Post-transplant lymphoproliferative disease - Newcastle Hospitals NHS Foundation Trust

What is post-transplant lymphoproliferative disease?

Post-transplant lymphoproliferative disease (PTLD) is a lymph gland swelling which may develop into lymphoma (a type of cancer).

Anyone who has had a transplant and needs to take immunosuppressant medication can develop it.

PTLD can occur in any area of the body but mostly affects the lymph glands. Although people who have had a transplant are slightly more prone to developing any type of cancer, PTLD is the commonest type.

Why does PTLD develop?

The body’s immune system not only destroys bacteria, viruses and other organisms which can make us ill and it also destroys cancer cells.

Although immunosuppressant medication is essential to prevent rejection of a transplanted organ, it also makes the immune system work less efficiently.

This is why people taking immunosuppressant medicines are more likely to develop not only infections but cancers too.

Are there different types of PTLD?

Yes, there are different types of PTLD affecting cells in the immune system called lymphocytes. There are different types of lymphocytes, and some are more commonly affected than others.

Over 75% of cases of PTLD are linked to the Epstein Barr virus (EBV) causing ‘EBV- positive PTLD’. The EBV virus infects the B lymphocytes.
Most people will have been infected with EBV by the time they are adults. The majority will not know they have had it although some people will have developed glandular fever.
After being exposed to the EBV virus, it lies dormant in the body and is normally kept under control by the immune system.
If a person is taking immunosuppressant medicines, their immune system will be less able to control EBV, leading to a flare up.
Some will only develop glandular fever but in some people, the EBV causes the lymphocytes to change into cancerous cells.
So PTLD is a spectrum; some changes in the cells in the lymph nodes may be very mild whereas others may be very aggressive and change rapidly
Most transplant patients who have been exposed to EBV will not go on to develop PTLD.

How common is PTLD?

Not everyone who has had a transplant will develop PTLD even if they have had EBV.

Around 1 in 10 children who have had a heart or lung transplant will develop some type of PTLD.

Can PTLD be prevented?

It is very difficult to prevent PTLD developing. EBV is a very common virus in the community.

There is not yet a vaccine available against EBV or any effective preventative antiviral medicines.

The transplant team will try very hard to balance the amount of immunosuppressant medication that your child takes to prevent rejection but allow their immune system to destroy bacteria, viruses and cancer cells.

What are the symptoms of PTLD?

The symptoms of PTLD are often very general and can be like other problems such as rejection or infection. Often the symptoms depend upon which area of the body is affected.

Some symptoms may include

Fever
Lethargy
Poor appetite
Weight loss
Night sweats
Swollen lymph glands – these may be in areas such as the neck, armpits or groin but may be in other less obvious parts of the body.

If your child is unwell or is not getting better, it’s very important to contact the transplant team on the numbers below to discuss the best course of action.

If your child is very unwell, please call 999 or visit your local emergency department.

How is PTLD diagnosed?

PTLD can look similar to other illness such as infection or rejection. Your child’s transplant team will test for these initially and then perform other investigations to identify whether they have PTLD. Tests may vary depending upon which part of the body seems to be affected.

Your child’s transplant team will keep you fully informed about the tests needed.

Tests may include

A check up to ensure there is no heart or lung transplant rejection.
Blood tests to check that basic bodily functions are normal.
Samples to check for signs of infection e.g. urine.
Blood tests to check for signs of infection, inflammation, and tumour markers.
EBV PCR blood test to measure the amount of EBV virus in the blood stream.
Ultrasound scans may be needed to look for enlarged lymph nodes.
CT scan to look in more detail for enlarged lymph nodes or extent of disease.
Bone marrow aspirate may be needed to check for abnormal cells in the bone marrow.
A biopsy may be needed to identify whether your child has PTLD.

What is the treatment for PTLD?

Although PTLD is a serious condition, it can usually be successfully treated especially if diagnosed early.

The type of treatment your child needs will depend upon which type of PTLD they have. The transplant team will work very closely with the children’s oncology team.

The results of the tests will give the medical team the information they need to work out the best treatment course. Sometimes advice is needed from other specialists depending upon which area of the body is affected and how widespread the PTLD is.

Reduce the amount of immunosuppression

The first step the transplant team will normally take is to reduce the amount of immunosuppression your child takes. This allows the immune system to recover enough to try to eliminate the EBV or lymphoma cells. Care is needed to avoid risking rejection of your child’s heart or lung transplant. More frequent monitoring with blood tests and transplant clinic reviews are needed to keep a close check on your child. In some cases of very early PTLD, reduction of immunosuppression and very close monitoring may be enough.

Most children however need additional treatment which the medical team will talk to you about in detail. The commonest additional treatments include antibody therapy and chemotherapy. There are also other less common treatments which may be required for some of the rarer types of PTLD including surgery to reduce the size of the swollen tissue, radiotherapy and cytotoxic T lymphocyte therapy.

Antibody therapy

Antibody therapy with medications such as rituximab may be used either on its own or in combination with other treatments for some types of PTLD. Rituximab only works with some types of B cell lymphomas and helps the body’s immune system recognise and kill the B cells.

Chemotherapy

Chemotherapy is also sometimes needed depending upon the type of PTLD or your child’s response to treatment. This usually involves giving a combination of different medications and may be in addition to reduction of immunosuppression and antibody therapy.

The medical teams will work together very closely and keep you and your child fully informed of the plans and what to expect.

If you have any questions or concerns about the information in this leaflet, please contact your transplant specialist nursing team on the numbers at the end of this leaflet.

Resources

Contact

For further information, contact the transplant nurse specialists.

Office hours: Monday to Friday, 9am to 5pm

[email protected]

Contact out-of-hours

Outside of these times please ring ward 23 if you have an urgent query affecting your child’s transplant health.

0191 213 7023

Further information

PALS (Patient Advice and Liaison Service) for help, advice, and information about NHS services. You can contact them on Freephone 0800 032 02 02, email [email protected]