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Mature T-Cell Lymphomas

Page Sections 

Adult T-Cell Leukaemia/Lymphoma

Anaplastic Large Cell Lymphoma (ALCL): Systemic-Type and Primary Cutaneous-Type

Cutaneous T-Cell Lymphoma (CTCL)

Peripheral T-Cell Lymphomas (PTCLs)


 

Adult T-Cell Leukaemia/Lymphoma

What is this?

  • Adult T-cell lymphoma is an aggressive type of T-cell lymphoma where the cancerous T-cells are found in the circulating blood.
  • Adult T-cell leukaemia/lymphoma is more common in countries such as Japan and China where a viral infection called HTLV-infection is more common. HTLV-1 infection can make people more likely to develop this type of T-cell lymphoma.
  • This type of NHL can occur at any age from young adulthood to old age. It occurs slightly more often in men than in women. 

What are the symptoms?

  • The most common symptoms include swollen, enlarged lymph nodes (lymphadenopathy) and an enlarged liver and spleen (hepatosplenomegaly).
  • There may also be signs of skin involvement, high calcium levels in the blood (hypercalcaemia), bone involvement and high levels of an enzyme called lactate dehydrogenase (LDH).
  • Diagnosis of adult T-cell leukaemia/lymphoma is made by lymph node biopsy. X-rays, bone marrow biopsy, CT scans and blood tests may also be performed. The presence of HTLV-1 virus must also be established.

How is it treated?

  • Adult T-cell leukaemia/lymphoma is treated using combination chemotherapy regimens. Some people can live with this cancer for a long time.

Anaplastic Large Cell Lymphoma (ALCL): Systemic-Type and Primary Cutaneous-Type

What is this?

  • Anaplastic refers to the appearance of the lymphoma cells, which look quite different from normal lymphocytes. ALCL can occur in two different forms:
    • Occurring throughout the body - "systemic"type
    • Occurring in the skin only - primary cutaneous type
  • ALCL systemic-type is an aggressive NHL, whereas the primary cutaneous type follows a more indolent course. The cancerous cell in either type of ALCL can be a T-cell, or a cell that is lacking B-cell or T-cell markers (called “null”).
  • People with ALCL are typically aged in their thirties and approximately 70% are male.
  • A benign condition that can mimic primary cutaneous ALCL is called lymphomatoid papulosis.

What are the symptoms?

ALCL systemic-type:
  • Enlarged and swollen lymph nodes, as well as involvement of other organs. Systemic symptoms and elevated levels of the enzyme lactate dehydrogenase (LDH) occur in approximately 50% of people. Bone marrow and the gastrointestinal tract are rarely involved, but skin involvement is common.
ALCL primary cutaneous-type:
  • Usually appears as a single lump or ulcerating tumour in the skin. Lymph nodes in the area may also become involved. The affected cells involved in this type of lymphoma have a certain protein on their surface called the CD30 antigen.
  • Both types of ALCL are diagnosed by biopsy of tumour tissue.

How is it treated?

ALCL systemic-type:
  • Treatments appropriate for other aggressive lymphomas, such as diffuse large B-cell lymphoma (DLBCL), are generally utilised in ALCL e.g. CHOP chemotherapy, radiation therapy, stem-cell transplants and steroids. With combination chemotherapy, many people with ALCL may be cured.
ALCL primary cutaneous-type:
  • Spontaneous remission may occur with this condition. If not, the most common treatments include radiation therapy to the area, surgery to remove the area of skin affected and chemotherapy (used only in people who have extensive involvement that cannot be treated with localised therapies).

Cutaneous T-Cell Lymphoma (CTCL)

What is this?

  • CTCL is a rare type of NHL caused by cancerous growth of T-cells in the skin. It is most common in adults between 40 and 60 years of age.

There are a few sub-types of CTCL, the most common being:

Sezary syndrome:
  • Large areas of skin or lymph nodes are affected. People with this lymphoma may have redness of the entire skin surface and tumour cells which circulate in the bloodstream. This type of CTCL often follows an aggressive course.
Mycosis fungoides:
  • The general name given to the other types of CTCL when the blood is not affected. Often, several years of eczema-like skin conditions occur before the diagnosis is finally established. In advanced stages, the lymphoma can spread to lymph nodes and other organs.

What are the symptoms?

  • CTCL can appear as small, raised, red patches on the skin, often on the breasts, buttocks, skin folds and face. These patches often look similar to eczema or psoriasis, and may be associated with hair loss in the affected area.
  • People in later stages may have ulcerating tumours that appear on the skin. Lymph nodes in the affected region may also be involved.
  • The diagnosis is made by skin biopsy of the area of affected skin. In the early stages of mycosis fungoides, biopsy may be difficult to interpret and the correct diagnosis can only be made after observing the person over time.

How is it treated?

Many therapies are used to treat CTCL. They include:

PUVA:
  • Also called photochemotherapy, this treatment is used if large areas of skin are affected. PUVA consists of a drug called psoralen plus ultraviolet A (UVA) light. Psoralen makes the skin more sensitive to the healing effects of the UVA light. The treatment is similar to sitting under a sunlamp and may be given several times a week.
UVB therapy:
  • Ultraviolet B (UVB) light slows the growth of the cancerous cells in the skin. This treatment does not include the use of a drug to make the skin more sensitive. Treatment may be given several times a week.
Radiation therapy:
  • Local radiation may be used for early-stage CTCL if only one or two small areas of skin are affected. Radiation therapy may also be used to treat the entire surface of the skin if the CTCL is more widespread. This type of radiation treatment is called total skin electron beam treatment. It is only given once and then may be followed up with further PUVA treatments if needed.
Chemotherapy:
  • Chemotherapy drugs may be applied directly to the skin in the form of an ointment. Intravenous chemotherapy may be used if the CTCL is more advanced.
Bexarotene (Targretin):
  • This is a medication in capsule form that may be used to treat advanced CTCL.
Interferon:
  • Interferon is a naturally occurring protein in the body and is an important part of a healthy immune system. A synthetic form of interferon can be injected under the skin to help boost the immune response and fight the CTCL.
Photopheresis:
  • This treatment is used particularly for Sezary syndrome. It involves passing the person’s blood through a machine where it is exposed to ultraviolet light.

Peripheral T-Cell Lymphomas (PTCLs)

What is this?

  • PTCLs are a group of aggressive NHL that affects a certain type of T-cell. They account for approximately 7% of all NHL cases. There are many distinct sub-types of PTCLs, including:
Subcutaneous panniculitis-like T-cell lymphoma:
  • This type of PTCL is quite rare and is often confused with a condition called panniculitis, an inflammation of fatty tissue in the body. The most common symptoms include nodules under the skin (subcutaneous nodules) which can progress to open, inflamed sores. Haemophagocytic syndrome—a serious condition in which there is uncontrolled activation of certain parts of the immune system— is also common in this cancer.
Hepatosplenic gamma delta T-cell lymphoma:
  • This type of PTCL affects the whole body, with infiltration of the liver, spleen and bone marrow by cancerous T-cells. Usually there are no actual tumours. It is associated with systemic symptoms (e.g., fever, weight loss, night sweats, fatigue) and is quite difficult to diagnose.
Enteropathy-type intestinal T-cell lymphoma:
  • This type of PTCL is very rare and occurs in people with untreated gluten-sensitive intestinal disease, called celiac disease. These people are often in a very weakened state and may have intestinal perforation (an abnormal hole in the wall of the intestine).
Extranodal T-cell lymphoma, nasal type:
  • This type of PTCL, previously referred to as angiocentric lymphoma, is more common in Asia and South America. It most frequently affects the nose and nasal passages but can involve other organs as well. It has an aggressive course, and haemophagocytic syndrome can also occur in this condition.
Angioimmunoblastic T-cell lymphoma:
  • This is a more common sub-type of PTCL, accounting for approximately 20% of all T-cell lymphomas. Symptoms include generalised lymphadenopathy (swollen lymph nodes), fever, weight loss, skin rash and high levels of antibodies in the blood.
PTCL, unspecified:
  • PTCL, unspecified is the most common PTCL sub-type. It represents all of the PTCLs lacking in a clear definition and thus not classifiable as a specific sub-type. Most people with PTCL, unspecified have lymph node involvement however a number of extranodal sites may also be involved (e.g., liver, bone marrow, intestinal tract, skin).

What are the symptoms?

  • The symptoms of PTCL are specific to the sub-type. Refer to the specific sub-type for a description of the most common symptoms.
  • The diagnosis of PTCLs requires a biopsy and thorough examination of the cancerous tissue. Other tests such as X-rays, bone marrow biopsy, CT scans and blood tests may also be performed.

How is it treated?

  • Treatment of PTCLs generally involves combination chemotherapy. Treatments are similar to those used for other aggressive lymphomas, such as CHOP chemotherapy, radiation therapy, stem-cell transplants and steroid therapy. The response to treatment is not often as effective in PTCLs as it is in DLBCL. As a result, stem-cell transplantation is sometimes considered an early treatment option in appropriate cases.