What is T-cell Lymphoma?
Lymphoma is a cancer of the lymph glands or tissue and is broadly divided into Hodgkin’s or non-Hodgkin’s lymphoma (NHL). T-cell lymphoblastic lymphoma (T-LBL) is a NHL that is usually found in children and adolescents. It grows quickly and presents as a mass in the chest, and patients may have bone marrow involvement and circulating T-lymphoblasts and are treated as T-cell ALL. It is estimated that over 100 cases of childhood NHL are diagnosed per year in Gujarat, and around 15-20 of those are T-LBL.
What is the cause of T-cell lymphoma?
We don’t know what causes T-LBL but it is not inherited, nor can it spread to other members in the family. There is nothing you did to cause this.
How are patients diagnosed?
Because there is often a mass in the chest compressing the airway, it is important to make an accurate diagnosis quickly, and treat aggressively with life-saving chemotherapy to achieve the best outcome. Steroids and low dose radiation may be needed to shrink the chest mass. Biopsy of a piece of the tumor is essential to diagnosing T-LBL and can be difficult because of the location of the tumor, needing CT guidance. Sometimes fluid from the chest can provide the cancer cells needed, or bone marrow sample.
A careful history and physical exam, in combination with review of the tumor slides by an experienced pathologist, with special stains, and characteristic changes in blood chemistry LDH, uric acid, potassium and phosphate usually allows a diagnosis to be made quickly. T-LBL lymphoma cells resemble T-lymphoblasts with surface markers for T-cells such as CD3, CD4 and CD8. Cytogenetics do not play much of a role in risk stratification.
How are patients staged?
Staging tells us how far the lymphoma has spread, and how aggressive treatment needs to be. Tests include total-body imaging and tissue sampling such as:
- CT, and/or MRI scans
- PET scans (although this does not change treatment in children so use is uncertain)
- Bone marrow biopsy and aspiration to distinguish T-LBL from acute leukemia
- Lumbar puncture to look for evidence of brain and spinal cord (CNS) involvement, which is rare. and but Bone Marrow (BM) involvement is common.