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What is Lymphoma Hodgkins ?
Hodgkin lymphoma, also called Hodgkin’s disease, is one category of lymphoma, a cancer of the lymphatic system. Lymphoma begins when cells in the lymphatic system change and grow uncontrollably, which may form a tumor or spread to other parts of the body.
About the lymphatic system
The lymphatic system is made up of thin tubes that branch out to all parts of the body. Its job is to fight infection and disease. The lymphatic system carries lymph, a colorless fluid containing lymphocytes (a type of white blood cell). Lymphocytes are part of our immune system and help fight germs in the body. B-lymphocytes (also called B cells) make antibodies to fight bacteria, and T-lymphocytes (also called T cells) kill viruses and foreign cells and trigger the B cells to make antibodies.
Groups of bean-shaped organs called lymph nodes are located throughout the body at different areas in the lymphatic system. Lymph nodes are found in clusters in the abdomen, groin, pelvis, underarms, and neck. Other parts of the lymphatic system include the spleen, which makes lymphocytes and filters the blood; the thymus, an organ under the breastbone; and the tonsils, which are located in the throat.
Hodgkin lymphoma most commonly affects lymph nodes in the neck or the area between the lungs and behind the breastbone. It can also begin in groups of lymph nodes under an arm, in the groin, or in the abdomen or pelvis.
If Hodgkin lymphoma spreads, it may spread to the spleen, liver, bone marrow, or bone. Spread to other parts of the body can also occur, but it is unusual.
Types of Hodgkin lymphoma
There are different types of Hodgkin lymphoma. It is important to know the type, as this may affect the choice of treatment. Doctors determine the type of Hodgkin lymphoma based on how the cells in a tissue sample look under a microscope and whether the cells contain certain abnormal proteins.
The American Joint Committee on Cancer (AJCC) recognizes these major categories of Hodgkin lymphoma:
Classical Hodgkin lymphoma. Classical Hodgkin lymphoma (CHL) is diagnosed when characteristic Reed-Sternberg cells are found. About 20% to 25% of people with CHL in the United States and Western Europe have also had an infection with the Epstein-Barr virus (EBV, the virus that causes infectious mononucleosis, also known as "mono"). However, the role of EBV in the development of Hodgkin lymphoma is not yet clear.
The following list describes the different CHL subtypes.
Nodular sclerosis Hodgkin lymphoma. Nodular sclerosis Hodgkin lymphoma is the most common form of CHL; up to 80% of people with CHL have this form. It is most common in young adults, especially women. In addition to Reed-Sternberg cells, there are bands of connective tissue in the lymph node. This type of lymphoma often involves the lymph nodes in the mediastinum (chest).
Lymphocyte-rich classical Hodgkin lymphoma. About 6% of people with CHL have this form. It is more common in men and usually involves areas other than the mediastinum. The tissue contains many normal lymphocytes, in addition to Reed-Sternberg cells.
Mixed cellularity Hodgkin lymphoma. This subtype of lymphoma occurs in older adults and, commonly, in the abdomen. It carries many different cell types, including large numbers of Reed-Sternberg cells.
Lymphocyte-depleted Hodgkin lymphoma. Lymphocyte-depleted Hodgkin lymphoma is the least common subtype of CHL, and about 1% of people with CHL have this form. It is most common in older adults, people with the human immunodeficiency virus (HIV, the virus that causes autoimmune deficiency syndrome or AIDS), and people in nonindustrial countries. The lymph node contains almost all Reed-Sternberg cells.
There is another type of Hodgkin lymphoma that is not a part of the CHL group; rather, it is more similar at the protein and genetic level to B-cell non-Hodgkin lymphoma.
Nodular lymphocyte-predominant Hodgkin lymphoma. About 5% of people with Hodgkin lymphoma have nodular lymphocyte-predominant Hodgkin lymphoma. It is most common in younger patients, and is often found in the neck lymph nodes. Nodular lymphocyte predominant Hodgkin lymphoma is often treated differently than CHL. Patients with this type of lymphoma tend to have a very good prognosis (chance of recovery). Sometimes, nodular lymphocyte predominant Hodgkin lymphoma will turn into an aggressive type of non-Hodgkin lymphoma called diffuse large B-cell lymphoma.
Symptoms and Signs
People with Hodgkin lymphoma may experience the following symptoms or signs. Sometimes, people with Hodgkin lymphoma do not show any of these symptoms, or these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor. Common symptoms associated with Hodgkin lymphoma include:
Painless swelling of lymph nodes in the neck, underarm, or groin area that does not go away in a few weeks
Unexplained fever that does not go away
Unexplained weight loss
Night sweats (usually drenching)
Pruritus (generalized itching)
Fatigue
Pain in the lymph nodes associated with alcohol intake
If the lymph nodes in the chest are affected, they may press on the windpipe and cause shortness of breath, cough, or chest discomfort.
The doctor may use certain symptoms to help describe the disease, called staging. Each stage may be subdivided into "A" and "B" categories.
A means that a person has not experienced B symptoms, listed below.
B means that a person has experienced the following symptoms:
Unexplained weight loss of more than 10% of original body weight during the six months before diagnosis
Unexplained fever, with temperatures above 38º C (100.4º F)
Drenching night sweats. Most patients say that either their nightclothes or the sheets on the bed are actually wet. Sometimes, heavy sweating occurs during the day.
Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.
If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.
Risk Factors
A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.
The exact cause of Hodgkin lymphoma is not known, but the following factors may raise a person’s risk of developing Hodgkin lymphoma:
Age. People between age 15 and 40 and people older than 55 are more likely to develop Hodgkin lymphoma.
Gender. Men are slightly more likely to develop Hodgkin lymphoma than women overall, although the nodular sclerosis subtype is more common among women.
Family history. Brothers and sisters of people with Hodgkin lymphoma have a higher chance of developing the disease, although the likelihood is small.
Virus exposure. People who are infected with EBV may be at increased risk for developing some types of Hodgkin lymphoma. However, there are probably several other factors involved. EBV is a very common disease, but Hodgkin lymphoma is very uncommon. People who have human immunodeficiency virus also have a higher risk of developing Hodgkin lymphoma, particularly lymphocyte-depleted Hodgkin lymphoma.
It is important to note that, although viruses may be involved in the development of Hodgkin lymphoma, there is no evidence that this type of cancer is contagious. Close contact with someone with Hodgkin lymphoma does not increase a person’s risk of developing the disease.
Diagnosis
Doctors use many tests to diagnose cancer and find out the extent of the disease. Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has spread. Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
Type of cancer suspected
Severity of symptoms
Previous test results
The following tests may be used to help diagnose Hodgkin lymphoma:
Medical history and physical examination. A thorough medical history and physical examination can show evidence of typical symptoms, such as night sweats, fevers, and enlarged lymph nodes or spleen.
Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but Hodgkin lymphoma can only be diagnosed after a biopsy of an affected piece of tissue. Most commonly, this will be a lymph node in the neck, under the arm, or in the groin. If there are no lymph nodes in these areas, a biopsy of other lymph nodes, such as those in the center of the chest, may be necessary. This type of biopsy usually requires minor surgery, although occasionally it is possible to do a biopsy using a needle and local anesthesia (medication to block the awareness of pain) during a scan, most commonly a computed tomography (CT or CAT) scan (see below). The CT scan is used to help the doctor guide the needle to the correct place.
The sample removed during the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). A biopsy of CHL usually has Reed-Sternberg cells. Reed Sternberg cells are often not found in the nodular lymphocyte-predominant Hodgkin lymphoma, which usually has a different type of cancerous cell, called the LP cell.
Once Hodgkin lymphoma is diagnosed, other tests can help find out the extent of the disease or stage and other information to help doctors plan treatment. These tests include the following:
Laboratory tests. Blood tests may include a complete blood count (CBC) and analysis of the different types of white blood cells, in addition to liver function tests. The doctor may also test for the erythrocyte sedimentation rate (ESR), also called the "sed rate."
Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the size of a tumor. Sometimes, a contrast medium (special dye) is injected into a patient’s vein or given orally (by mouth) to provide better detail. A CT scan of the chest, abdomen, and pelvis can help find cancer that has spread to the lungs, lymph nodes, or liver.
Positron emission tomography (PET) scan. A PET scan is another imaging test that can help find cancer in the body. A small amount of a radioactive substance (called radioactive glucose) is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that produce the most energy. Because cancer tends to use energy actively, it absorbs more of the substance. A scanner then detects this substance to produce images of the inside of the body. PET scans may be used to determine the stage of Hodgkin lymphoma, although they are usually done with a CT scan. PET scans may also be used to see how well the lymphoma is responding to treatment.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture. This is sometimes used for Hodgkin lymphoma.
Bone marrow biopsy and aspiration. These two procedures are similar and often done at the same time. Bone marrow (the soft, spongy tissue that is found inside the center of bones) has both a solid and a liquid part. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. An aspiration removes a sample of fluid with a needle. The sample(s) are then analyzed by a pathologist.
Lymphoma often spreads to the bone marrow, so looking at a sample of the bone marrow can be important for doctors to diagnose lymphoma and determine the stage. The sample removed during the aspiration is also used to find any chromosome changes. Whether a bone marrow biopsy is needed depends on the extent of the disease and the results of laboratory tests (see above).
The most common site for a bone marrow biopsy and aspiration is the iliac crest of the pelvic bone, located in the lower back of the hip. This is generally a safe area of the body to perform the procedure because there are no veins or nerves in that area that could be damaged. The skin and bone in that area are numbed with medication beforehand, and other types of anesthesia may be used.
Stages
Staging helps to describe where the Hodgkin lymphoma is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer’s stage, so staging may not be complete until all tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis. There are different stage descriptions for different types of cancer.
When staging Hodgkin lymphoma, doctors evaluate the following:
The number of cancerous lymph node areas
The location of the cancerous lymph nodes: localized (located only in one area of the body) or generalized (located in many areas of the body)
Whether the cancerous lymph nodes are on one or both sides of the diaphragm (the thin muscle under the lungs and heart that separates the chest from the abdomen)
Whether the disease has spread to the bone marrow, spleen, or extralymphatic organs (organs outside the lymphatic system; noted using an “E” below), such as the liver, lungs, or bone
The stage of lymphoma describes the extent of the spread of the tumor, using the terms stage I through IV (one through four). As explained in Symptoms, each stage may also be subdivided into “A” and “B” categories, based on the presence or absence of specific symptoms.
Stage I: The cancer is found in one lymph node region (stage I).
Stage II: Either one of these conditions:
The cancer is in two or more lymph node regions on the same side of the diaphragm (stage II).
The cancer involves a single organ and its regional lymph nodes (lymph nodes located near the site of the lymphoma), with or without cancer in other lymph node regions on the same side of the diaphragm (stage IIE).
Stage III: There is cancer in lymph node areas on both sides of the diaphragm (stage III). In addition, there may be involvement of an extralymphatic organ (stage IIIE), involvement of the spleen (using the letter “S,” stage IIIS), or both (stage IIIES).
Stage IV: There is disseminated (multifocal) involvement, meaning that the lymphoma has spread throughout more than one area. Common sites for disseminated disease include the liver, bone marrow, or lungs.
Recurrent: Recurrent lymphoma is lymphoma that comes back after treatment. Lymphoma may return in the area where it first started or in another part of the body. Recurrence may occur shortly after the first treatment or years later. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.
Prognostic factors. In addition to stage, doctors use other prognostic factors to help plan the best treatment and predict how well treatment will work. For patients with Hodgkin lymphoma, several factors can predict whether the disease will return and which treatments will be successful. A patient may be described as having high-risk disease or low-risk disease based on how many of the following prognostic factors there are.
Below are prognostic factors for patients with early-stage Hodgkin lymphoma. In general, the prognosis for all patients with stage I and stage II disease is good, but the more poor prognostic factors a patient has, the more intense the treatment might be. These prognostic factors include:
Older age is associated with a poorer prognosis.
Women have a better prognosis than men.
A higher ESR (described in the Diagnosis section) is associated with a poorer prognosis.
People with lymphocyte-predominant Hodgkin lymphoma, nodular sclerosis Hodgkin lymphoma, and lymphocyte-rich classical Hodgkin lymphoma have a better prognosis, compared with other subtypes of Hodgkin lymphoma.
A large mediastinal mass (a large lymph node mass in the center of the chest that is larger than 10 centimeters) is associated with a poorer prognosis. (Small mediastinal masses are not associated with a poorer prognosis.)
A higher number of lymph node sites involved is associated with a poorer prognosis.
Below are poor prognostic factors for patients with advanced Hodgkin lymphoma.
Having low blood albumin (a type of protein) levels (less than 4 g/L)
Having low hemoglobin (red blood cell count) (less than 10.5 g/dL)
Being a male
Being age 45 and older
Having stage IV disease
Having a white blood cell count of greater than 15,000 per cubic millimeter
Having a lymphocyte count of less than 600 per cubic millimeter, less than 8% of the total white blood cell count, or both
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