Lymphoblastic lymphoma

Overview

Lymphoblastic lymphoma is a very rare form of non-Hodgkin lymphoma in adults, accounting for less than 3 in 100 cases overall. However, it accounts for more than 1 in 3 of all cases occurring in children and teenagers. It more commonly affects males than females. It may develop from either T- or B-lymphocytes, but in 4 out of 5 cases the T-lymphocytes are affected.

Lymphoblastic lymphoma is very similar to the condition acute lymphoblastic leukaemia (ALL). In ALL, the abnormal lymphocytes are mainly in the blood and bone marrow, whereas in lymphoblastic lymphoma they are generally in the lymph nodes or thymus gland. The two conditions are often treated in very similar ways.

Symptoms

The first sign of the condition is often a painless swelling in the neck, caused by enlarged lymph nodes. The thymus gland, or lymph nodes deep within the chest (the mediastinal lymph nodes), are also often affected. Sometimes more than one group of nodes is affected. The lymphoma may spread to involve various organs in the body such as the liver, spleen, bone marrow, skin, the testicles and the brain. Some people experience a loss of appetite| and tiredness|.

• Other symptoms may include:
• night sweats
• unexplained high temperatures
• weight loss

Causes

The causes of lymphoblastic lymphoma are unknown. Lymphoblastic lymphoma, like other cancers, is not infectious and cannot be passed on to other people.

Prevention

Not known.

Diagnosis

The diagnosis is made by removing some of the affected tissue and examining the cells under a microscope (this is called a biopsy). You will be referred to a surgeon for this procedure. It is a very small operation and may be done under local or general anaesthetic.

Additional tests – including blood tests, x‑rays, scans, a lumbar puncture| lumbar puncture to examine the cerebrospinal fluid (which protects your brain and spinal cord), and bone marrow samples – are then used to get more information about the type of lymphoma and how far it has spread in the body. This information is used to help decide which treatment is most appropriate for you.

Prognosis

Lymphoblastic lymphoma is a high-grade lymphoma. This means that it is fast-growing and needs prompt treatment.

Staging:
The stage of non-Hodgkin lymphoma describes how many groups of lymph nodes are affected, where they are in the body, and whether other organs such as the bone marrow or liver, are affected.

• Stage 1 The lymphoma is only in one group of lymph nodes, in one particular area of the body.
• Stage 2 More than one group of lymph nodes is involved, but all the involved nodes are contained within either the upper half or the lower half of the body. The upper half of the body is above the diaphragm (the sheet of muscle underneath the lungs), and the lower half is below it.
• Stage 3 Lymphoma is present in lymph nodes in both the upper and lower parts of the body (ie in lymph nodes both above and below the diaphragm). The spleen is considered as a lymph node in this staging system.
• Stage 4 The lymphoma has spread beyond lymph nodes to other lymphatic organs – for example, to sites such as the bone marrow, liver or lungs.
• The stage usually includes the letter A or B, which describes whether the B symptoms are present or not (eg stage 2B). Sometimes the lymphoma can start in areas outside the lymph nodes, and this is represented by the letter E, which stands for extranodal (eg stage 3AE).

Treatment

Chemotherapy:
The type of chemotherapy used is similar to that used for acute lymphoblastic leukaemia. Lymphoblastic lymphoma is usually treated more intensively than other types of lymphoma. In the initial phase of treatment, called the induction phase, multiple chemotherapy drugs are given weekly, usually for 10 weeks. Following this early phase of treatment, high-dose chemotherapy may be considered (see below), or else less intense maintenance doses of chemotherapy are given over several months.

As there is a risk of this type of lymphoma spreading to the cerebrospinal fluid, chemotherapy drugs are also usually injected into the spine (intrathecal chemotherapy|). This treatment is used if lymphoma cells have been detected here, or as a preventative measure against future spread to the cerebrospinal fluid.

Radiotherapy:
Sometimes chemotherapy is combined with radiotherapy to the brain and spine, if lymphoma cells are present in the cerebrospinal fluid.

High-dose treatment with stem cell support:
High-dose chemotherapy with bone marrow or stem cell infusion may be considered for patients with lymphoblastic lymphoma after the initial chemotherapy has been completed. This type of treatment involves very intensive chemotherapy and sometimes radiotherapy.

As the side effects can be severe, some types of high-dose treatment are not given to people over the age of 45–50 and others can be given to people of up to 65 years if they are fit enough. This is because the intensity of the treatment increases the risks of serious side effects for people older than this.

Approved therapies:
Nelarabine (Arranon) - FDA-approved indication: Treatment of patients with T-cell acute lymphoblastic leukemia and T-cell lymphoblastic lymphoma whose disease has not responded to or has relapsed following treatment with at least two chemotherapy regimens 

Resources

Refer to Research Publications.