Lymphoblastic lymphoma in children and teenagers

Lymphoblastic lymphoma is a type of non-Hodgkin’s lymphoma that particularly affects children and adolescents. Lymphoblastic lymphoma affects around 25-30% of non-Hodgkin’s lymphomas in paediatric patients.

The cancer cells of lymphoblastic lymphoma are very young lymphocytes called lymphoblasts. The affected cells are the same as in acute lymphoblastic leukaemia in children. In fact, if more than 25% of bone marrow is composed of lymphoblasts, the disease is classified and treated as acute lymphoblastic leukaemia as opposed to lymphoma. 80% presents as acute leukaemia and the rest affects the lymph nodes, bones and skin. 

The majority of lymphoblastic lymphomas are developed in the T-cells and are called precursor T-cell lymphoblastic lymphomas. T-cell lymphoblastic lymphomas are most common in adolescents, young patients and males.

A small number of lymphoblastic lymphomas (10%) are developed in the B cells and are called precursor B-cell lymphoblastic lymphomas.

Research in this field is important and the genetic changes that occur in the affected cells are becoming known. However, the triggers or factors that have given rise to the lymphoma are unknown.

These begin when a white blood cell that fights infections, known as a lymphocyte, suffers a genetic mutation. The mutation tells the cell to multiply quickly, creating many diseased lymphocytes that continue to multiply. This produces too many diseased and ineffective lymphocytes in the lymph nodes and causes the lymph nodes, spleen and liver to swell.

Leukaemia and lymphomas, like other cancers, are not contagious. See section Leukaemia, bone marrow and blood cells.

T-cell lymphoblastic lymphoma often originates in the thymus and forms a mass behind the breastbone and in front of the windpipe. This can lead to breathing problems, which can be the first symptom of lymphoma in many patients. It can grow very quickly and therefore needs to be diagnosed and treated quickly.

70% of patients may also present with affected and inflamed lymph nodes, especially in the clavicle, cervical spine and armpits. The bone marrow and nervous system may also be affected, although this is present in fewer patients.

Not all children or adolescents with lymphoma present with the same symptoms. Other symptoms may include:

• Lumps in the neck, armpits, stomach, testicles or groin (sometimes called “swollen glands”)
• Fever
• Night sweats
• Fatigue
• Unexplained weight loss and/or loss of appetite
• Itchy skin
• Shortness of breath
• Cough
• Chest pain
• Swollen abdomen

Generally, if a child presents the characteristic symptoms, a blood test will be undertaken. The doctor will also request a biopsy of the swollen lymph node in order to establish a diagnosis. During the biopsy, all or part of the abnormal lymph node will be extracted and observed under a microscope in a laboratory

The diagnosis is confirmed by a bone marrow puncture (iliac crests) and analysis of the cells obtained.

Lymphoblastic lymphoma grows rapidly, but its prognosis has changed significantlysince patients have been treated with guidelines similar to those used in acute lymphoblastic leukaemia, with central nervous system prophylaxis. 

Treatment is based on intensive chemotherapy and radiotherapy for central nervous system prophylaxis. Bone marrow transplant may be used in cases of relapsed disease.

Approximately 80% of children who undergo treatment are free of disease five years after diagnosis.

• Childhood Lymphoblastic Lymphoma. Dana Farber Cancer Institute

• Types of Non-Hodgkin Lymphoma in Children. American Cancer Society

• Childhood Non-Hodgkin Lymphoma Treatment. National Cancer Institute

• Lymphoma in Children. St Jude Children’s Research Hospital

All these organisations are external to the Josep Carreras Foundation.

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