What is Lymphoblastic lymphoma (non-Hodgkin’s lymphoma)

Lymphoma is cancer of the lymphatic system, and can be further divided into Hodgkin’s and non-Hodgkin’s lymphoma. Lymphoblastic lymphoma is classified under non-Hodgkin’s lymphoma.
The lymphatic system is an important part of the body’s immune system that helps us fight infection. It is composed of the lymph nodes, thymus (a gland behind the breast bone), spleen and bone marrow, which are connected by tiny lymph vessels.
Lymph is a colourless fluid that circulates in the lymphatic system. It contains lymphocytes which are white blood cells that fight infection. There are 2 types of lymphocytes: B cells and T cells. Lymphoblastic lymphoma involves immature T cells in 85-90% of cases and immature B cells in the remainder of cases.
Lymphoblastic lymphoma is very similar to the leukaemia called acute lymphoblastic leukaemia (ALL). ALL mainly involves lymphocytes in the blood and bone marrow, whereas lymphoblastic lymphoma involves lymphocytes in the thymus and lymph nodes. However, it may also affect sites other than these, e.g. the spleen and bone marrow.

Statistics on Lymphoblastic lymphoma (non-Hodgkin’s lymphoma)

Lymphoblastic lymphoma is an uncommon form of non-Hodgkin’s lymphoma, accounting for about 4% of all non-Hodgkin’s lymphomas. Lymphoblastic lymphoma , however, is more common in children and adolescents, making up about one third of all non-Hodgkin’s lymphomas in childhood. It more commonly affects males than females.

Risk Factors for Lymphoblastic lymphoma (non-Hodgkin’s lymphoma)

The exact cause is unknown.
Possible factors that increase the risk of developing lymphoblastic lymphoma:

Progression of Lymphoblastic lymphoma (non-Hodgkin’s lymphoma)

Patients with the B-cell subtype of lymphoblastic lymphoma usually come with leukaemia, as the disease rarely manifest as solid tumours. Most cases occur in children.
The T-cell subtype, on the other hand, typically causes a tumour in the thymus (behind the breastbone) but may also cause enlargement and disease of lymph nodes. This type of lymphatic involvement is usually followed by development of leukaemia.
Like all other non-Hodgkin’s lymphomas, lymphoblastic lymphoma is staged from I to IV.
Stage I – Lymphoma involving 1 lymph node site
Stage II – Multiple sites of lymphoma on 1 side of diaphragm
Stage III – Lymphoma on both sides of diaphragm, including spleen
Stage IV – Lymphoma in sites outside the lymph nodes (eg, marrow, brain)

How is Lymphoblastic lymphoma (non-Hodgkin’s lymphoma) Diagnosed?

The doctor will draw some blood to be sent to the lab for assessment of the blood cell amount and function, organ function (e.g. liver), level of the enzyme LDH for prognosis prediction.

Prognosis of Lymphoblastic lymphoma (non-Hodgkin’s lymphoma)

Lymphoblastic lymphoma is an aggressive disease but potentially curable. This is particularly so when the B-cell subtype occurs in children.
With current treatment, the overall survival rate after 5 years in children is 80-90%, whereas in adults it is 45-55%.
The disease has a poorer outlook if the following factors are present:

  • Age (>30-40)
  • Increased level of an enzyme called lactate dehydrogenase (LDH)
  • Brain, spinal cord, bone marrow, and extranodal (outside the lymph nodes) involvement
  • Stage IV disease
  • B symptoms (high fever, night sweats, weight loss)
  • Anaemia
  • Partial or late response to treatment

How is Lymphoblastic lymphoma (non-Hodgkin’s lymphoma) Treated?

Chemotherapy produces good response. The regimen for acute lymphoblastic leukaemia is usually used. In adult patients, alternative regimen e.g. the CHOP regimen can be used.
In adults, bone marrow transplant (BMT) may offer increased overall survival, usually in patients in whom the disease comes back for the first time and who have favorable prognostic factors. In lymphoblastic lymphoma, allogeneic transplant (tranplant from another unrelated human being) may produce a higher response rate, but also has a higher rejection rate.

Lymphoblastic lymphoma (non-Hodgkin’s lymphoma) References

  1. Hoelzer D, Gokbuget N. Treatment of lymphoblastic lymphoma in adults. Best Pract Res Clin Haematol 2003; 15(4): 713-28.
  2. Lymphoblastic lymphoma [online]. 2004 [cited 2005 September 1st]. Available from: [URL Link]
  3. Souhaimi et al, editors. Oxford Textbook of Oncology. 2nd ed. Oxford ; New York : Oxford University Press; 2002.
  4. Wang SE, Wun T. Lymphoma, lymphoblastic [online]. 2004 [cited 2005 September 1st]. Available from: URL: http://www.emedicine.com/med/topic1364.htm

 
 

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