Leukemia - Chronic T-Cell Lymphocytic

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What is Leukemia - Chronic T-Cell Lymphocytic?

Leukemia is a cancer of the blood cells. Leukemia begins when normal blood cells change and grow uncontrollably. Blood cells are made in the bone marrow, the spongy tissue inside the larger bones in the body. There are different types of blood cells, including red blood cells that carry oxygen throughout the body, white blood cells that fight infection, and platelets that help the blood to clot. Changes in the bone marrow cells can cause too many or too few of certain blood cells.

Types of leukemia are named after the specific blood cell that becomes cancerous, such as the lymphoid cells (a type of white blood cell) or the myeloid cells (bone marrow cells that turn into cells that fight bacterial infections). There are four main types of leukemia in adults:

Acute lymphocytic leukemia (ALL)

Chronic lymphocytic leukemia (CLL)

Acute myeloid leukemia (AML)

Chronic myeloid leukemia (CML)

About T-cell leukemia

There are also less common types of leukemia, but they are generally subcategories of one of the four main categories listed above. This section focuses on different types of chronic T-cell lymphocytic leukemia, a subtype of chronic lymphocytic leukemia (CLL). A T cell is a type of white blood cell that directly helps body’s immune system fight infection. The subtypes of T-cell leukemia include:

Large granular lymphocytic leukemia (LGLL). LGLL is a slow-growing T-cell leukemia and is more common in women than in men. The cause of LGLL is unknown, although about 30% of people with LGLL also have rheumatoid arthritis (a chronic disease causing swelling in the joints of the hands, feet, wrists, knees, hips, or shoulders).

T-cell prolymphocytic leukemia (T-PLL). T-PLL is an aggressive subtype of CLL. It is more common in older men, but women may also develop T-PLL. It can affect the skin, but in a different way than Sezary syndrome (see below).

Adult T-cell leukemia/lymphoma (ATLL). ATLL has four subtypes. Depending on the different features, it is subclassified as smoldering, chronic, acute, or adult T-cell lymphoma (cancer of the lymph system). The acute and the adult T-cell lymphoma subtypes grow quickly. ATLL is caused by a retrovirus called the human T-cell leukemia virus (HTLV1). 

Sezary syndrome. Sezary syndrome is a form of mycosis fungoides, a T-cell lymphoma that occurs in the skin. Sezary syndrome is usually slow-growing and takes years to develop from mycosis fungoides, which is located only on the skin. Sezary syndrome is generally diagnosed when large numbers of the lymphoma cells are found in the blood, often together with reddening of the skin.

Symptoms and Signs

People with T-cell leukemia may experience the following symptoms or signs. Sometimes, people with T-cell leukemia do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

Recurrent infections from low numbers of neutrophils (infection-fighting white blood cells)

Bleeding or bruising easily

Unexplained tiredness (fatigue)

Persistent, unexplained abdominal pain on the left side from a swollen spleen (LGLL, T-PLL)

Swollen lymph nodes (T-PLL, ATLL)

Rash or skin lesions (T-PLL, ATLL, Sezary syndrome)

Frequent urination and/or constipation from high levels of calcium in the blood, called hypercalcemia (ATLL).

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

If leukemia is diagnosed, relieving symptoms and side effects remains an important part of care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

Risk Factors

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

In general, leukemia can be caused by a genetic mutation (change) that is inherited (passed from generation to generation within a family) or environmental factors, such as smoking or exposure to chemicals or radiation. However, most often the cause of leukemia is not known.  

The following risk factor can increase a person’s chance of developing T-cell leukemia:

Race/Ethnicity. ATLL is more common in people of Japanese, West Indian, and African American descent.

Diagnosis

Doctors use many blood and bone marrow tests to diagnose leukemia and to find out how much it may have spread. Although a patient’s signs and symptoms may cause a doctor to suspect leukemia, it is diagnosed only by blood and/or bone marrow tests. Some tests may also determine which treatments may be the most effective. Your doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of cancer suspected

Severity of symptoms

Previous test results

In addition to a physical examination, the following tests may be used to diagnose T-cell leukemia:

Blood tests. The diagnosis of T-cell leukemia begins with a blood test called a complete blood count (CBC) that measures the numbers of different types of cells in the blood. If the blood contains many white blood cells, T-cell leukemia may be suspected.

Bone marrow biopsy and aspiration. These two procedures are similar and often done at the same time. Bone marrow has both a solid and a liquid part. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. An aspiration removes a sample of fluid with a needle. The sample(s) are then analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). A common site for a bone marrow biopsy and aspiration is the pelvic bone, which is located in the lower back by the hip. The skin in that area is usually numbed with medication beforehand, and other types of anesthesia (medication to block awareness of pain) may be used.

Molecular testing. Your doctor may recommend running laboratory tests on the leukemia cells to identify specific genes, proteins, and other factors unique to the disease. Immunophenotyping is the examination of antigens, a specific type of protein, on the surface of the leukemia cells. Immunophenotyping allows the doctor to confirm the exact type of leukemia. Cytogenetics is the examination of the leukemia cells for chromosome (long strands of genes) abnormalities. It helps doctors confirm the diagnosis and may help determine the person’s prognosis (chance of recovery). Results of these tests will also help decide whether your treatment options include a type of treatment called targeted therapy .

Skin biopsy.  A skin biopsy is a procedure in which a sample of skin tissue is removed and examined under a microscope to look for T cells in the skin.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities, including swollen lymph nodes or a swollen spleen. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

Stages

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis. There are different stage descriptions for different types of cancer.