Hodgkins disease & Hodkins lymphoma
HODGKINS DISEASE (HODGKIN'S LYMPHOMA)
A disease marked by chronic enlargement of the lymph nodes, often local at the onset and later generalized, together with enlargement of the spleen and often of the liver, no pronounced leukocytosis, and commonly anemia and continuous or remittent (Pel-Ebstein) fever; considered to be a malignant neoplasm of lymphoid cells of uncertain origin (Reed-Sternberg cells), associated with inflammatory infiltration of lymphocytes and eosinophilic leukocytes and fibrosis; can be classified into lymphocytic predominant, nodular sclerosing, mixed cellularity, and lymphocytic depletion type; a similar disease occurs in domestic cats. Syn: lymphadenoma, Hodgkin lymphoma.
Alternative Names: Lymphoma - Hodgkin's; Hodgkin's disease; Cancer - Hodgkin's lymphoma.
Lymphomas represent tumors of the lymphatic system. This system is a network of organs, ducts, and nodes. The system interacts with the blood's circulatory system to transport a watery clear fluid called lymph throughout the body. The lymphatic system contains lymphocytes, which are important cells involved in defending the body against infections. This system also restores 60% of the fluid that leaks out from blood capillaries back into circulation. Its ducts provide transportation for fats, proteins, and other substances collected from the body's tissues.
The lymphatic system helps produce and transport lymphocytes, white blood cells that are a primary component of the immune system. Some lymphocytes produce antibodies that can target and attack specific foreign substances (antigens).
Lymphocytes develop in the bone marrow or thymus gland. They are categorized as either B cells (bone marrow-derived cells) or T cells (thymus gland-derived cells).
- Lymphatic vessels begin as tiny tubes. They lead to larger lymphatic ducts and branches, and drain into two ducts in the neck, where the fluid re-enters the bloodstream.
- Along the way, the fluid passes through lymph nodes, which are oval structures composed of lymph vessels, connective tissue, and white blood cells. Here, the lymphocytes are either filtered out or added to the contents of the node.
- Both leukemia and lymphomas (Hodgkin's disease and non-Hodgkin's lymphomas) are cancers of lymphocytes. The difference is that leukemia starts in the bone marrow while lymphomas originate in lymph nodes and then spread to the bone marrow or other organs.
Lymph Nodes: In a lymph node, lymphocytes typically receive their initial exposure to foreign substances, such as bacteria. This exposure prompts the lymphocytes to perform their immune functions. The size of a lymph node varies generally from that of a pinhead to a bean. Most nodes are clustered throughout the body. Important node clusters are found in the neck, lower arm, armpit, and groin.
Other Structures in the Lymphatic System: The tonsils and adenoids are secondary lymphatic organs. They are composed of masses of lymph tissue that also play a role in the lymphatic system. The spleen is another important organ that processes lymphocytes from incoming blood.
Hodgkin's disease is a type of lymphoma. Lymphomas are cancers of the lymphatic system. They are generally subdivided into two groups: Hodgkins disease (Hodgkins disease) and non-Hodgkin's lymphoma (NHL). NHL is discussed in another report. Hodgkin's disease is also called Hodgkin's lymphoma.
Hodgkin's disease is marked by the presence of abnormal large cells called Reed-Sternberg cells. Reed-Sternberg cells are derived from B cell lymphocytes (white blood cells). Reed-Sternberg cells are specific to Hodgkin's disease. They are not found in non-Hodgkin's lymphoma.
Most often HD starts in B cell lymphocytes located in lymph nodes in the neck area, although any lymph node may be the site of initial disease.
There are two major types of Hodgkins disease: Classical Hodgkins disease lymphoma and nodular lymphocyte-predominant Hodgkin's disease.
Classical Hodgkin's Lymphoma: Classical Hodgkin's lymphoma accounts for about 95% of Hodgkin's disease cases. It has four major subtypes:
Nodular Sclerosis: Nodular sclerosis is the most common subtype, representing about 60 - 80% of HD cases. Younger patients are more likely to have this type. The nodes first affected are often those located in the center of the chest (the mediastinum) or the neck.
- Mixed Cellularity: Mixed cellularity is the next most common HD form, occurring in about 15 - 30% of patients, mostly in older adults. Mixed cellularity refers to the presence of Reed-Sternberg cells and other cell types.
- Lymphocyte Rich: The lymphocyte-rich subtype accounts for about 5% of all Hodgkins disease cases. It tends to affect men more than women.
- Lymphocyte Depleted: The lymphocyte-depleted subtype is the least common type of Hodgkins disease, occurring in only about 1% of cases. It is usually seen in older people and patients infected with HIV. It is also more common in less developed countries. The cancer tends to be diagnosed when it is widespread, affecting the spleen, bone marrow, and liver as well as abdominal lymph nodes.
Nodular Lymphocyte-Predominant Hodgkin's Disease: Nodular lymphocyte-predominant Hodgkin's disease occurs in about 5% of patients. It is distinct from classical Hodgkin's lymphoma. The cells look like and are referred to as “popcorn” cells, which are variants of Reed-Sternberg cells. This type of Hodgkins disease typically affects younger patients and usually originates in the neck lymph nodes. This type of Hodgkins disease is sometimes confused with non-Hodgkin's lymphoma (NHL). In fact, there is a 3 - 5% risk that nodular lymphocyte-predominant Hodgkin's disease can transform into diffuse large B-cell NHL.
Spread of Cancer: Hodgkin's disease usually progresses in an orderly way from one lymph node region to the next. This process may be slow, particularly in younger people, or very aggressive. The disease typically spreads downward from the initial site.
If it spreads below the diaphragm, it usually reaches the spleen first; the disease may then spread to the liver and bone marrow. If the disease starts in the nodes in the middle of the chest, it may spread outward to the chest wall and areas around the heart and lungs.
Studies indicate that survival rates 15 years after treatment are 85% of all patients. More than 80% of patients with Hodgkin's disease reach complete remission after initial treatment. Unlike other cancers, Hodgkin's disease is even potentially curable in late stages, with up to 85% of patients in late stages surviving 10 years and longer.
The disease recurs after treatment in about 20 - 35% of patients after an average of 10 years, but it is still potentially curable even in these cases. In one study, of those who relapsed two or more years after remission, survival was over 80%. (In other cancers, late stages or relapses are very rarely curable.)
Symptoms of Hodgkin's disease are non-specific and may include:
Painless swelling of one or more lymph nodes, with no recently accompanying infection.
- Symptoms stemming from pressure of swollen lymph nodes on nearby organs or structures. These may include cough and shortness of breath, abdominal pain and swelling, Horner's syndrome (a neurological problem affecting the face and eyes, due to damage to nerves in the neck), nerve pain, and leg swelling.
- Fever, either persistent or alternating with periods of normal temperatures.
- Pain in lymph nodes after drinking alcohol.
- Drenching night sweats.
- Unintentional weight loss (more than 10% over six months)
- Bone pain.
- Increased susceptibility to infections.
- Total body itching.
Causes and Risk factors:
The exact cause of Hodgkin's disease is unknown. There are five types of Hodgkin's disease — all among a group of cancers called lymphomas — cancers of the lymphatic system. The lymphatic system includes the lymph nodes (lymph glands), which are located throughout your body and are connected by small vessels called lymphatics. The spleen, thymus gland and bone marrow also are part of the lymphatic system.
Hodgkin's disease commonly begins in lymph nodes located in the upper part of your body. Some lymph nodes are in areas more readily noticed, such as in your neck, above your collarbone, under your arms or in your groin area. Enlarged lymph nodes in the chest cavity also are common. Eventually, Hodgkin's disease may spread outside your lymph nodes to virtually any part of your body.
Abnormal B cells: A key step in Hodgkin's disease involves the development of abnormal B cells, a type of lymph cell that's an important part of your immune system's response to foreign invaders. B cells normally work with T cells, which derive from the thymus, to fight infection. T cells kill foreign invaders directly. B cells become plasma cells, which in turn produce antibodies that neutralize foreign invaders.
When B cells develop into large abnormal cells, these abnormal, cancerous cells are called Reed-Sternberg cells, after the two pathologists who first discovered them. Instead of undergoing the normal cell cycle of life and death, these Reed-Sternberg cells don't die, and they continue to produce abnormal B cells in a malignant process.
Age: The rate of Hodgkins disease is high in two age groups: young adults (age 15-40) and older adults (age 55+).
Sex: In general, both Hodgkin's disease and non-Hodgkin's lymphoma affect men more often than women.
Infections: The risk of Hodgkins disease is somewhat higher in people who have been infected with viruses, such as Epstein-Barr virus (EBV). EBV infects the B-cells and causes an illness known as infectious mononucleosis (sometimes referred to as "mono"). In people who have mono, the infected B-cells are sought out and killed by the body's T-cells. But if the patient has a T-cell shortage, the EBV-infected B-cells build up within the bloodstream and increase the risk for genetic mutations that can cause lymphoma. It is important to note that there is no evidence of EBV infection in many Hodgkins disease patients, so its role in development of the disease remains uncertain.
- Specific medical conditions may increase the risk for Hodgkin's disease and non-Hodgkin's lymphoma. HD and NHL are more common among patients who have a weakened immune system, such as those with:
- Acquired immunodeficiency syndrome (AIDS)—disease caused by human immunodeficiency virus (HIV).
- Autoimmune diseases (e.g., rheumatoid arthritis, lupus, multiple sclerosis, under- or overactive thyroid)—disease caused by the body's immunologic attacks against its own tissues.
- Inherited immune deficiency syndromes (e.g., ataxia telangiectasia).
- Organ transplants that require the use of immunosuppressant drugs.
Some experts believe that Hodgkins disease disease is caused by a complex deficiency in cellular immunity. Such a deficiency may be due to chronic overstimulation by cytokines—substances that draw germ-fighting white blood cells to areas of infection.
Genetics: The rates of lymphoma and leukemia (e.g., chronic lymphocytic leukemia, CLL) are especially high in some Jewish populations, whereas Asian populations rarely develop CLL. Among first-degree relatives (parents, children, siblings) of CLL patients there is a two- to four-fold increased risk for this cancer.
The doctor will take a medical history and perform a physical examination. If these simple procedures point to Hodgkin's disease, a number of additional tests may be needed to either rule out other diseases or confirm HD and determine the extent of the cancer.
Physical Examination: The doctor will examine not only the affected lymph nodes but also the surrounding tissues and other lymph node areas for signs of infection, skin injuries, or tumors. The consistency of the node is sometimes indicative of certain conditions. For example, a stony, hard node is often a sign of cancer, usually one that has metastasized (spread to another part of the body). A firm, rubbery node may indicate lymphoma (including Hodgkin's). Soft nodes suggest infection or inflammatory conditions.
Blood Tests: Blood tests are performed to measure white and red blood cells, blood protein levels, the uric acid level, blood proteins, and the liver's function. Another blood test is the erythrocyte sedimentation rate (ESR), which is sometimes elevated in Hodgkin's disease (although it is not specific for this condition).
Chest X-Ray:A chest x-ray shows the lymph nodes in the chest and neck area, where Hodgkin's disease usually starts. It a useful step for detection of enlarged lymph nodes.
Computer Tomography: Computed tomography (CT) scans are more accurate than x-rays. They can detect abnormalities in the chest and neck area, as well as revealing the extent of the cancer and whether it has spread. CT scans are used to evaluate symptoms and help diagnose lymphomas, help with staging of the disease, monitor response to treatment, and evaluate when the symptoms occur. A CT scan is also often used in detecting lymphomas in the abdominal and pelvic areas, the brain, and chest area.
Positron Emission Tomography (PET): PET scans combined with CT scans can help doctors clarify the location of the cancer. PET scans can also provide information on whether or not an enlarged lymph node is benign or cancerous and are more accurate than CT scans or other imaging tests for staging lymphomas. PET scans may also help doctors determine how well a patient has responded to treatment, if any residual cancer exists, and if a patient has achieved remission.
Biopsy: A biopsy of the suspicious lymph node is the most definitive way to diagnose Hodgkin's disease. The lymph node sample will be examined by a pathologist for the presence of Reed-Sternberg cells or other abnormal features.
The type of biopsy performed depends in part on the location and accessibility of the lymph node. The doctor may surgically remove the entire lymph node (excisional biopsy) or a small part of it (incisional biopsy). In some cases, the doctor may use fine needle aspiration to withdraw a small amount of tissue from the lymph node. Biopsies of bone marrow may also be performed in patients with existing Hodgkins disease if the doctor suspects that it may have spread to the marrow.
Treatment for Hodgkin's disease is very effective for most people. The type and length of treatment varies, depending on the stage and type of the disease; where the disease is found in the body; and the person's age, physical maturity, and overall health.
The most common treatment for non-Hodgkin's lymphoma is chemotherapy. Chemotherapy kills or stops the growth of cancer cells. Patients are also sometimes treated with radiation therapy. In addition, some people may have surgery to remove tumors.
For some patients who are getting very aggressive chemotherapy or radiation treatments, doctors may perform bone marrow or stem cell transplants to replace cells that have been damaged by the treatment. These procedures involve taking the cells from bone marrow or blood that has either been taken from the patient or donated by another person. These cells are then inserted into the patient's bloodstream to replace those that have been damaged or destroyed.
Researchers are constantly working on new treatments for cancer. Some people decide to participate in clinical trials, which are ways to test new cancer treatments or compare their effectiveness with existing treatments. If you have Hodgkin's disease, your doctor can tell you whether these are a good idea for the particular type you have.
Treatment for Hodgkin's disease is powerful. It destroys good cells along with bad, which can create certain side effects.
Although the side effects depend on the individual and the medicine that a doctor prescribes, the most common short-term side effects of chemotherapy are nausea, vomiting, and a flu-like feeling. Some people feel weak or dizzy after their treatments, or they run a fever. Others get sores in their mouths or suddenly don't feel much like eating. It's also common for people to lose some or all of their hair.
The short-term side effects of radiation can be similar to those of chemotherapy, although the side effects of radiation are usually more localized, meaning they affect only the area that receives the radiation treatment. People can continue to feel side effects for weeks after their treatment ends.
Chemotherapy and radiation treatments can weaken the immune system. If you're getting one of these treatments, steer clear of friends and family with colds, the flu, or other infections. You also need to avoid cuts and other injuries. It's best to put sports and the more strenuous forms of physical activity on hold, but you can still stay active with gentle forms of exercise, like walking.
Tell your doctor if you experience any side effects of treatment. Your doctor can also tell you about possible long-term side effects of the type of treatment you are having.
It can be hard to deal with the side effects of treatment. Perhaps you feel tired and nauseous, and you have to deal with losing your hair. It's important to lean on your parents, other family, and friends. If you want, ask to talk to a psychologist, who will listen to your feelings privately and without judgment. You also can join a support group, where you'll meet and talk to people who have Hodgkin's or other cancers and are dealing with the same problems you are.
If you have or have had Hodgkins disease, it's important to see your doctor regularly for the years following treatment. Occasionally, cancer may return, and follow-up appointments with your cancer specialist can help you catch it early if it does.
After Hodgkin's disease is gone, most people never get it again. However, some do. The term "recurrent" describes Hodgkin's disease that returns after treatment to the same area or a new one. If you have symptoms, tell your parents and your doctor. Some people can also develop other cancers after being treated for Hodgkin's, which will require more treatments.
Medicine and medications:
ABVD, which consists of doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine.
- BEACOPP, which consists of bleomycin, etoposide, Adriamycin, cyclophosphamide, vincristine (Oncovin), procarbazine and prednisone.
- COPP/ABVD, which consists of cyclophosphamide, Oncovin, procarbazine, prednisone, Adriamycin, bleomycin, vinblastine and dacarbazine.
- Stanford V, which consists of Adriamycin, vinblastine, mechlorethamine, etoposide, vincristine, bleomycin and prednisone.
- MOPP, which consists of mechlorethamine, Oncovin, procarbazine and prednisone.
- MOPP had been the basic regimen, but it's very toxic. ABVD is a newer regimen, with less-severe side effects, and is currently the preferred treatment.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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