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Adult T-Cell Leukemia/Lymphoma

Overview
Subtypes
Treatment Options
Treatments Under Investigation
Clinical Trials
Follow Up
Support
Transportation Assistance Fund
Resources

Overview

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Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Lymphoma occurs when cells of the immune system called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood, or other organs, and form a mass called a tumor. The body has two main types of lymphocytes that can develop into lymphomas: B- lymphocytes (B-cells) and T-lymphocytes (T-cells).

Adult T-cell leukemia/lymphoma (ATLL) is a rare and often aggressive (fast-growing) T-cell lymphoma that can be found in the blood (leukemia), lymph nodes (lymphoma), skin, or multiple areas of the body. ATLL has been linked to infection by the human T-cell lymphotropic virus type 1 (HTLV-1); however, less than five percent of individuals with HTLV-1 will develop ATLL. Currently, physicians have no way of predicting which infected patients will develop ATLL. The HTLV-1 virus is most common in parts of Japan, the Caribbean, and some areas of South and Central America and West Africa. The HTLV-1 virus is believed to be transmitted through sexual contact or exposure to contaminated blood; however, most often it is passed from mother to child through breastfeeding.

 

Subtypes of ATLL

There are four subtypes of ATLL: acute, lymphomatous, chronic, and smoldering. Acute and lymphomatous are fast-growing forms of ATLL, whereas chronic and smoldering are less aggressive:

Acute: In individuals with acute ATLL, symptoms develop rapidly and may include fatigue, skin rash, and enlarged lymph nodes in the neck, underarm, or groin. The hallmarks of acute ATLL are a high white blood cell count often accompanied by an elevated level of calcium in the blood (hypercalcemia), which can cause irregular heart rhythms and severe constipation.

Lymphomatous: This aggressive type of ATLL is found primarily in the lymph nodes but may also cause high white blood cell counts..

Chronic: This slow-growing type of ATLL can result in elevated lymphocytes in the blood, enlarged lymph nodes, skin rash, or fatigue. It can also be found in other areas of the body such as the spleen and liver.

Smouldering: This slow-growing type of ATLL is associated with very mild symptoms, such as a few skin lesions.

Depending on the subtype, diagnosing ATLL may require removing a small sample of tumor tissue or abnormal skin tissue, called a biopsy, and looking at the cells under a microscope. A blood test may also be necessary to measure the white blood cell count and calcium levels. Other tests, such as a bone marrow biopsy, a computed axial tomography (CAT) scan of the chest, abdomen, liver, and spleen, and/or a positron emission tomography (PET) scan may be used to determine where the cancer is located.

Treatment Options

Observation without treatment, called "watch and wait" or "watchful waiting," may be appropriate for some people who have one of the slower-growing subtypes of ATLL with mild or no symptoms, although follow-up monitoring is required. For ATLL affecting the skin, skin-directed therapies (for example, topical steroids or local radiation) may be prescribed.

Because ATLL is such a rare disease, there have not been enough patients enrolled in clinical trials to establish treatment standards in the United States and Europe, especially for the acute and lymphomatous subtypes. As a result, common first-line therapies used to treat ATLL are the same as those used to treat other types of T-cell lymphomas. These include:

• CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone)
• EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide and prednisone)

VCAP-AMP-VECP (vincristine, cyclophosphamide, doxorubicin, prednisone, ranimustine, vindesine, etoposide, and carboplatin) is a regimen piloted in Japan. Other treatments may include zidovudine (Retrovir), also known as AZT, in combination with interferon-alpha to treat the underlying HTLV-1 virus infection. This may be effective in people with the slower-growing forms of ATLL. In some patients, bone marrow transplantation may be appropriate following remission.

Similar to the first-line setting, standard treatment for relapsed (disease returns after treatment) ATLL has not been established. Many regimens used to treat other T-cell lymphomas following relapse are also being used to treat ATLL, including the following::

• GND (gemcitabine, vinorelbine and doxorubicin)
• DHAP (dexamethasone, cytarabine and cisplatin)
• ESHAP (detoposide, methylprednisolone, cytarabine and cisplatin)
• GDP (gemcitabine, dexamethasone and cisplatin)
• ICE (ifosfamide, carboplatin and etoposide)

Treatments Under Investigation

Several new drugs are being studied in clinical trials for ATLL, as single-agent therapy or as part of a combination therapy regimen, including the following:

• Bortezomib (Velcade)

• Lenalidomide (Revlimid)

• Mogamulizumab (Poteligeo; approved in Japan for relapsed/refractory [disease does not respond to treatment] ATLL)

• Pralatrexate (Folotyn)

High-dose chemotherapy followed by allogeneic stem cell transplantation (in which patients receive stem cells from a donor) is also being evaluated as a potential treatment for ATLL patients.

It is critical to remember that today's scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with their physician or with the Lymphoma Research Foundation (LRF) for any treatment updates that may have recently emerged.

Clinical Trials

Clinical trials are crucial for identifying effective drugs and determining optimal doses for patients with lymphoma. Because ATLL is such a rare disease, enrolling enough patients in clinical trials is difficult. The rarity of the disease also means that the most novel treatments are often available only through clinical trials. Patients interested in participating in a clinical trial should talk to their physician or contact the LRF Helpline for an individualized clinical trial search by calling (800) 500-9976 or emailing helpline@lymphoma.org.

Follow Up

Once treatment is completed and ATLL is in remission, physicians will continue to monitor the health and status of each patient during follow-up care. Patients in remission should have regular visits with a physician who is familiar with their medical history and the treatments they have received. Medical tests (such as blood tests and CAT scans) may be required at various times during remission to evaluate the need for additional treatment.

Some treatments can cause long-term effects or late effects, which can vary based on duration and frequency of treatments, age, gender, and the overall health of each patient at the time of treatment. A physician will check for these effects during follow-up care. Visits may become less frequent the longer the disease remains in remission.

Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our factsheet on survivorship issues.

Support

A lymphoma diagnosis often triggers a range of feelings and raises many concerns. In addition, cancer treatment can cause physical discomfort. Support groups and online message boards can help patients connect with other people who have lymphoma. One-to-one peer support programs, such as LRF's Lymphoma Support Network, match lymphoma survivors (or caregivers) with volunteers who have gone through similar experiences.

LRF T-Cell Lymphoma Transportation Assistance Fund 
The Lymphoma Research Foundations (LRF) T-Cell Lymphoma Transportation Assistance Fund grant program provides financial assistance to T-cell lymphoma patients who are uninsured or have adequate medical insurance but struggle to pay for transportation costs (e.g., mileage, parking, tolls, gas, train tickets, etc.) to get to and from treatment. To see a list of additional organizations providing financial assistance, please see LRF's Financial Assistance webpage.

Resources

LRF offers a wide range of resources that address treatment options, the latest research advances, and ways to cope with all aspects of lymphoma. LRF also provides many educational activities, from in-person meetings to teleconferences and webcasts. For more information about any of these resources, visit www.lymphoma.org or contact the Helpline at (800) 500-9976 or helpline@lymphoma.org.

 

Webcasts/Podcasts
•  T-Cell Lymphomas: An Overview NEW! Andrei Shustov, MD (2014) View Webcast

Teleconferences
In-person Educational Programs
Support Programs

View or order the following publications from our booklets/factsheets webpage:
Adult T-Cell Leukemia/Lymphoma Fact Sheet NEW!
Understanding Non-Hodgkin Lymphoma: A Guide for Patients, Survivors and Loved Ones

 

Lymphoma Helpline

Professional staff members are available to answer your questions and provide individual support to you and your loved ones. Contact our Helpline, available Monday through Friday from 8:00am - 5:00pm Pacific Standard Time (PST). Call (800) 500-9976 or e-mail.