Diagnosis
and treatment

PTLD is related to your transplant and immunosuppressants. Your transplant team needs to be aware so they can diagnose and treat it quickly.

Diagnosis and treatment
Slick How is PTLD diagnosed?

Your transplant team will review your medical and transplant history. They may also ask you to meet with other healthcare providers. You may have to go in for:

Biopsies
Icon biopsies
Common types of tissue samples taken:
  • blood
  • bone marrow
  • cerebral spinal fluid (if the doctor suspects the central nervous system is involved)
Medical imaging
Icon medical imaging
Common types of imaging:
  • CT scan
  • PET scan
  • MRI

Afterwards, your transplant team will run tests on the tissue samples and analyze the results.
Two important tests are immunophenotyping and looking at levels of EBV.

What you can do
What you can do

Ask your transplant team if you have questions about PTLD. You can do this even if you haven’t seen them in a while. They are the experts on your transplant history and the immunosuppressants you are taking.
If you can’t see your transplant team right away, see your primary care provider, or if under 18, a pediatrician. They may be able to reach the transplant team for you.

Questions you can ask
Questions you can ask your transplant team
  • What are the tests that I need to have done?
  • What can I do to prepare for these tests?
  • How long do these tests take and when will the results come back?
What are the types of PTLD?

PTLDs range from mild, benign growths to aggressive cancers.

When your transplant team is analyzing the results of diagnostic tests, they are trying to understand:
  • the type of lymphocyte that is affected
  • if EBV is involved
  • the type of growth or lymphoma it is
These three factors, and whether the PTLD is new or has come back, will affect the treatment plan.
Types of PTLD

PTLD affects your lymphocytes. In most cases, your B cells are affected, but PTLD can also affect T cells and natural killer (NK) cells.

EBV can be linked to the development of PTLD. If there is a link to EBV, it's called EBV‑positive PTLD. If there is no link, it's called EBV‑negative PTLD.

The World Health Organization (WHO) has four categories for growths and lymphomas that are considered PTLDs. You may hear your doctor use these categories or the name of the growth or lymphoma.

Non-destructive: are usually benign growths. The following are considered non-destructive PTLDs if they appear after a transplant:

  • plasmacytic hyperplasia
  • infectious mononucleosis
  • florid follicular hyperplasia

Classical Hodgkin lymphoma (CHL): is the least common of the four categories. CHL that appears after transplant has its own category.

Polymorphic: is the most common type of PTLD in children.
Monomorphic: is the most common type of PTLD. The following are considered monomorphic PTLDs if they appear after a transplant:

  • diffuse large B-cell lymphoma (DLBCL)
  • Burkitt lymphoma (BL)
  • plasma cell myeloma or plasmacytoma
  • EBV‑positive marginal zone lymphomas
  • peripheral T-cell lymphoma (PTCL, NOS)
  • anaplastic large cell lymphoma (ALCL)
  • hepatosplenic T-cell lymphoma (HSTCL)
Questions you can ask
Questions you can ask your transplant team
  • What type of PTLD do I have?
  • What are the characteristics?
  • What treatment options are available to me?
  • What is my prognosis?
What types of treatments are there?
There are many types of treatment. The treatment options available to you will depend on factors like:
  • your general health and medical history
  • the type of PTLD you have
  • what treatments you've had
  • whether your PTLD is new or has come back
Since PTLD involves the immune system, many of the treatment options are immunotherapies.
Immunotherapies work by helping the immune system to fight a disease.
Adjusting your immunosuppressants

Your immunosuppressants may be carefully adjusted by your transplant team. This is done to help your immune system control PTLD while keeping your transplant healthy.

Adjusting your immunosuppressants
EBV-directed therapy

EBV-directed therapy targets and kills cells that are infected by EBV. It is used to treat diseases linked to EBV, including EBV‑positive PTLD.

EBV-directed therapy
Antibody therapy

Antibody therapy helps the immune system recognize and fight specific cells. In PTLD, it may be used to target B cells.

Antibody therapy
Surgery and radiotherapy

Surgery and radiotherapy are not usually used to treat PTLD. They may be used to control or reduce symptoms.

Surgery and radiotherapy
CAR T-cell therapy

CAR T-cell therapy is made from the patient’s T cells. The patient’s T cells are genetically modified in the lab to attack specific types of cancer cells.

CAR T-cell therapy
Clinical trials

Clinical trials are research studies. In a clinical trial, patients may receive an existing treatment or a new treatment that is being studied.

Clinical trials
Chemotherapy

Chemotherapy kills cells that grow and multiply quickly, including cancer cells. When it is used with antibody therapy, it’s called chemo-immunotherapy.

Chemotherapy

There may be other treatment options not listed here.

What you can do
What you can do
You can work with your transplant team to create a treatment plan. You can write down questions that come to mind over time and have a caregiver help take notes.
Questions
Questions you can ask your transplant team
  • What treatment options do I have and how will we choose?
  • How are these treatments administered or given?
  • How well did these treatments work for other patients?
  • Are there other patients I can talk to about their treatment experience?
  • How soon can I get treatment? Are there wait times?
  • What will treatment be like? (e.g., will I need to travel or take time off work?)
  • What will monitoring look like after treatment?
Who might be involved in my PTLD care?

Your transplant team will need to be involved. This is because PTLD is related to your transplant and immunosuppressants. You may also see other specialists, depending on your transplant type, age, and other health conditions.

Solid organ transplant

If you had a solid organ transplant, you may see:

  • your transplant team
  • a blood cancer specialist (also called a hematologist‑oncologist or heme‑onc)
  • other specialists, such as pediatricians, organ specialists, and radiologists
Blood stem cell transplant

If you had a blood stem cell transplant, you may see:

  • your heme-onc who performed the transplant
  • other specialists, such as pediatricians and radiologists
Questions
Questions you can ask your transplant team
  • Who might be involved in my PTLD diagnosis and treatment?
  • How often will I see the different people involved?
  • Who will be my main point of contact?
What is PTLD? Diagnosis and treatment What can I do next? Support and resources