Dialog Box

Loading...

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 (A-TLL)

Adult T-cell lymphoma (A-TLL) is an aggressive type of lymphoma where the cancerous T-cells are found circulating in the blood stream. A - TLL is more common in countries such as Japan and China where a viral infection called HTLV-infection -1 is more common. HTLV-1 infection can make people more likely to develop this type of lymphoma.A-TLL 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 lymph nodes 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 medicines with a monoclonal antibody, targeted therapies and stem cell transplant if required. 

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

ALCL 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

ALCL is an aggressive lymphoma, whereas the primary cutaneous type follows a more indolent course.

  • Patiients with ALCL are typically aged in their thirties and approximately 70% are male.

What are the symptoms?

ALCL systemic-type:
  • ALCL systemic - type has enlarged  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.

Both types of ALCL are diagnosed by biopsy of tumour tissue.

How is it treated?

ALCL systemic-type:

ALCL systemic type treatments are similar to the aggressive lymphomas, such as diffuse large B-cell lymphoma (DLBCL), Treatments generally utilised in ALCL can include combination chemotherapy medicines, radiotherapy, targeted therapies and stem-cell transplants when required. With combination therapy, many people with ALCL may be cured.

ALCL primary cutaneous-type:
  •  ALCL primary cutaneous type has treatments that include radiotherapy to the area 
  • Surgery to remove the area of skin affected and combination chemotherapy  
    medicines (used only in patients who have extensive involvement that cannot be treated with localised therapies). 
  • Spontaneous remission may also occur with this lymphoma. 

Cutaneous T-Cell Lymphoma (CTCL)

Cutaneous T Cell Lymphoma  ( CTCL) is a rare type of lymphoma 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.
  • Patients 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:
  • PUVA consists of a medicine 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 and is generally used when the lymphoma is over large areas of the skin..
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 medicine to make the skin more sensitive. Treatment may be given several times a week.
Radiotherapy:
  • Local radiation may be used for early-stage CTCL if only one or two small areas of skin are affected. Radiotherapy may also be used to treat the entire surface of the skin if the CTCL is more widespread. This type of radiotherapy 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 medicines may be applied directly to the skin in the form of an ointment. Intravenous chemotherapy may be used if the CTCL is more advanced.
Monoclonal Antibodies and Targeted Therapies:
  • Monoclonal antibodies and targeted therapies. Monoclonal antibodies in combination with chemotherapy medicines and targeted therapies are also available for patients with 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 and a medicine before returning it back to the patient. This procedure takes around 3 hours to complete and can be given every month, every fortnight, every week or more frequently depending on the person's skin and their response to treatment.

Peripheral T-Cell Lymphomas (PTCLs)

PTCLs are a group of aggressive lymphomas that affect a certain type of T-cell. They account for approximately 7% of NHL cases. There are many 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  T-cells. Usually there are no actual tumours. It is associated with systemic symptoms iincluding 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) when they are diagnosed..
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. This 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 such as liver, bone marrow, intestinal tract and skin.

How are they treated?

  • Treatment of PTCLs are similar to those used for other aggressive lymphomas, such as combination chemotherapy medicines, radiaotherapy, targeted therapies and stem-cell transplant when required. The response to treatment is not often as effective in PTCLs as it is in DLBCL. and as a result, stem-cell transplantation is sometimes considered an early treatment option in appropriate cases.

T-Cell Acute Lymphoblastic Leukaemia/Lymphoma (T-ALL)

T-cell Acute Lymphoblastic Lymphoma (T-ALL) s a type of aggressive lymphoma that occurs mainly in children and adolescents and more often in males than females. A second peak of occurrence is seen later in life in people over 40 years of age. Lymphoblastic cancers are classified as either lymphoblastic leukaemias or lymphoblastic lymphomas.

The most common symptoms include breathing difficulties and other problems resulting from a large mass in the mediastinal area (the centre area of the upper chest), as well as fluid accumulation around the lungs. This type of lymphoma can spread to the central nervous system and neurological symptoms may also be present at diagnosis.

How is it treated?

Intensive combination chemotherapy medicines are the most common treatment for older children and young adults with aggressive lymphoblastic lymphoma. Young people with localised disease have an excellent prognosis. Adults with later stage disease may have a stem cell transplant as part of their initial treatment plan.