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Hodgkin Lymphoma (HL)

Overview
Common Types of HL
Treatment Options
Treatments Under Investigation
Clinical Trials
Follow-up
Resources

Overview

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Focus On HL

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).

Hodgkin lymphoma, also known as Hodgkin disease, is not as common as NHL. According to the American Cancer Society, approximately 9,000 new cases of HL are projected each year. Although the cancer can occur in both children and adults, it is most commonly diagnosed in young adults between the ages of 15 and 35 and in older adults over age 50.

Hodgkin lymphoma is characterized by the presence of very large cells called Reed-Sternberg (R-S) cells, although other abnormal cell types may be present. HL usually starts in the lymph nodes, and often spreads from one lymph node to another and can also spread to other organs.

Common signs and symptoms of HL include swelling of the lymph nodes (which is often but not always painless), fever, night sweats, unexplained weight loss, and lack of energy. While most people who have these complaints will not have HL, anyone with persistent symptoms should be seen by a physician to make sure that lymphoma is not present.

Common Types of HL

Hodgkin lymphoma has been divided into two main classifications; these are classical HL (CHL), which accounts for most cases of HL, and nodular lymphocyte predominant HL. The type of HL a patient has may affect treatment choices.

Classical Hodgkin Lymphoma

Nodular Sclerosis CHL iis the most common subtype of HL, accounting for between 60 percent and 80 percent of all cases of HL. In nodular sclerosis CHL, the involved lymph nodes contain R-S cells mixed with normal white blood cells. The lymph nodes often contain a lot of scar tissue, which is where the name nodular sclerosis (scarring) originates. The disease is more common in women than men, and it usually affects adolescents and adults under 50. The great majority of patients are cured with current treatments.

Mixed Cellularity CHLaccounts for about 15 percent to 30 percent of all cases of HL and is found more commonly in men than women. With this type of CHL, the lymph nodes contain many R-S cells in addition to several other cell types. It primarily affects older adults. More advanced disease is usually present by the time this subtype is diagnosed.

Lymphocyte-depletion CHL is rarely diagnosed. Abundant R-S cells and few normal lymphocytes are present in the lymph nodes of patients with this subtype, which is aggressive and usually not diagnosed until it is widespread through the body.

Lymphocyte-rich CHLaccounts for less than five percent of HL cases. The disease may be diffuse (spread out) or nodular (knot-like) in form and is characterized by the presence of numerous normal-appearing lymphocytes and classical R-S cells. This subtype of HL is usually diagnosed at an early stage in adults and has a low relapse (returns after treatment) rate.

Lymphocyte Predominant Hodgkin Lymphoma

Nodular Lymphocyte Predominant HL accounts for five percent to 10 percent of all HL cases. It affects men more often than women and is usually diagnosed before the age of 35. In nodular lymphocyte predominant HL, most of the lymphocytes found in the lymph nodes are normal (not cancerous). Typical R-S cells are usually not found in this subtype, but large, abnormal B-cells, sometimes referred to as popcorn cells, can be seen, as well as small B-cells, which may be distributed in a nodular (knot-like) pattern within the tissues. This subtype is usually diagnosed at an early stage and is not very aggressive. In many ways, this form of HL resembles indolent (slowgrowing) B-cell NHL with late recurrences.

Treatment Options

Over 80 percent of patients with HL are cured. Most patients treated for HL will receive some form of chemotherapy, and sometimes radiation therapy, as their first treatment. The recommended first-line therapy for HL is ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine) with or without radiation therapy or other agents depending on the patient's type and stage of HL as well as overall health status. Stem cell transplantation is typically used in the relapsed (disease returns after treatment) or refractory (disease does not respond to treatment) setting. Brentuximab vedotin (Adcetris) was approved in 2011 for the treatment of relapsed/ refractory HL after stem cell transplantation by the U.S. Food and Drug Administration.

Treatments Under Investigation

Although the cure rate in HL is already high, research continues to look for ways to treat the small minority of patients who are resistant (refractory) to treatment and those who relapse. Many promising therapies are currently under investigation in clinical trials for HL. Types of therapy under investigation include:

  • Bendamustine (Treanda)
  • Bortezomib (Velcade)
  • Lenalidomide (Revlimid)
  • Panobinostat
  • Temsirolimus (Torisel)
  • Vorinostat (Zolinza)

Clinical Trials

Clinical trials are crucial in identifying effective drugs and determining optimal doses for lymphoma patients. 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

Lymphoma survivors 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 computed axial tomography (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 overall health at the time of treatment. Doctors will also 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 fact sheet on survivorship issues.

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.FocusOnHL.org. You can also contact the Helpline at (800) 500-9976 or helpline@lymphoma.org.

Webcasts/Podcasts

Hodgkin Lymphoma - Nancy Bartlett, MD (2012)
Hodgkin Lymphoma: New Therapies and Clinical Trials - Anas Younes, MD (2012)

Teleconferences
Emerging Therapies in Hodgkin and T-Cell Lymphomas - January 30, 2013

In-person Educational Programs
Support Programs

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

Hodgkin Lymphoma Fact Sheet
Hodgkin Lymphoma Relapsed/Refractory Fact Sheet
Understanding 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 helpline@lymphoma.org.