Adult T-cell leukemia/lymphoma (ATLL) is a group of cancers that develops from a type of white blood cell called T lymphocytes, or T cells. ATLL is classified as leukemia if the cancer forms in the blood or as lymphoma if it forms in the lymph nodes. ATLL is considered one form of non-Hodgkin lymphoma (NHL), one of the two main broad categories of lymphoma. More specifically, ATLL falls under a subtype of NHL called peripheral T-cell lymphoma, meaning the cancer forms outside of the bone marrow.

ATLL is overall a very rare T-cell malignancy (cancer). ATLL is not often diagnosed in the United States and is more common in regions including Japan, South and Central America, the Caribbean, and Africa.

Types of Adult T-Cell Leukemia/Lymphoma

There are four subtypes of ATLL, and each disease has its own set of unique characteristics.

• Acute ATLL — This is an aggressive (fast-growing) cancer with a leukemic presentation, meaning the cancer cells are primarily in the blood.
• Lymphomatous ATLL — This is another aggressive cancer, but it’s located in the lymph nodes and other lymphatic organs, rather than the blood.
• Chronic ATLL — This is a low-grade, or slow-growing, cancer. It develops gradually over many years.
• Smoldering ATLL — This is another low-grade disease, with the term “smoldering” meaning it can persist for a long time without causing any symptoms or problems. A person may notice mild skin lesions or rash.

Cause of Adult T-Cell Leukemia/Lymphoma

ATLL is strongly associated with human T-cell lymphotropic virus type 1 (HTLV-1) infection. HTLV-1 infection is most frequently seen in certain regions of Japan, Central and South America, the Caribbean, and Africa. Consequently, ATLL has a higher prevalence in these regions relative to the United States.

HTLV-1 is thought to cause ATLL by infecting T-cells and inserting the viral genome into the DNA of the T-cells. This can lead to an abnormal multiplication of T-cells, resulting in cancer.

Signs and Symptoms of Adult T-Cell Leukemia/Lymphoma

The signs and symptoms caused by ATLL vary depending on the subtype. (Symptoms are what a person experiences, such as a headache or nausea. Signs are measurable and observable, such as a fever or high blood pressure.) Common signs and symptoms caused across different ATLL subtypes include:

• Bloating or abdominal pain due to a swollen liver or spleen
• Swollen lymph nodes
• An itchy skin rash

The aggressive subtypes (acute and lymphomatous ATLL) often cause the types of symptoms as many other forms of lymphoma. These are called B symptoms and include fevers, night sweats, and unexplained weight loss. Other signs and symptoms of acute and lymphomatous ATLL can include:

• Increased rate of infections
• Watery diarrhea, constipation, or fatigue due to high levels of calcium in the blood

The low-grade subtypes of ATLL (chronic and smoldering) cause overall fewer symptoms. Other than a skin rash in some cases, smoldering ATLL often doesn’t produce any symptoms. In addition to the common signs and symptoms listed above, chronic ATLL may cause high levels of white blood cells in the blood.

How Is Adult T-Cell Leukemia/Lymphoma Diagnosed?

ATLL can have various clinical features (i.e., signs and symptoms) that must be investigated by a doctor using different tests to make a diagnosis. The doctor may use blood tests, biopsies, and imaging tests.

Blood Test

Performing a blood test is one of the first steps in making a diagnosis. A blood test can detect the presence of the HTLV-1 antibody, a protein made by the immune system in a person with an HTLV-1 infection. The presence of the HTLV-1 antibody is the primary factor used to support an ATLL diagnosis. A blood test can also detect cancer cells in the blood — a key characteristic of the acute and chronic subtypes of ATLL — as well as elevated levels of calcium and white blood cells.

Lymph Node Biopsy

A lymph node biopsy involves surgically removing a lymph node and analyzing it for the presence of ATLL cancer cells. This test is particularly important for diagnosing lymphomatous ATLL.

Imaging Technologies

CT scans, as well as a combination of positron emission tomography (PET) and CT called PET-CT, are used to image the tissues within the body. CT or PET-CT can look for hepatosplenomegaly (swelling of the spleen and liver), which is a common feature of ATLL.

Treatment Options for Adult T-Cell Leukemia/Lymphoma

There are different approaches to treat ATLL. The specific prescribed treatment depends on the ATLL subtype.

Active Surveillance

Also referred to as the watch-and-wait approach, this option is best suited for low-grade subtypes of ATLL that cause no symptoms, or minor ones. A doctor can monitor disease progression through regular follow-up visits. This approach postpones therapy and allows doctors to act quickly in the event the cancer starts to cause problems or grow more quickly.

Chemotherapy

For aggressive forms of ATLL, chemotherapy is the typical initial treatment approach. There are several different chemotherapeutic drugs and they are often administered in certain combinations, including:

• CHOP — Cyclophosphamide (sold as Cytoxan), doxorubicin (also known as hydroxydaunorubicin and sold as Adriamycin), vincristine (formerly sold as Oncovin), and prednisone
• CHEOP — CHOP plus VP-16 (etoposide)
• Dose-adjusted EPOCH — Etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin; during treatment cycles, doses of some drugs may be increased or decreased to reduce side effects
• Hyper-CVAD — Cyclophosphamide, vincristine, doxorubicin, and dexamethasone; the term “hyper” stands for “hyperfractionated,” referring to how it's administered in small doses

Clinical Trials

Due to the lack of targeted treatments for aggressive ATLL, people with the condition may be encouraged to take part in a clinical trial. Several existing drugs are already being explored as possible treatments for ATLL, including Velcade (bortezomib) and Treanda (bendamustine), which are currently approved to treat other forms of non-Hodgkin lymphoma. Another drug, Poteligeo (mogamulizumab), is a monoclonal antibody drug, meaning it can specifically recognize certain cell types. It is under investigation and has already been approved by the U.S. Food and Drug Administration for mycosis fungoides and Sezary syndrome.

Allogeneic hematopoietic stem cell transplantation (bone marrow transplant) is also being explored as a potential treatment option for people who are responsive to chemotherapy. This approach involves replacing the cells in a person’s bone marrow with those of a healthy donor in order to increase the tolerance for higher doses of chemotherapy.

Outlook for Adult T-Cell Leukemia/Lymphoma

ATLL has an overall poor prognosis (outlook). Aggressive subtypes (acute and lymphomatous) have a typical survival of less than one year from diagnosis and four-year survival rates of 11 percent and 16 percent, respectively. Low-grade ATLL (chronic and smoldering types) have a more favorable outlook with respective four-year survival rates around 36 percent and 52 percent. Additional research and participation in clinical trials will hopefully lead to the discovery of more effective treatments for ATLL and better survival.

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