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T-Cell Acute Lymphoblastic Leukemia: An Overview

Posted on June 30, 2021
Medically reviewed by
Todd Gersten, M.D.
Article written by
Maureen McNulty

T-cell acute lymphoblastic leukemia (T-ALL) is a fast-growing type of leukemia and a type of acute lymphoblastic leukemia (ALL). Also called T-cell acute lymphocytic leukemia or T-lymphoblastic leukemia, the condition develops from immature lymphocytes, a type of white blood cell.

About 5,700 people are diagnosed with ALL each year in the United States. ALL is the most common type of cancer in children, but it can occur in adults as well. Of all cases of ALL, about 10 percent to 25 percent are T-cell ALL.

What Is T-Cell ALL?

Acute lymphoblastic leukemia develops when gene changes cause blood cells to grow out of control. Leukemias are divided into categories based on how fast they grow and which type of blood cell is affected. “Acute” means that the leukemia grows quickly, and “lymphoblastic” means that the leukemia develops from lymphoblasts. Lymphoblasts are immature lymphocytes — white blood cells that play different roles in helping the body fight infections.

ALL is further divided into subtypes depending on which type of lymphocyte is involved. The types of lymphocytes are B cells, T cells, and natural killer (NK) cells. T-ALL develops from T cells, which are responsible for activating the immune system and destroying other cells when they become infected.

Risk Factors for T-Cell ALL

Doctors don’t usually know why any one case of leukemia develops. However, certain risk factors may increase a person’s chances of developing ALL. Risk factors for ALL include:

  • Older age
  • Exposure to high amounts of radiation
  • Exposure to certain chemicals, such as benzene
  • Having certain genetic disorders, such as Down syndrome, Fanconi anemia, and Bloom syndrome
  • Having an identical twin who had ALL in childhood

Symptoms of T-Cell Acute Lymphoblastic Leukemia

Different subtypes of ALL tend to lead to the same sets of symptoms. These symptoms may include:

  • Bone or joint pain
  • Night sweats
  • Loss of appetite
  • Weight loss
  • Feelings of pain upon breathing
  • Splenomegaly (enlarged spleen) or hepatomegaly (enlarged liver), which may lead to pain or feelings of fullness in the abdomen
  • Swollen lymph nodes, which feel like hard lumps in the armpits, neck, or groin
  • Anemia (low levels of red blood cells), leading to tiredness, dizziness, and shortness of breath
  • Thrombocytopenia (low platelet counts), leading to bleeding problems and bruising
  • Leukopenia (low levels of healthy white blood cells), possibly leading to fevers and infections

T-cell ALL also causes additional symptoms. Some people with this condition have an enlarged thymus (an organ behind the breastbone). An enlarged thymus may lead to breathing problems, coughing, or headaches.

By the time people are diagnosed with T-ALL, the leukemia cells have often spread to their central nervous system (CNS) — which includes the brain and spinal cord. When the brain is affected, T-cell ALL may cause headaches, blurry vision, and seizures.

Diagnosis of T-Cell ALL

If your doctor thinks you may have leukemia, they may prescribe tests to confirm a diagnosis. Blood tests are an important part of diagnosing leukemia. A test called a complete blood count measures the levels of each type of blood cell. Additional blood tests can identify possible problems with organs such as the kidneys or liver.

Most people with T-cell ALL need to have bone marrow tests. Bone marrow, the tissue found inside the bones, produces new blood cells. People with leukemia generally have many cancerous cells in their bone marrow. Testing the bone marrow helps a doctor diagnose blood cancer and identify which type of leukemia a person has. Bone marrow tests usually have two parts: During a bone marrow biopsy, a small sample of cells is removed from the bone. During a bone marrow aspiration, a sample of fluid is taken.

Cells from bone marrow and blood samples may be used for further laboratory testing. This may include cytogenetic tests to look at gene changes. A doctor may also use flow cytometry testing to look at a cell’s immunophenotype (proteins found on a cell’s surface). The information from these tests helps doctors determine effective treatments and estimate the prognosis (outlook) for a person’s particular condition.

Many people with ALL also need to have imaging tests. These can show if cancer cells have spread to certain organs and determine whether lymph nodes around the body are enlarged. Imaging tests might include:

  • X-rays
  • Ultrasounds
  • CT scans
  • MRI scans

Because T-ALL often spreads to the CNS, people with this condition will often require a lumbar puncture. During this procedure, doctors take a sample of fluid from the area around the spinal cord. They will then check this fluid for leukemia cells.

T-Cell ALL Treatment

T-cell ALL is often treated in the same way as ALL that develops from B cells. However, people with T-ALL may be given more aggressive treatments.

When recommending a treatment plan, doctors will estimate a person’s risk level: high, standard, or low. This information helps doctors predict a person’s prognosis. People with T-cell ALL are usually classified as having a high-risk disease.

Chemotherapy

T-cell ALL is often treated with a combination of chemotherapy and steroid drugs. This treatment usually kills most of the leukemia cells. Chemotherapy is often given in multiple different phases: initial therapy, consolidation therapy, and maintenance therapy. During each phase, a person may receive different drugs at different doses. Initial and consolidation therapy phases may be more aggressive. Maintenance therapy often consists of low doses of chemotherapy drugs taken for two to three years.

Chemotherapy drugs that may be used to treat T-cell ALL include:

Pediatric (childhood) T-cell ALL is typically treated with multiple different high-dose chemotherapy drugs. These medications may include:

  • Asparaginase
  • Daunorubicin
  • Dexamethasone
  • Vincristine

T-ALL is often treated with therapies that can prevent or treat the spread of leukemic cells to the central nervous system. These treatments often include intrathecal chemotherapy, in which chemotherapy drugs are injected into the fluid that surrounds the brain and spinal cord.

Targeted Therapy

Targeted therapy drugs are sometimes given along with chemotherapy. These medications recognize and attack specific cancer genes or proteins. Most targeted therapies developed for ALL are used in B-cell ALL and won’t be effective against T-cell ALL. However, there are some options that may help certain people with T-ALL.

Arranon (nelarabine) is a targeted therapy drug approved by the U.S. Food and Drug Administration to treat T-cell ALL that is refractory (resistant to other treatments) or relapsed (came back after being treated with induction therapy). Nelarabine can help both children and adults have better outcomes. Now, researchers are also studying whether this drug may be helpful as a part of induction therapy for people undergoing treatment for the first time.

Some people with ALL have a gene change called the Philadelphia chromosome. Leukemia cells with this change can be targeted with tyrosine kinase inhibitors, a type of targeted therapy. These medications include Gleevec (imatinib), Sprycel (dasatinib), and Tasigna (nilotinib). However, this gene change is rare in T-cell subtypes of ALL.

Stem Cell Transplant

If chemotherapy does not get rid of a person’s T-ALL cells, doctors may recommend stem cell transplantation. During this procedure, a person’s stem cells are replaced. Stem cells are the cells in the bone marrow that make new blood cells. A stem cell transplant consists of aggressive chemotherapy or radiation therapy treatments to kill as many leukemia cells as possible and make room in the bone marrow. This treatment is followed by an infusion of new stem cells, which replace damaged blood cells.

A stem cell transplant can cause serious side effects — such as graft-versus-host disease — so it is often not recommended for people who are older or who have other health problems.

Radiation Therapy

Radiation therapy may be used along with stem cell transplants. T-cell ALL is also sometimes treated with radiation treatments to the brain to help treat leukemia cells that have spread there.

Prognosis for T-Cell ALL

Overall, about 85 percent of people with T-ALL live for five years or more after being diagnosed. Children with T-ALL are likely to have better outcomes than adults. T-cell ALL can be cured (meaning all signs of leukemia disappear and don’t return) in about 4 out of 5 children. However, fewer than half of adult T-ALL cases can be cured.

One of the main factors that affects a person’s outlook is how well induction treatments work. If signs of cancer are gone at the end of induction therapy or at the end of consolidation therapy, a person usually has a good prognosis.

T-cell ALL is more likely to relapse than B-cell ALL. If T-ALL relapses, it is more difficult to treat. Therapies don’t often work as well, and people have an increased risk of having a worse outcome. People who have had multiple relapses may be able to try different chemotherapy drugs or participate in clinical trials.

Talk With Others Who Understand

MyLeukemiaTeam is the social network for people with leukemia and their loved ones. On MyLeukemiaTeam, more than 8,600 members come together to ask questions, give advice, and share their stories with others who understand life with leukemia.

Are you living with T-cell acute lymphoblastic leukemia? Share your experiences in the comments below, or start a conversation by posting on MyLeukemiaTeam.

References
  1. Key Statistics for Acute Lymphocytic Leukemia (ALL) — American Cancer Society
  2. Pediatric T-Cell Lymphoblastic Leukemia — Atlas of Genetics and Cytogenetics in Oncology and Haematology
  3. T-Cell Acute Lymphoblastic Leukemia — National Cancer Institute
  4. Types of Leukemia — Memorial Sloan Kettering Cancer Center
  5. Leukemia — Acute Lymphocytic — ALL: Introduction — American Society of Clinical Oncology
  6. Risk Factors for Acute Lymphoblastic Leukemia (ALL) — American Cancer Society
  7. Adult T-Cell Lymphoma — Leukaemia Foundation
  8. Acute Lymphoblastic Leukemia — Signs & Symptoms — Leukemia & Lymphoma Society
  9. Signs and Symptoms of Acute Lymphocytic Leukemia (ALL) — American Cancer Society
  10. Acute Lymphoblastic Leukaemia (ALL) — Leukaemia Foundation
  11. Overview — Acute Lymphoblastic Leukaemia — National Health Service
  12. Leukemia — Acute Lymphocytic — ALL: Diagnosis — American Society of Clinical Oncology
  13. T-Cell Acute Lymphoblastic Leukaemia — Leukaemia Care
  14. T-cell Acute Lymphoblastic Leukemia: A Roadmap to Targeted Therapies — Blood Cancer Discovery
  15. Acute Lymphocytic Leukemia — Mayo Clinic
  16. Leukemia Acute Lymphocytic ALL: Treatment Options — Cancer.Net
  17. Childhood Acute Lymphoblastic Leukemia Treatment (PDQ) — Patient Version — National Cancer Institute
  18. Evolving Therapy of Adult Acute Lymphoblastic Leukemia: State-of-the-Art Treatment and Future Directions — Journal of Hematology & Oncology
  19. FDA Drug Approval Summary: Nelarabine (Arranon) for the Treatment of T‐Cell Lymphoblastic Leukemia/Lymphoma — The Oncologist
  20. Philadelphia Chromosome Positive Pre-T Cell Acute Lymphoblastic Leukemia: A Rare Case Report and Short Review — Indian Journal of Hematology & Blood Transfusion
  21. Adult Acute Lymphoblastic Leukemia Treatment (PDQ) — Patient Version — National Cancer Institute
  22. T-Cell Acute Lymphoblastic Leukemia — The American Society of Hematology Education Program
  23. Graft-Versus-Host Disease — Leukemia & Lymphoma Society
Todd Gersten, M.D. is a hematologist-oncologist at the Florida Cancer Specialists & Research Institute in Wellington, Florida. Review provided by VeriMed Healthcare Network. Learn more about him here.
Maureen McNulty studied molecular genetics and English at Ohio State University. Learn more about her here.

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