Post-transplant lymphoproliferative disorder (PTLD)

What is post-transplant lymphoproliferative disorder (PTLD)?

Post-transplant lymphoproliferative disorder (PTLD) is a group of rare disorders of the lymphatic system in which white blood cells grow and spread too quickly. The disorder is usually caused by a viral infection and happens after an organ transplant when immunosuppressant medicines lower your immune system's ability to fight infection. 

Your lymphatic system is a group of organs, vessels and tissues that work as part of your immune system to protect you from infection. This system moves a colorless, watery fluid (lymph) that includes blood cells throughout your body. The different types of PTLD affect people in different ways. Some people have mild symptoms while others develop cancer. There are four main types of PTLD:

• Monomorphic PTLD: This is the most common form of PTLD and has one type of cell.
• Early lesion: This type can go away with a lower dose of immunosuppression, allowing your immune system to fight off PTLD. 
• Polymorphic PTLD: This has a mix of different types of cells.
• Classic Hodgkin lymphoma — PTLD type: This cancer is the least common form of PTLD and is the most serious.

About 3 in 100 (3%) people who have a kidney transplant get PTLD. It is most common in children and in older adults.

What causes PTLD?

PTLD is usually caused by a virus. The virus infects the lymphatic system that is part of your immune system, causing white blood cells to quickly grow out of control. This can happen because the immune system is weakened by immunosuppressants you take to keep your body from rejecting your new kidney.
PTLD usually happens within three years of a transplant, but it can happen any time after a transplant.

PTLD that happens within three years of an organ transplant is usually caused by an infection from the Epstein-Barr virus (EBV). EBV is a very common virus that usually doesn't cause symptoms in people with a healthy immune system. More than 9 of 10 adults in the U.S. have had an EBV infection, and the virus can remain in the body for life. 

After an organ transplant, EBV can become active and cause a type of white blood cell called B-cells to start growing too fast. The EBV may have already been in your body or may be in the donor kidney.

PTLD that happens more than three years after an organ transplant may be caused by an infection from other viruses. Doctors and researchers are still learning about the causes. 
 

What are the symptoms of PTLD?

PTLD symptoms can start anytime, from a few months after a transplant to several years later. Symptoms may include:

• Feeling tired (fatigue)
• Fever
• Not wanting to eat as much as usual
• Unexpected weight loss
• Sweating at night
• Swollen lymph nodes, which may feel like a lump in your neck, armpits, or groin

Tell your doctor right away about any symptoms, because finding and treating PTLD early gives you the best chance for recovery.
 

What are the tests for PTLD?

To find out if you have PTLD, your doctor will do a physical exam and tests that may include:

• Blood tests, including a test to check for EBV 
• Urine (pee) test
• Imaging tests such as CT, MRI or PET scans
• Bone marrow biopsy, which is a test where doctors get a small sample of your bone marrow to look at under a microscope
• Lymph node biopsy, a test where doctors take a small sample of a gland in your body called a lymph node to look at it closely under a microscope 
   

How is PTLD treated?

Your transplant doctors will need to work closely with your other doctors to treat your PTLD. 

Doctors will slowly lower the dose (amount) of immunosuppressant medicines you take. They must do this carefully because you still need enough immunosuppressants to keep your body from rejecting your transplanted kidney. Based on the type of PTLD you have, lowering your immunosuppressant medicine dose may be the only treatment you need.

Most people with PTLD also need treatment such as:

• Antibody therapy with rituximab, which is medicine that works with your immune system to fight the cancer (immunotherapy)
• Chemotherapy
• Radiation therapy 
• Surgery