55 Kenosia Avenue
Danbury, CT 06810
Phone: 203.744.0100
Toll Free: 1.800.999.6673
http://rarediseases.org

Hodgkin's Disease

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Copyright 1989, 1992, 1995, 1996, 2000, 2004

Synonyms of Hodgkin's Disease

Disorder Subdivisions

General Discussion

Hodgkin's disease is one of a group of cancers known as a lymphoma. Lymphoma is a general term used to describe cancers that affect the lymphatic system, especially the lymph nodes. Tumors often form in the lymph nodes (places where lymphatic vessels unite) and/or the area around the nodes. Fever, night sweats, and weight loss may occur along with swollen lymph nodes. The exact cause of Hodgkin's disease is unknown.

Symptoms

Usually, the first sign of Hodgkin's disease is a swollen lymph node. Two-thirds of the time, a lymph node in the neck is affected. Otherwise, lymph nodes in the armpits, chest, groin, or abdomen are affected. The disease may spread to other lymph nodes, the area around the nodes, the spleen, liver, lungs, and bone marrow. Some affected individuals may also have fever, night sweats, weight loss, and, rarely, bone pain. In some cases, abnormal enlargement of the spleen (splenomegaly) or the liver (hepatomegaly) may occur. Additional symptoms may include abnormalities affecting the gastrointestinal system and/or kidneys.

Hodgkin's disease affects the tissues and lymph nodes of the lymphatic system. The lymphatic system, which is part of the body's immune system, consists of a complex series of thin vessels (lymph vessels) that are similar to blood vessels and run throughout the body. Lymph vessels carry a clear liquid called lymph that contains a type of white blood cell (lymphocyte). Periodically, small rounded organs appear amid the lymph vessels. These bean-shaped organs, known as lymph nodes, create cells needed to fight infections. Groups of clusters of lymph nodes are found near the armpits, groin, and neck. Additional parts of the lymphatic system include the spleen, thymus, and tonsils.

Causes

The exact cause of Hodgkin's disease is not known. Some preliminary research suggests that an infectious agent, such as a virus (e.g., Epstein Barr Virus) may play some role in Hodgkin's disease. Recent studies indicate that there may be a genetic susceptibility to Hodgkin's disease in the young adult form of the disorder. It is also felt that environmental factors or immune system deficiencies may play a role in the development of Hodgkin's disease. Thus, the cause may be multifactorial.

Hodgkin's disease is a form of cancer. It occurs when cells in the lymphatic system divide too rapidly and grow out of control. Hodgkin's disease may develop in any part of the lymphathic system. Since lymphatic tissue may be found in many different parts of the body, Hodgkin's disease may occur and spread almost anywhere in the body.

Affected Populations

The majority of individuals with the adult form of Hodgkin's disease are between 15 and 40 years of age at the time of diagnosis. A smaller number of individuals are affected after the age of 50. Hodgkin's disease may also affect children. Hodgkin's disease accounts for less than one percent of all cases of cancer in the United States.

Four main types of Hodgkin's disease have been identified, marked by the type of cells that are present. The four types are: nodular sclerosis, mixed cellularity, lymphocyte predominant, and lymphocyte depleted.

Related Disorders

Symptoms of the following disorders can be similar to those of Hodgkin's Disease. Comparisons may be useful for a differential diagnosis:

Non-Hodgkin's lymphomas are a group of cancers of the lymphatic system. Swollen lymph nodes in the neck or groin occur and usually spread throughout the body. Anemia (abnormally low levels of red blood cells), leukemia (abnormally high levels of abnormal white blood cells), weight loss, fever, night sweats, and weakness may also occur. The cause of non-Hodgkin's lymphoma is unknown but it may be caused by a virus. Non-Hodgkin's Lymphoma may occur at any age.

Burkitt's lymphoma is a lymphatic system cancer that affects the lymph nodes as well as other areas of the body. Tumors may occur in the kidneys, gonads, bone marrow, or central nervous system as well as the lymph nodes. Burkitt's lymphoma may be infectious. It rarely occurs in the United States and is more common in Central Africa.

The presenting symptoms of Hodgkin's disease may mimic many infectious diseases. One of the symptoms of leukemia and other forms of cancer may be enlarged lymph nodes. It is important to rule out these other possibilities, before a diagnosis can be made.

Standard Therapies

Diagnosis
A diagnosis of Hodgkin's disease is suspected, based upon a thorough clinical evaluation, a detailed patient history, and identification of characteristic findings (swollen lymph nodes, flu-like symptoms, etc.). A diagnosis may be confirmed by surgical removal and microscopic examination (biopsy) of tissue from a lymph node. The tissue is studied to determine whether particular cells called Reed-Sternberg cells are present.

It is important to determine how far the disease has spread since this determines the appropriate treatment program. Usually physicians use the Ann Arbor Classification System to determine what 'stage' the disease is in. The stage depends on the number and location of malignant sites, whether or not the sites are lymphatic or not, and the presence or absence of weight loss, fever, and sweats.

A variety of x-ray tests may be used to determine how far the disease has spread including CT scans, magnetic resonance imaging (MRIs), and a special x-ray technique that focuses on the lymphatic system (lymphangiogram).

Treatment
Treatment of Hodgkin's disease depends on the stage of the disease. Radiotherapy and/or chemotherapy are the two main treatment options.

Radiotherapy (radiation therapy) destroys lymphocytes and shrinks enlarged lymph nodes by exposing the body to powerful x-rays. Radiotherapy may be used alone or in conjunction with chemotherapy.

Chemotherapy involves the use of 'anticancer' drugs. There are several drug regimens now in use. The drug combination of nitrogen mustard, Oncovin (vincristine), procarbazine, and prednisone is called MOPP. The use of Adriamycin (doxorubicin), bleomycin, vinblastine, and decarbazine (DTIC) is called ABVD. MOPP and ABVD have been used together, alternating every month. MOP-BAP is the use of nitrogen mustard, Oncovin (vincristine), procarbazine, bleomycin, Adriamycin, and prednisone. Chlorambucil, vinblastine, procarbazine, and prednisone is known as ChlVPP. All these drugs may produce adverse side effects and must be carefully monitored by a doctor.

The drug Leukine, manufactured by Immunex Corp., has received approval by the Food and Drug Administration (FDA) for the treatment of Hodgkin's disease.

Other treatment is symptomatic and supportive.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.org. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Information about clinical trials related to Hodgkin's disease and other forms of cancer is available on the www.clinicaltrials.gov web site, at www.cancer.gov, and by phone at (800) 4-CANCER.

In 2003, the U.S. Food and Drug Administration (FDA) granted orphan drug status to the monoclonal antibody SGN-30 for the treatment of Hodgkin's disease. In January 2004, the drug's manufacturer, Seattle Genetics, began a phrase II trial of SGN-30 for patients with refractory or recurrent Hodgkin's disease. For information, contact Carol Schmidt (866) 633-SGEN or at clinicaltrials@seagen. For information on SGN-30, write to:

Seattle Genetics, Inc.
21823-30th Drive S.E.
Bothell, WA 98021
Phone: (425) 527-4000
Website: www.seagen.com

Researchers at the National Institutes of Health (NIH) are studying families affected by Hodgkin's disease. For information or to participate in these studies, contact:

NIH/National Cancer Institute
Attn: Referral Team
(301) 496-4375

Researchers are studying the use of new ways of giving radiation therapy and chemotherapy, new drugs and new drug combinations, and biological therapies. High-dose chemotherapy with bone marrow or peripheral blood stem cell transplantation is also being evaluated. During peripheral blood stem cell transplantation, an affected individual's stem cells are removed from the blood. Stem cells function as "parent" cells, undergoing a series of divisions that result in the formation of all the different types of blood cells (e.g., red blood cell, platelets, etc.). After removal from the blood, stem cells are treated with drugs to eradicate cancer cells and then frozen until they are transplanted back into the affected individual. This procedure may be performed by itself or in combination with an autologous (from the patient rather than another donor) bone marrow transplant. More research is needed to determine the long-term safety and effectiveness of this procedure for the treatment of Hodgkin's disease.

Hodgkin's Disease Resources

Organizations:

References

TEXTBOOKS
Stein, J, ed. Internal Medicine. 4th ed. St. Louis, MO: Mosby Year-Book, Inc. 1994.899-905.

JOURNAL ARTICLES
Morrison C, et al., Hodgkin's disease in primary care. Nurse Pract. 2000;25:44, 47-50, 56.

Johnston LJ, et al., Autologous hematopoietic cell transplantation in Hodgkin's disease. Biol Blood Marrow Transplant. 2000;6:289-300.

Santoro A, et al., Gemcitabine in the treatment of refractory Hodgkin's disease: results of a multicenter phase II study. J Clin Oncol. 2000;18:2615-9.

Scully RE, et al., Case records of the Massachusetts General Hospital. N Engl J Med. 1997;337:1753-61.

Mack TM, et al., Concordance for Hodgkin's disease in identical twins suggesting genetic susceptibility to the young adult form of the disease. N Engl J Med. 1995;332:413-8.

Diehl V, et al., Hodgkin's disease-environmental or genetic? N Engl J Med. 1995;332:463-4.

Bonadonna G, Treatment strategies for Hodgkin's disease. Semin Hematol. 1988;25:51-7.

FROM THE INTERNET
McKusick VA., ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:236000; Last Update:6/13/00.

Report last updated: 2008/04/28 00:00:00 GMT+0