Peripheral T-cell lymphoma

What is peripheral T-cell lymphoma?

Peripheral T-cell lymphoma is a rare type of non-Hodgkin lymphoma. It develops from mature T-cells and accounts for approximately 7% of all non-Hodgkin lymphoma cases.

What are the types of peripheral T-cell lymphoma?

Researchers are classifying new subtypes of peripheral T-cell lymphoma all the time, making understanding of this rare type of non-Hodgkin lymphoma somewhat confusing. Better identification of sub types however, creates a better overall understanding of peripheral T-cell lymphoma, which will eventually lead to a more favourable general prognosis for this complex disease.

• Subcutaneous panniculitis-like T-cell lymphoma – Extranodal. Rare. Commonly involving nodules under the skin (subcutaneous) which can progress to open inflamed sores.
• Hepatosplenic (Gamma-Delta) T-cell lymphoma – Extranodal. Systemic (affects whole body). Infiltration of liver, spleen and bone marrow by malignant T-cells. Usually without tumours. Difficult to diagnose.
• Enteropathy-associated T-cell lymphoma – Extranodal. Very rare. Can occur in some people who have the integrated gluten-sensitive intestinal disease Coeliac Disease, often referred to as simply ‘Coeliacs’.
• Extranodal NK (natural killer)/T-cell lymphoma, nasal type – Extranodal. Previously referred to as angiocentric lymphoma and is more common in Asia and South America. Frequently occurs in the nose and nasal passages as the name suggests, but can also involve other organs. Has an aggressive course and can cause the serious condition- haemophagocytic syndrome.
• Angioimmunoblastic T-cell lymphoma – Nodal. One of the most common subtypes of peripheral T-cell lymphoma, accounting for approximately 20% of all T-cell lymphomas. Common symptoms include generalised lymphadenopathy (swollen/enlarged lymph nodes), fever, weight loss and skin rash.
• Peripheral T-cell lymphoma, not otherwise specified – Nodal and/or Extranodal. Most common subtype of PTCL representing a diverse group of diseases for which evidence is lacking a clearer definition. Extranodal sites which may be involved include the liver, bone marrow, intestinal tract and the skin.

How does Peripheral T-cell lymphoma affect the body?

Peripheral T-cell lymphoma is a lymphoma of the T-cells (also referred to as T-lymphocytes), a type of white blood cell that play a central role in the immune response of the body. T-cells mature in the thymus (a specialised organ of the immune system which sits in the upper chest region under the breastbone)- which is how they get their name, and circulate within the lymphatic system. Although there are many different sub types of peripheral T-cell lymphoma, they often present in a similar way, being widespread, enlarged, painless lymph nodes in the neck, armpit or groin. Other symptoms may include enlargement of the liver and spleen, night sweats, fever, weight loss and a skin rash.

Who does peripheral T-cell lymphoma commonly affect?

Peripheral T-cell lymphoma usually affects people over 60, but it can occur anytime in adulthood. It is slightly more common in men than it is in women.

What causes peripheral T-cell lymphoma?

Whilst the exact cause of peripheral T-cell lymphoma is unknown, it can be associated with the exposure to Epstein-Barr virus (EBV) or to the human T-cell leukaemia virus-1 (HTLV-1). The Epstein-Barr virus is also called human herpes virus 4 (HHV-4), and is one of the most common viruses in humans. Infection with EBV occurs by the oral transfer of saliva.

The HTLV-1 virus is a retrovirus, and is in the same class of virus as the HIV/AIDS virus. It is believed that the HTLV-1 virus is transmitted through sexual contact, exposure to contaminated blood and breastfeeding.

How is peripheral T-cell lymphoma treated?

Treatment for peripheral T-cell lymphoma aims to cure and includes the use of combination chemotherapy regimens, localised radiotherapy, stem cell transplants and steroid therapy. Just as individuals vary in terms of their medical condition, age and other factors; so too does the exact treatment one will receive for PTCL. It is vital that all treatment options be discussed with your specialist, including the possibility of a clinical trial as initial treatment where appropriate.