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People who have received a heart, lung, liver or kidney transplant usually need to take drugs that suppress their immune system for the rest of their lives. Long-term immunosuppressionimmunosuppressionSuppression of the body’s immune system so it is less able to fight infections or diseases. leads to a higher risk of developing a second cancer. The most common cancers in people who receive organ transplants are skin cancer and post-transplant lymphoproliferative disorder (PTLD). PTLD behaves like an aggressive lymphoma. Lymphoproliferative disorders are among the most serious complications of long-term immunosuppression for people who have organ transplants. With lymphoproliferative disorders, lymphocytes (a type of white blood cell) are over-produced or act abnormally.
PTLD occurs in about 1–2% of people who receive organ transplants. PTLD can also occur after allogeneic stem cell transplant, especially if people received stem cells with many of the T-cells removed (T-cell depleted).
PTLD is almost always related to a virus that has been linked to lymphoma, the Epstein-Barr virus (EBV). However, people who are EBV negative can also develop PTLD.
The majority of PTLDs are B-cell lymphomas. About 12–14% of PTLDs are T-cell lymphomas.
People with PTLD present with enlarged lymph nodes (lymphadenopathy) or a malignant mass. The cancer has often spread to organs or tissues outside of the lymph nodes (extranodal involvement). Common extranodal sites include the lung and gastrointestinal tract. PTLD also spreads to the central nervous system (CNS) in some cases.
Most cases of PTLD occur within the first year of organ transplant. EBV-negative PTLD often occurs later (about 5 years after a transplant). EBV-negative PTLD is associated with a poorer prognosis.
Other factors associated with a poorer prognosis at the time PTLD is diagnosed include:
PTLD is treated based on the unique needs of the person with cancer. The following are treatment options for PTLD.
In most cases, reducing immunosuppressant therapy can be effective in managing the lymphoma. Other types of therapy often need to be given as well.
If reducing immunosuppressant therapy is not an option or doesn’t help manage the lymphoma, doctors may consider giving a monoclonal antibody called rituximab (Rituxan). Sometimes, a combination of an antiviral drug called acyclovir (Zovirax) and interferon alfa (Intron A, Wellferon) may be used.
If the drug therapies mentioned above do not seem to be working, a doxorubicin-based combination chemotherapy may be used, including:
People with localized PTLD may be treated with surgery or radiation therapy alone. Localized tumour can grow over a period of months, and tend to occur within weeks or a few months after a transplant.
A donor lymphocyte infusion (DLI) may be given if the person has PTLD after an allogeneic stem cell transplant. T-cells from the donor work against PTLD.
For more detailed information on specific drugs, go to sources of drug information.