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Anaplastic Large Cell Lymphoma

Overview
Treatment Options
Treatments Under Investigation
Clinical Trials
Follow-up
Support
Resources

Overview

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Focus On ALCL

Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma 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).

Anaplastic large cell lymphoma (ALCL) is a rare type of NHL, but one of the more common subtypes of T-cell lymphoma. ALCL comprises about three percent of all NHLs and 10 percent to 30 percent of all NHLs in children. Initial symptoms of ALCL can include painless swelling of lymph nodes, rapid weight loss, and tiredness.

ALCL can initially appear either in the skin, in lymph nodes, or in organs throughout the body. ALCL that appears in the skin is called primary cutaneous ALCL, which has a less aggressive disease course than the systemic types. The characteristic features of primary cutaneous ALCL include the appearance of solitary or multiple raised red skin lesions that do not go away, have a tendency to ulcerate, and may itch. These ALCL lesions are tumors, and they can appear on any part of the body, often grow very slowly, and may be present for a long time before being diagnosed. Approximately 10 percent of the time, primary cutaneous ALCL extends beyond the skin to lymph nodes or organs. If this occurs, it is usually treated like the systemic forms of ALCL.

Patients with systemic ALCL are divided into two groups, depending on whether or not their cells have an abnormal form of a protein on their surface called "anaplastic lymphoma kinase" (ALK). Although both lymphomas are treated as aggressive (fast-growing) lymphomas, the disease course is different in patients who have ALK-positive (have the abnormal protein) or ALK-negative (do not have the abnormal protein) ALCL. ALK-positive ALCL responds well to standard chemotherapy treatments, putting most patients in long-term remission. Most people with ALK-negative ALCL initially respond to treatment, but many people will relapse within five years. Because of this, ALK-negative patients are sometimes treated more aggressively, often with a stem cell transplant after remission. ALK-positive ALCL usually affects children and young adults. ALK-negative ALCL is more common in patients over age 60.

A diagnosis of ALCL requires taking a small sample of tumor tissue or abnormal skin tissue, called a biopsy, and looking at the cells under a microscope. Additional tests may be conducted that give doctors more information about the disease and whether or not it has spread (and if so, how far) in the body. The assessments can include blood tests, a computed axial tomography (CAT) scan, a positron emission tomography (PET) scan, a magnetic resonance imaging (MRI) scan, and a bone marrow biopsy.

Treatment Options

Treatment of primary cutaneous ALCL depends on the extent of skin lesions. If the disease is confined to a single lesion or area, radiation therapy will result in long-term remission in approximately half of patients. If there are multiple lesions or relapsed disease in the skin, radiation can eradicate the skin lesions, but will not reduce the likelihood of new lesions developing. Although ALCL tends to relapse, the long-term prognosis remains excellent as long as relapses are confined to the skin. At relapse, radiation, topical treatments, mild chemotherapies, biologic therapies, or excisions for small lesions have all been shown to be successful in controlling the disease.

For those with primary cutaneous ALCL appearing in multiple sites on the body, systemic treatment, which travels through the blood and affects many parts of the body, is usually needed.

Although newly diagnosed systemic ALCL patients respond well to common first-line chemotherapy regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), long-term disease outcome varies depending on the subtype. Patients with ALK-positive disease usually respond very well to CHOP, with a long-term disease-free survival rate of over 70 percent. In contrast, patients with ALK-negative disease have less than a 50 percent long-term disease-free survival rate with similar treatments. Some patients with relapsed or refractory disease do not respond to combination therapy; for those patients, higher doses of chemotherapy followed by a stem cell transplant may be prescribed.

Brentuximab vedotin (Adcetris) was approved by the U.S. Food and Drug Administration in 2011 for the treatment of patients with relapsed or refractory systemic ALCL. Both ALK-positive and ALK-negative subtypes of ALCL have been shown to respond well to brentuximab vedotin.

Treatments Under Investigation

New treatments for ALCL are being researched all the time. There are several new drugs now in clinical trials that are showing promising results, including:

  • Bortezomib (Velcade)
  • Brentuximab vedotin (Adcetris)
  • Crizotinib (Xalkori)
  • Pralatrexate (Folotyn)
  • Romidepsin (Istodax)

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 LRF or with their physician 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 ALCL. Patients interested in participating in a clinical trial should talk to their physician or contact LRF's Helpline for an individualized clinical trial search by calling (800) 500-9976 or emailing helpline@lymphoma.org.

Follow-up

The therapies listed above are often effective for helping many patients achieve a remission; however, relapse occurs in up to 50 percent of patients with ALCL. Therefore, long-term follow-up care is required. Patients in remission should have regular visits with a physician who is familiar with their medical history as well as with 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, each patients age, gender, and the overall health of each patient at the time of treatment. The doctor will check for these effects during follow-up care.

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.

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 the website at www.lymphoma.org or www.FocusOnALCL.org. You can also contact the Helpline at (800) 500-9976 or emailing helpline@lymphoma.org.

Webcasts/Podcasts
Teleconferences
In-person Educational Programs
Support Programs

View or order the following publications from our booklets/factsheets webpage:

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.