Peripheral T-cell lymphoma, unspecified

Peripheral T-cell lymphoma, unspecified (PTCLU) is a type of non-Hodgkin lymphoma (NHL) that starts in mature T cells. PTCLU includes a group of mature T-cell lymphomas that do not have consistent features, so they don’t fit into a specific type of T-cell lymphoma.

PTCLU is more common in Japan and other Asian countries than it is in North American or European countries. PTCLU usually occurs in people in their 60s. It develops more often in men than in women.

PTCLU mainly affects the lymph nodes. However, it can sometimes affect other organs or tissues, such as the bone marrow, skin, liver, spleen or organs in the gastrointestinal (GI) tract.

PTCLU is considered an aggressive lymphoma because it tends to grow quickly and is usually throughout the body, when it is diagnosed. Most people are diagnosed at an advanced stage (stage 3 or 4).

Symptoms

PTCLU often causes itchy skin and B symptoms (unexplained fever, drenching night sweats and unexplained weight loss).

Treatments

The main treatment for PTCLU is usually a combination of chemotherapy drugs. Sometimes radiation therapy, targeted therapy or a stem cell transplant may also be treatment options.

Chemotherapy

The combination of chemotherapy drugs used most often to treat PTCLU is CHOP:

• cyclophosphamide (Cytoxan, Procytox)
• doxorubicin (Adriamycin)
• vincristine (Oncovin)
• prednisone

Sometimes the following chemotherapy drugs may be used alone:

• fludarabine (Fludara)
• cladribine (Leustatin)
• pentostatin (deoxycoformycin, Nipent)
• gemcitabine (Gemzar)
• pralatrexate (Folotyn) – for relapsed or refractory PTCLU

Radiation therapy

Sometimes external beam radiation therapy is used in addition to chemotherapy. It is given to affected lymph node areas in people with early stage PTCLU.

Targeted therapy

Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.

Brentuximab vedotin (Adcetris) is a targeted therapy drug that targets CD30. It may be used along with chemotherapy to treat PTCLU that expresses CD30.

Targeted therapy may be used to treat PTCLU if it comes back (recurs) after treatment or other treatments stop working. The drugs used include:

• alemtuzumab (Campath)
• romidepsin (Istodax)

Stem cell transplant

A stem cell transplant may be offered to some people who are in remission after chemotherapy or if the PTCLU comes back after treatment.