What is post-transplant lymphoproliferative disease?
Post-transplant lymphoproliferative disease (PTLD), also known as post-transplant lymphoproliferative disorder, is a rare complication of organ transplantation.
PTLD causes your white blood cells (lymphocytes) to multiply abnormally. The white blood cells are part of your body’s immune system and are used to fight infection. In some cases of PTLD, the excess of white blood cells is mild and does not cause any problems. However, in other cases it can lead to lymphoma, a type of blood cancer.
PTLD affects around 1 in 50 people who have had a kidney transplant in the UK. It is more common in children and older adults. Men and women are affected equally.
The chances of developing PTLD are higher if you have had more than one transplant, either at the same time (such as a joint liver and kidney transplant for PKD) or repeated kidney transplants.
What are the signs and symptoms of PTLD?
Most cases of PTLD develop within six months of a transplant when you are taking the strongest dose of immune suppressant medicines to prevent your body from rejecting the new kidney. However, PTLD can develop up to ten years after a transplant.
Your transplant team will talk to you about your risk of developing PTLD.
Common symptoms of PTLD include:
- a low fever
- excess tiredness (fatigue)
- reduced appetite
- unexplained weight loss
- night sweats
- lumps in your neck, armpits or groin. These are swollen lymph nodes caused by the excess lymphocytes.
Your kidney team will monitor you carefully in the months after your transplant. You should discuss any symptoms or concerns about your health with them.
What causes PTLD?
Most cases of PTLD are linked to infection by the Epstein-Barr virus (EBV). This is a very common virus that affects 9 out of 10 people. Most people don’t know they have it as it doesn’t usually cause any symptoms. EBV stays in your body but is usually controlled by your immune system. When it isn’t controlled, it can cause some of your lymphocytes to become cancerous.
After a transplant, you will be given immune suppressant medicines to lower your immune system so that your body does not reject the transplanted kidney.
If you have already been infected by EBV, it may flare up when your immune system is lowered.
If you haven’t been previously infected, the transplanted organ may contain EBV cells which then infect you.
In rare cases, PTLD develops that is not linked to EBV infection. It is not yet known why these infections occur.
How is PTLD diagnosed?
Early diagnosis of PTLD improves the chances of successful treatment.
Blood and urine tests can check the levels of EBV in your body. High levels mean that you are at risk of developing PTLD.
PTLD is diagnosed by a biopsy of the affected part of the body. Blood tests and a CT scan may also be used to identify exactly which parts of the body are affected.
Does PTLD affect other parts of the body?
The lymphoma that PTLD causes can develop anywhere in your body.
Does PTLD run in families?
PTLD does not run in families.
How is PTLD treated?
Most cases of PTLD can be treated successfully. Treatment for PTLD has improved a great deal in the last few decades. Be careful of researching online as websites or research papers may have out of date information.
If tests show that you are at high risk of PTLD, your kidney team may recommend that you have preventive treatment to stop it from developing. This may with medication such as rituximab or by reducing the dose of your immunosuppressants.
If you develop PTLD, the most common treatments are:
- Reducing your immunosuppressive medicines to the lowest possible effective dose. This will be done gradually over several months to prevent your immune system from rejecting your kidney transplant. Your kidney team will monitor you carefully during this time for any signs of transplant rejection.
- An antibody therapy called rituximab which helps your immune system to recognise and destroy the cancerous cells.
- Chemotherapy
- Surgery to remove the affected lymph nodes. This is usually only recommended if the PTLD is affecting just one part of your body.
Your healthcare team will monitor you very carefully during your treatment for PTLD. Lymphoma treatment reduces your white blood cell count which makes you more vulnerable to infections. This can increase your risk of transplant rejection.
If your transplant is rejected, your kidney team may recommend that you wait at least a year after your PTLD treatment before you have another transplant, in order to reduce the risk of the infection coming back. You may need to have dialysis during this time.
Where can I get more information or support about PTLD?
For more information on PTLD, including its diagnosis, symptoms and treatment, visit Lymphoma Action.