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Lymphoblastic lymphoma

What is lymphoblastic lymphoma?

Lymphoblastic lymphoma is an aggressive form of non-Hodgkin lymphoma. It is relatively rare, accounting for approximately 2% of all non-Hodgkin lymphomas.

Lymphoblastic lymphoma usually develops from T-lymphocytes but occasionally develops from B-lymphocytes. Clinically, lymphoblastic lymphoma behaves very similarly to acute lymphoblastic leukaemia (ALL), and the two conditions are often treated in similar ways. In lymphoblastic lymphoma, the abnormal lymphocytes are present in the lymph nodes or thymus gland, whereas in ALL, the abnormal lymphocytes are mainly in the blood and bone marrow.

How does lymphoblastic lymphoma affect the body?

People usually present with painless swelling in the neck, armpit or groin. This is caused by enlarged lymph nodes. In 50-75% of people a central inner chest lump (mediastinal mass) occurs which may cause difficulty breathing (dyspnoea) and chest pain. Other areas of the body that can be affected include the thymus gland, liver, spleen, skin, testes and brain. Fatigue, loss of appetite and weight loss often occur; in addition to drenching night sweats and unexplained fevers.

Who does lymphoblastic lymphoma commonly affect?

Lymphoblastic lymphoma is very rare in adults, most commonly affecting people under the age of 35. It is much more prevalent in children and teenagers, with the most common age at diagnosis being 20 years. It is slightly more common in men than it is in women.

Do we know what causes lymphoblastic lymphoma?

Whilst the exact causes of lymphoblastic lymphoma are unknown, factors such as exposure to radiation or pesticides, and congenital or acquired immunosuppression have been associated with an increased risk of developing lymphoblastic lymphoma.

How is lymphoblastic lymphoma treated?

Intensive chemotherapy is the main treatment for lymphoblastic lymphoma. Chemotherapy treatment is usually divided into three phases: induction, consolidation and maintenance. Chemotherapy may be given directly into the cerebrospinal fluid that surrounds the brain and spinal cord (intrathecal chemotherapy). Radiotherapy may also be given to the brain and spine if lymphoma cells are present in the cerebrospinal fluid; and/or to the chest or other areas if there is a large mass present. Some people are offered a stem cell transplant using cells  from a donor (allogeneic transplant), or sometimes with their own cells (autologous transplant). Prompt treatment with chemotherapy generally produces an excellent response for lymphoblastic lymphoma, however relapse is relatively common. New treatments are being researched all the time and people may be invited by their doctor to take part in a clinical trial to compare a new treatment against the best available standard treatment.

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