What is T-Cell Lymphoblastic Lymphoma?

T-Cell Lymphoblastic Lymphoma (T-LBL) is a very rare subtype of non-Hodgkin's lymphoma. It tends to develop in young patients—meaning those who aren't much older than around 35 (although it can strike older folks).

T-LBL only affects a very small percentage of the population. There are roughly 70,000 new lymphoma diagnoses in the United States each year, and no more than 2% are lymphoblastic lymphoma. That comes out to around 1,400 new cases each year.

Of that figure, about 1,200 will develop the T-cell phenotype of the disease, while the rest will develop the B-cell phenotype.

It has long been understood that the disease shares a lot In common with acute lymphoblastic leukemia (ALL), which also strikes young people moreso than older people. Only recently, however, has the World Health Organization addressed the issue and acknowledged that in fact they are the same clinical entity. Therefore technically, the term T-LBL is no longer to be used; rather the disease should be referred to as precursor T-cell lymphoblastic leukemia/lymphoma.

For the sake of clarity, we'll stick with T-LBL here.

What happens in T-Cell Lymphoblastic Lymphoma?

No one can say what exactly happens to cause any cancer, although changes in the cell DNA via exposure to some external source or an internal genetic one are likely at work. In T-LBL, the consequence is that the bone marrow is producing blood cells that are supposed to mature into B or T lymphocytes. Because of their acquired defect, however, these blood cells remain in the immature stage, called lymphoblasts.

They leave the bone marrow and go to lymph nodes or lymphatic organs or extra-lymphatic sites, and do what all other cancers do: occupy space, hog resources, and make it very difficult for that part of the body to do the job it is required to do. The patient's immune system can be compromised, allowing for infections to run riot in the body or they can form bulky masses and get in the way of organ function.

Diagnosis for T-Cell Lymphoblastic Lymphoma

The first thing people with this disease notice is typically a swollen, painless, rubbery-feeling lymph node, often in their neck region.

It's not uncommon for patients to have chest pain as well, which would be symptomatic of a so-called mediastinal mass—a tumor of sorts that develops in the chest, and can cause pain if it presses on nerve endings or can cause a patient to fatigue easily due to compromised lung capacity.

Treatment for T-Cell Lymphoblastic Lymphoma

T-LBL is regarded as a very aggressive cancer. While a number of options exist to treat it—including a bone marrow transplant and radiotherapy—the treatment modality that sees the highest cure rate is that of combination chemotherapy regimens.

The same regimens that are used against ALL are most often used in patients with T-LBL. As with many cancers, early-stage disease is more treatable than advanced-stage. The regimens known as CHOP and Hyper-CVAD have achieved remission in as many as 70% of patients treated but the disease is more difficult to treat in older patients (older compared to pediatric ones, that is). Furthermore, relapse is not uncommon, with as many as half of all adult patients relapsing.

Photo: Pexels

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