximab (Mabthera®) attacks the lymph node cells. It is generally prescribed for less acute forms which are not associated with a fever or anaemia. Corticosteroids may also be used to control the disease in its initial stages but the effects may wane over time and long-term side effects make continued use difficult.


What are the risks or side effects associated with these treatments ? 

These treatments affect the immune system in general. They may conceal the warning signs of an emerging infection. It is therefore important to look out for symptoms which could suggest and infection, even in the absence of a fever, and to take antibiotics straight away.


What is the HHV8 virus ? 

It is a virus which is probably transmitted through the saliva and often occurs in childhood. This virus is usually present in the body throughout a person's life without showing any symptoms. In certain cases, however, this virus re-emerges and can cause diseases such as Castleman disease.


Is Castleman disease associated with the HHV8 virus transmissible ? 

No, the HHV8 virus is transmissible but the disease is not.


Is Castleman disease associated with the HHV8 virus serious ?

Yes, this disease can quickly develop into an acute form, which can be life-threatening if left untreated. However, if a diagnosis is obtained, specific treatment is usually effective very quickly.


How is Castleman disease associated with the HHV8 virus treated ? 

Emergency treatment involves the use of a drug which is commonly used to treat other diseases of the lymph nodes (Vepeside®, Celltop®). Basic treatment involves immunotherapy with the antibody rituximab (Mabthera®) which will destroy the affected cells.


What are the risks or side effects associated with these treatments ? 

This treatment is generally very well tolerated. It is important to monitor the condition over the weeks and months following the treatment by means of blood samples.


Is Castleman disease associated with the HHV8 virus a complication of HIV infection ? 

Yes, it may be considered a complication of HIV infection in this context.


Is the treatment different in the case of HIV infection ?

The treatment is the same for all forms of Castleman disease associated with the HHV8 virus. However, it is extremely important in this case to combine it with a treatment for HIV infection so that the viral load of HIV becomes or remains undetectable in the blood.