INTRODUCTION — Hodgkin's disease, also known as Hodgkin's lymphoma, is one of several types of cancer of the body's lymphatic system. It is named after the British physician who first described the disease in 1832.
The lymphatic system is a network of lymph nodes and interconnecting lymph vessels (show figure 1). Lymph nodes are small, pea-shaped organs that make and store lymphocytes, a type of white blood cell that fights infection. Lymph vessels are similar to blood vessels, and carry a watery fluid (lymphatic fluid) that contains lymphocytes. The thymus, spleen, and bone marrow are other organs in the lymphatic system.
In Hodgkin's lympoma, a cancerous tumor develops in a lymph node, usually in the neck or chest. If the disease spreads, it spreads first to adjacent lymph nodes, and then to the spleen, liver, or bone marrow. As it progresses, Hodgkin's lympoma affects the body's ability to fight infection.
The following discussion will review the risk factors for Hodgkin's disease and how it is diagnosed. Issues regarding the staging and treatment of the disease are discussed in a separate topic review. (See "Patient Information: Staging and treatment for Hodgkin's disease").
RISK FACTORS — There are several characteristics that are known to be associated with an increased risk of developing Hodgkin's lymphoma. However, these factors account for only a small number of the total number of people who develop it.
Age — Hodgkin's disease is more common in persons between ages 20 to 30, and also in those over age 50 (show figure 2). Approximately 7800 new cases of Hodgkin's lympoma are diagnosed in the United States annually. This compares to the other common cancer of the lymphatic system, non-Hodgkin's lymphoma, which is diagnosed in approximately 54,000 persons per year.
Epstein-Barr virus history — A number of studies have suggested that the Epstein-Barr virus (EBV), which causes infectious mononucleosis ("mono"), may play a role in the development of Hodgkin's lympoma.
Family history — Close relatives of younger patients with Hodgkin's lympoma have a three to five times higher risk of developing Hodgkin's lympoma, probably due to genetic and environmental exposures. There is no increased risk for the families of older adults with Hodgkin's lympoma. Men are at slightly higher risk for Hodgkin's lympoma than women.
Persons with immune deficiencies — Persons who have a weakened immune system due to disease (such as infection due to the virus causing AIDS), medications (such as chemotherapy or immunosuppressant drugs), or from immune deficiencies (such as autoimmune disease) have an increased risk of developing Hodgkin's lympoma.
CAUSES — The exact cause of Hodgkin's lympoma is not known. The cancer cells in Hodgkin's lympoma are known as Reed-Sternberg cells (named after the physicians who discovered them). Reed-Sternberg cells are believed to be a cancerous type of B lymphocyte. B cell lymphocytes normally function to make antibodies (proteins) that help fight infection. Instead of following the normal pattern of production of B cells and eventual cell death, Reed-Sternberg cells produce more abnormal B cells and do not die.
There are two main types of Hodgkin's lympoma: classical Hodgkin's lympoma (which includes the subtypes nodular sclerosis, mixed cellularity, lymphocyte depleted, and unclassified) and nodular lymphocyte predominant Hodgkin's lympoma. Nodular sclerosis is the most common type of Hodgkin's lympoma in developed countries.
DIAGNOSIS — The diagnosis of Hodgkin's lympoma is based upon a patient's history and physical examination, a tissue biopsy, laboratory studies, and imaging studies. The initial evaluation provides important information about prognosis, staging, and appropriate treatment.
History and physical examination — Most patients with Hodgkin's lympoma are diagnosed after the patient or clinician notices a painless, enlarged lymph node in the neck. Enlarged nodes may also be found above the clavicle, in the armpit (axilla), or the inguinal (groin) area. Some patients are diagnosed after a mass is seen with a chest x-ray, often done for other reasons. Patients may also have symptoms such as fever, night sweats, and weight loss.
Patients who are suspected of having Hodgkin's disease should be seen by a physician who specializes in cancer treatment (called a hematologist/oncologist) for further evaluation and diagnosis.
Tissue biopsy — If the history and physical exam suggests Hodgkin's lympoma, the enlarged lymph node will be surgically removed, usually from the neck. This is usually done as a day surgery procedure with general (the patient is completely asleep) or local (numbing medicine is injected in the area to be biopsied) anesthesia.
If the lymph node to be removed is in an easily accessible area, it may be removed as an outpatient procedure, under local anesthesia. If it is not easily accessible, it may have to be removed as an inpatient procedure, under general anesthesia. The lymph node, after it has been sectioned and treated with specific stains, is then examined under a microscope by a specifically trained pathologist (called a hematopathologist) for evidence of lymphoma.
Bone marrow biopsy — A bone marrow biopsy may be recommended if the clinician suspects that Hodgkin's lympoma is advanced, if the patient has fever, weight loss, and night sweats, or if the patient has an abnormal blood count. This test determines if there are cancerous cells in the bone marrow, which is, by definition, associated with an advanced stage of Hodgkin's lympoma.
A bone marrow biopsy involves removal of a sample of bone marrow fluid, usually from the pelvic or hip bones. Patients are given local anesthesia, which prevents pain during the procedure. The bone marrow fluid is then examined under a microscope to determine if it is involved with Hodgkin's lympoma.
Laboratory studies — Once the diagnosis of Hodgkin's disease is established by biopsy, other blood tests are recommended, including a complete blood count, erythrocyte sedimentation rate (ESR) (a measure of inflammation), and tests of liver, bone, and kidney function.
Imaging studies — Following the diagnosis of Hodgkin's lympoma, additional imaging studies are done, including a chest X-ray and computed tomography (CT scan) of the chest, abdomen, and pelvis. Some patients may undergo PET scanning, which can detect biochemical changes due to the presence of actively growing cancer cells.
STAGING AND TREATMENT — After a person is diagnosed with Hodgkin's lympoma, he/she is grouped according to the number of lymph nodes and other organs found to contain cancer. The recommended treatment of Hodgkin's lympoma depends upon this grouping (called staging). A full discussion of this is available in a separate topic review. (See "Patient information: Staging and treatment for Hodgkin's disease").
WHERE TO GET MORE INFORMATION — Your healthcare provider is the best source of information for questions and concerns related to your medical problem. Because no two patients are exactly alike and recommendations can vary from one person to another, it is important to seek guidance from a provider who is familiar with your individual situation.
This discussion will be updated as needed every four months on our web site (www.patients.uptodate.com). Additional topics as well as selected discussions written for healthcare professionals are also available for those who would like more detailed information.
A number of web sites have information about medical problems and treatments, although it can be difficult to know which sites are reputable. Information provided by the National Institutes of Health, national medical societies and some other well-established organizations are often reliable sources of information, although the frequency with which they are updated is variable. National Library of Medicine
(www.nlm.nih.gov/medlineplus/healthtopics.html)
National Cancer Institute
(www.cancer.gov)
American Cancer Society
(www.cancer.org)
The Leukemia & Lymphoma Society
(www.leukemia-lymphoma.org)
Cure for Lymphoma Foundation
(www.cfl.org)
Lymphoma Research Foundation of America
(www.lymphoma.org)
National Marrow Donor Program
(www.marrow.org)
People Living With Cancer: The official patient information
website of the American Society of Clinical Oncology
(www.plwc.org/portal/site/PLWC)
[1-5]
Use of UpToDate is subject to the Subscription and License Agreement. REFERENCES 1. Lister, TA, Crowther, D, Sutcliffe, SB, et al. Report of a committee convened to discuss the evaluation and staging of patients with Hodgkin's disease: Cotswolds meeting. J Clin Oncol 1989; 7:1630.
2. Mauch, PM, Kalish, LA, Kadin, M, et al. Patterns of presentation of Hodgkin disease. Implication for etiology and pathogenesis. Cancer 1993; 71:2062.
3. Alexander, FE, Jarrett, RF, Lawrence, D, et al. Risk factors for Hodgkin's disease by Epstein-Barr virus (EBV) status: prior infection by EBV and other agents. Br J Cancer 2000; 82:1117.
4. Hjalgrim, H, Askling, J, Sorensen, P, et al. Risk of Hodgkin's disease and other cancers after infectious mononucleosis. J Natl Cancer Inst 2000; 92:1522.
5. Mauch, PM, Armitage, JO, Diehl, V, et al (editors). Hodgkin's Disease. Lipincott, Philadelphia, 1999.
No comments:
Post a Comment