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hodgkin-disease

Hodgkin Disease

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What is Hodgkin disease?

Hodgkin disease (Hodgkin lymphoma) is a type of lymphoma, a cancer that starts in white blood cells called lymphocytes. Lymphocytes are part of the body’s immune system. There are 2 kinds of lymphomas:

Hodgkin disease (named after Dr. Thomas Hodgkin, who first recognized it)

Non-Hodgkin lymphoma

These 2 main types of lymphomas differ in how they behave, spread, and respond to treatment, so it is important to tell them apart. Doctors can usually tell the difference between them by looking at the cancer cells under a microscope or by using sensitive lab tests.

Both children and adults can develop Hodgkin disease. This document discusses treatment in both groups.

The lymph system and lymphoid tissue

To understand what Hodgkin disease is, it helps to know about the body’s lymph system.

The lymph system (also known as the lymphatic system) is part of the body’s immune system, which helps fight infections and some other diseases. It also helps fluids move around within the body. The lymph system is composed mainly of:

Lymphoid tissue: includes the lymph nodes and related organs (see below) that are part of the body’s immune and blood-forming systems

Lymph: a clear fluid that travels through the lymph system, carrying waste products and excess fluid from tissues, as well as lymphocytes and other immune system cells

Lymph vessels: small tubes, similar to blood vessels, through which lymph travels to different parts of the lymph system

Lymphocytes

Lymphoid tissue is made up of cells called lymphocytes, a type of white blood cell that fights infection. There are 2 major types of lymphocytes: B lymphocytes (B cells) and T lymphocytes (T cells). Normal T cells and B cells have different jobs.

B lymphocytes: B cells help protect the body from germs (bacteria and viruses). They do this by maturing into plasma cells, which make antibodies (immune proteins). These antibodies attach to the germs, marking them for destruction. Almost all cases of Hodgkin disease start in B lymphocytes.

T lymphocytes: There are several types of T cells, and each has a special job. Some T cells can directly destroy certain kinds of bacteria or cells infected with viruses or fungi. Other types of T cells play a role in either boosting or slowing the activity of other immune system cells.

Organs that have lymphoid tissue

Because lymphoid tissue is in many parts of the body, Hodgkin disease can start almost anywhere. The major sites of lymphoid tissue are:

Lymph nodes: Lymph nodes are small, bean-sized collections of lymphocytes and other immune system cells found throughout the body, including inside the chest, abdomen, and pelvis. They can sometimes be felt under the skin in the neck, under the arms, and in the groin. Lymph nodes are connected to each other by a system of lymph vessels.

Lymph nodes get bigger when they fight an infection. Lymph nodes that grow in reaction to infection are calledreactive nodes or hyperplastic nodes. These often hurt when they are touched. People with sore throats or colds might have enlarged neck lymph nodes. An enlarged lymph node is not always a sign of a serious problem, but it can be a sign of Hodgkin disease. 

Spleen: The spleen is an organ under the lower part of the rib cage on the left side of the body. The spleen makes lymphocytes and other immune system cells to help fight infection. It also stores healthy blood cells and filters out damaged blood cells, bacteria, and cell waste.

Bone marrow: The bone marrow is the spongy tissue inside certain bones, which is where new white blood cells (including some lymphocytes), red blood cells, and platelets are made.

Thymus: The thymus is a small organ behind the upper part of the breastbone and in front of the heart. It is important in the development of T lymphocytes.

Digestive tract: The back of the throat (adenoids and tonsils), stomach, intestines, and many other organs also have lymphoid tissue.

Start and spread of Hodgkin disease

Because lymphoid tissue is in many parts of the body, Hodgkin disease can start almost anywhere. Most often it starts in lymph nodes in the upper part of the body. The most common sites are in the chest, in the neck, or under the arms.

Hodgkin disease most often spreads through the lymph vessels in a stepwise fashion from lymph node to lymph node. Rarely, and late in the disease, it can invade the bloodstream and spread to other sites in the body, including the liver, lungs, and/or bone marrow.

The Hodgkin disease cell

The cancer cells in most cases of Hodgkin disease are called Reed-Sternberg cells, after the 2 doctors who first described them. These cells are usually an abnormal type of B lymphocyte. Under a microscope, Reed-Sternberg cells are much larger than normal lymphocytes and also look different from the cells of non-Hodgkin lymphomas and other cancers.

In Hodgkin disease, the enlarged lymph nodes usually have a small number of Reed-Sternberg cells and a large number of surrounding normal immune cells. It is mainly these other immune cells that account for the enlarged lymph nodes.

Types of Hodgkin disease

Different types of Hodgkin disease are classified by how they look under the microscope. This is important because types of Hodgkin disease may grow and spread differently and may be treated differently. The 2 main types are:

Classic Hodgkin disease (which has several subtypes)

Nodular lymphocyte predominant Hodgkin disease

All types of Hodgkin disease are malignant (cancerous) because as they grow they may invade and destroy normal tissue and spread to other tissues. There is no benign (non-cancerous) form of Hodgkin disease.

Classic Hodgkin disease

Classic Hodgkin disease (HD) accounts for about 95% of all cases of Hodgkin disease in developed countries. It has 4 subtypes, all of which have classic-looking Reed-Sternberg cells.

Nodular sclerosis Hodgkin disease: This is the most common type of Hodgkin disease in developed countries, accounting for about 60% to 80% of cases. It occurs mainly in younger people, about equally in men and women. It tends to start in lymph nodes in the neck or chest. Under the microscope, the lymph nodes have fibrous bands that criss-cross the node and encircle abnormal nodules of lymph tissue.

Mixed cellularity Hodgkin disease: This is the second most common type (15% to 30%) and is seen mostly in older adults. It can start in any lymph node but most often occurs in the upper half of the body. Under the microscope, many different kinds of cells can be seen, including Reed-Sternberg cells and normal immune system cells.

Lymphocyte-rich Hodgkin disease: This subtype accounts for about 5% of Hodgkin disease cases. It usually occurs in the upper half of the body and is rarely found in more than a few lymph nodes. Under the microscope it looks very much like mixed cellularity Hodgkin disease, except that most of the cells are small lymphocytes.

Lymphocyte-depleted Hodgkin disease: This is the least common form of Hodgkin disease, making up less than 1% of cases. It is seen mainly in older people. The disease is more likely to be advanced when first found, in lymph nodes in the abdomen as well as in the spleen, liver, and bone marrow. When seen under a microscope, there are few normal lymphocytes or other immune system cells, and many Reed-Sternberg cells.

Nodular lymphocyte predominant Hodgkin disease

Nodular lymphocyte predominant Hodgkin disease (NLPHD) accounts for about 5% of Hodgkin disease. It can occur at any age, and is more common in men than in women. This type usually involves lymph nodes in the neck and under the arm. It contains large cells, often called popcorn cells (because they look like popcorn), which are variants of Reed-Sternberg cells. Under the microscope, there is a pattern of sheets of lymphocytes arranged in nodules.

What are the risk factors for Hodgkin disease?

A risk factor is anything that affects your chance of getting a disease such as cancer. Different cancers have different risk factors. Some cancer risk factors, like smoking, can be changed. Others, like a person’s age or family history, can’t be changed.

But risk factors don’t tell us everything. Having a risk factor, or even several, does not mean that you will definitely get the disease. And many people who get the disease may have few or no known risk factors. Even if a person with Hodgkin disease has one or more risk factors, it is often very hard to know how much these factors might have contributed to the lymphoma.

Scientists have found a few risk factors that may make a person more likely to develop Hodgkin disease, although it’s not always clear why these factors increase risk.

Epstein-Barr virus infection/mononucleosis

People who have had infectious mononucleosis (sometimes called mono for short), an infection caused by the Epstein-Barr virus (EBV), have an increased risk of Hodgkin disease. The risk appears to be a few times higher than for people who have not had mono, although the overall risk is still very small.

The exact role of EBV in the development of Hodgkin disease is not clear. DNA from the virus is found in Reed-Sternberg cells in about half of patients with Hodgkin disease. But the other half has no evidence of EBV in their cancer cells. Many people are infected with EBV, but very few develop Hodgkin disease.

Age

Anyone can be diagnosed with Hodgkin disease, but it is most common in early adulthood (ages 15 to 40, especially in a person’s 20s) and in late adulthood (after age 55).

Gender

Hodgkin disease occurs slightly more often in males than in females.

Geography

Hodgkin disease is most common in the United States, Canada, and northern Europe, and is least common in Asian countries.

Family history

Brothers and sisters of young people with this disease have a higher risk for Hodgkin disease. The risk is very high for an identical twin of a person with Hodgkin disease. But a family link is still uncommon, and is seen in only around 5% of all cases.

It’s not clear why family history might increase risk. It might be because family members have similar childhood exposures to certain infections (such as Epstein-Barr virus), inherited gene changes that make them more susceptible, or some combination of these factors.

Socioeconomic status

The risk of Hodgkin disease is greater in people with a higher socioeconomic background. The reason for this is not clear. One theory is that children from more affluent families might be exposed to some type of infection (such as Epstein-Barr virus) later in life than children from less affluent families, which might somehow increase their risk.

HIV infection

The risk of Hodgkin disease is increased in people infected with HIV, the virus that causes AIDS.

Do we know what causes Hodgkin disease?

The exact cause of Hodgkin disease is not known. However, scientists have found that the disease is linked with a few conditions, such as infection with the Epstein-Barr virus. Some researchers think that this may lead to DNA changes in B lymphocytes, leading to the development of the Reed-Sternberg cell and Hodgkin disease.

Normal human cells grow and function mainly based on the information contained in each cell’s chromosomes. Each cell has 23 pairs of chromosomes, which are long molecules of DNA. DNA is the chemical that makes up our genes — the instructions for how our cells function. We look like our parents because they are the source of our DNA. But DNA affects more than how we look.

Some genes contain instructions for controlling when cells grow and divide into new cells. Certain genes that help cells grow and divide or help them live longer are called oncogenes. Others that slow down cell division or cause cells to die at the right time are called tumor suppressor genes. Cancers can be caused by DNA changes that turn on oncogenes or turn off tumor suppressor genes.

Scientists have found many gene changes in Reed-Sternberg cells that help the cells to grow and divide or prevent the cells from dying when they should. Reed-Sternberg cells also make substances called cytokines, which attract many other cells into the lymph node, enlarging it. In turn, these non-cancerous cells release substances that promote growth of the Reed-Sternberg cells.

Despite these advances, scientists do not yet know what sets off these processes. An abnormal reaction to the Epstein-Barr virus or to other infections may be the trigger in some cases. But more research is needed to understand what causes Hodgkin disease.

Can Hodgkin disease be prevented?

Few known risk factors for Hodgkin disease can be changed, so it is not possible to prevent most cases of the disease at this time.

Infection with HIV, the virus that causes AIDS, is known to increase risk, so one way to limit your risk is to avoid known risk factors for HIV, such as intravenous drug use or unprotected sex with many partners.

Another risk factor for Hodgkin disease is infection with the Epstein-Barr virus (the cause of infectious mononucleosis, or mono), but there is no known way to prevent this infection.

Can Hodgkin disease be found early?

At this time, there are no widely recommended screening tests for this cancer. (Screening is testing for cancer in people without any symptoms.) Still, in some cases Hodgkin disease can be found early.

The best way to find Hodgkin disease early is to pay attention to possible symptoms. The most common symptom is enlargement of one or more lymph nodes, causing a lump or bump under the skin which is usually not painful. This is most often on the side of the neck, in the armpit, or in the groin. More often this is caused by something like an infection, rather than Hodgkin disease, but it is important to have such lumps checked by your doctor.

Other symptoms can include fever that doesn’t go away, drenching night sweats that often require changing bed sheets or night clothes, and unexplained weight loss. Severe and constant itching can be another symptom of Hodgkin disease. However, very early in the disease, many people with Hodgkin disease may not have any symptoms. For more on possible symptoms, see “How is Hodgkin disease diagnosed?”

Careful, regular medical checkups may be helpful for people with known risk factors for Hodgkin disease, such as a strong family history. These people do not commonly develop Hodgkin disease, but they and their doctors should know about possible symptoms and signs.

How is Hodgkin disease diagnosed?

Most people with Hodgkin disease see their doctor because they have felt a lump that hasn’t gone away, they have some of the other symptoms listed below, or they just don’t feel well and go in for a checkup.

If a person has signs or symptoms that suggest Hodgkin disease, exams and tests are done to find out for sure and, if so, to determine the exact type.

Signs and symptoms of Hodgkin disease

You or your child can have Hodgkin disease and feel perfectly well. However, there are some symptoms that this disease may cause.

Lump(s) under the skin

You may notice a lump in the neck, under the arm, or in the groin, which is an enlarged lymph node. Sometimes this may go away, only to come back. Although it doesn’t usually hurt, the area may become painful after you drink alcohol. The lump may become more noticeable over time. There may even be several areas of enlarged lymph nodes.

But Hodgkin disease is not the most common cause of lymph node swelling. Most enlarged lymph nodes, especially in children, are caused by an infection. If this is the case, the node should return to its normal size a couple of weeks or months after the infection goes away.

Other cancers can also cause swollen lymph nodes. If you have an enlarged lymph node, especially if you haven’t had a recent infection, it is best to see your doctor so that the cause can be found and treated without delay, if needed.

General (non-specific) symptoms

Some people with Hodgkin disease have what are known as B symptoms:

Fever (which can come and go over several days or weeks)

Drenching night sweats

Weight loss without trying (at least 10% of body weight over 6 months)

These symptoms can help find Hodgkin disease, but they are also important in determining the stage and prognosis (outlook) if Hodgkin disease is found.

Other possible symptoms of Hodgkin disease include:

Itching skin

Tiredness

Loss of appetite

Sometimes the only symptom may be being tired all the time.

Cough, trouble breathing, chest pain

If Hodgkin disease affects lymph nodes inside the chest, the swelling of these nodes may press on the windpipe (trachea) and make you cough or even have trouble breathing, especially when lying down. Some people may have pain behind the breast bone.

Having one or more of the symptoms above does not mean you have Hodgkin disease. In fact, many of these symptoms are more likely to be caused by other conditions, such as an infection. Still, if you or your child has any of these symptoms, it is very important to have them checked by a doctor so that the cause can be found and treated, if needed.

Medical history and physical exam

If the symptoms suggest you or your child might have Hodgkin disease, your doctor will want to get a thorough medical history, including information about symptoms, possible risk factors, family history, and other medical conditions.

Next, the doctor will do a physical exam, paying special attention to the lymph nodes and other areas of the body that may be involved, including the spleen and liver. Because infections are the most common cause of enlarged lymph nodes, especially in children, the doctor will look for an infection in the part of the body near any swollen lymph nodes.

The doctor also might order blood tests to look for signs of infection or other problems. If the doctor suspects that Hodgkin disease may be causing the symptoms, he or she will recommend a biopsy of the area.

Biopsies

Many of the symptoms of Hodgkin disease are actually more likely to be caused by non-cancerous problems or by other kinds of cancers. For example, enlarged lymph nodes are more often caused by infections than by Hodgkin disease. Because of this, doctors often wait a few weeks to see if they shrink on their own as the infection goes away. Antibiotics may also be prescribed to see if they cause the nodes to shrink.

If the nodes don’t shrink or continue to grow, either a small piece of a node or, more commonly, the entire node is removed to be looked at under the microscope and for other lab tests. This procedure, called a biopsy, is needed to be sure of the diagnosis. If it is Hodgkin disease, the biopsy can also tell what type it is.

Types of biopsies

There are different types of biopsy methods, and doctors choose one based on your situation.

Excisional or incisional biopsy: This is the preferred and most common type of biopsy for an enlarged lymph node. The doctor uses surgical tools to cut through the skin and remove the tumor or lymph node. If the doctor removes the entire lymph node, it is called an excisional biopsy. If a small part of a larger tumor or node is removed, it is called anincisional biopsy.

If the node is near the skin surface, this is a fairly simple operation that can sometimes be done with numbing medicine (local anesthesia). But if the node is inside the chest or abdomen, the patient is sedated or given general anesthesia (where he or she is in a deep sleep). This type of biopsy almost always provides enough of a tissue sample to make a diagnosis of the exact type of Hodgkin disease.

Fine needle aspiration (FNA) or core needle biopsy: In an FNA biopsy, the doctor uses a very thin, hollow needle attached to a syringe to withdraw (aspirate) a small amount of fluid and tiny bits of tissue from a lymph node or an organ in the body. For a core needle biopsy, the doctor uses a larger needle to remove a slightly larger piece of tissue.

If the enlarged node is near the surface of the body, the doctor can aim the needle while feeling the node. If a tumor is deep inside the body, the doctor can guide the needle using a computed tomography (CT) scan or ultrasound.

A needle biopsy does not require an incision, but in many cases it might not remove enough of a sample to diagnose Hodgkin disease (or to determine which type it is). Most doctors do not use needle biopsies (especially FNA biopsies) to diagnose Hodgkin disease. But if the doctor suspects that your lymph node swelling is caused by an infection or by the spread of cancer from another organ (such as the breast, lungs, or thyroid), a needle biopsy might be the first type of biopsy done. An excisional biopsy may still be needed to diagnose Hodgkin disease, even after a needle biopsy has been done.

After Hodgkin disease has been diagnosed, needle biopsies are sometimes used to check areas in other parts of the body that might be Hodgkin disease spreading or coming back after treatment.

Bone marrow aspiration and biopsy: These tests are not used to diagnose Hodgkin disease, but they may be done after the diagnosis is made to see if Hodgkin disease is in the bone marrow.

Lab tests of biopsy samples

All biopsy samples are looked at under a microscope by a pathologist (a doctor specially trained to recognize cancer cells), who looks at the size and shape of the cells and determines if any of them are Reed-Sternberg cells. The pathologist also looks at how the cells are arranged, which could point to the type of Hodgkin disease.

Because diagnosing Hodgkin disease can be tricky, it helps if the pathologist specializes in diseases of the blood. Sometimes the first biopsy does not give a definite answer and more biopsies are needed.

Immunohistochemistry: Looking at the samples under the microscope is often enough to diagnose Hodgkin disease (and what type it is), but sometimes further tests are needed. Special stains of the specimen can spot certain proteins, such as CD15 and CD30, on the surface of the Reed-Sternberg cells. These are typically found in classic Hodgkin disease. Tests for other proteins may point to nodular lymphocyte predominant Hodgkin disease, to non-Hodgkin lymphoma rather than Hodgkin disease, or to other diseases entirely.

How is Hodgkin disease staged?

Once Hodgkin disease is diagnosed, tests will be done to determine the stage (extent of spread) of the disease. The treatment and prognosis (outlook) for a person with Hodgkin disease depend to some extent on both the type and the stage of the disease.

Hodgkin disease generally starts in the lymph nodes. If it spreads, it is usually to another set of nearby lymph nodes. It may invade (grow into) nearby organs as well. Rarely, Hodgkin disease will start in an organ other than lymph nodes, such as a lung. The current staging system is based on these facts.

Staging is based on:

Your medical history (if you have certain symptoms)

The physical exam

Biopsies

Imaging tests, which typically include a chest x-ray, CT (computed tomography) scan of the chest/abdomen/pelvis, and PET (positron emission tomography) scan

Blood tests

Bone marrow aspiration and biopsy (sometimes but not always done)

Imaging tests used to stage Hodgkin disease

Imaging tests use x-rays, sound waves, magnetic fields, or radioactive particles to make pictures of the inside of the body. Imaging tests may be done for a number of reasons, including:

To look for possible causes of certain symptoms, such as enlarged lymph nodes in the chest

To help determine the stage of the lymphoma

To help determine if treatment is working

To look for possible signs of cancer coming back after treatment

Chest x-ray

Hodgkin disease often enlarges lymph nodes in the chest, and they can usually be seen on a plain chest x-ray.

Computed tomography (CT) scan

The CT scan is an x-ray test that produces detailed cross-sectional images of the body. Unlike a regular x-ray, CT scans can show the detail in soft tissues (such as internal organs). This scan can help tell if any lymph nodes or organs in your body are enlarged. CT scans are useful for looking for Hodgkin disease in the chest, abdomen, pelvis, and neck.

Before the test, you may be asked to drink a contrast solution and/or get an intravenous (IV) injection of a contrast dye to better outline abnormal areas in the body. You might need an IV line through which the contrast dye is injected. The injection can cause some flushing (a feeling of warmth, especially in the face). Some people are allergic to the dye and get hives or a flushed feeling or, rarely, have more serious reactions like trouble breathing and low blood pressure. Be sure to tell the doctor if you or your child has any allergies or has ever had a reaction to any contrast material used for x-rays.

A CT scanner has been described as a large donut, with a narrow table in the middle opening. You will need to lie still on the table while the scan is being done. CT scans take longer than regular x-rays, and you might feel a bit confined by the ring while the pictures are being taken. Doctors may advise medicine for some children to help keep them calm or even asleep during the test.

Instead of taking one picture like an x-ray, a CT scanner takes many pictures as it rotates around your body. A computer combines these pictures into detailed images of the part of the body that is being studied.

In some cases, a CT can be used to guide a biopsy needle precisely into a suspicious area. For this procedure, called a CT-guided needle biopsy, you remain on the CT scanning table while a radiologist moves a biopsy needle through the skin and toward the location of the mass. CT scans are repeated until the needle is within the mass. A biopsy sample is then removed and looked at under a microscope.

Magnetic resonance imaging (MRI) scan

This test is rarely used in Hodgkin disease, but if your doctor is concerned about spread to the spinal cord or brain, MRI is very useful for looking at these areas.

Like CT scans, MRI scans provide detailed images of soft tissues in the body. But MRI scans use radio waves and strong magnets instead of x-rays. The energy from the radio waves is absorbed by the body and then released in a pattern formed by the type of body tissue and by certain diseases. A computer translates the pattern into a very detailed image of parts of the body. A contrast material called gadolinium is often injected into a vein before the scan to show details better. This contrast material usually does not cause allergic reactions.

MRI scans take longer than CT scans, often up to an hour. You may have to lie inside a narrow tube, which is confining and can be distressing to some people. Some children may need sedation. Newer, more open MRI machines might be another option. The MRI machine makes loud buzzing and clicking noises that you might find disturbing. Some places give you headphones or earplugs to help block this noise out.

Positron emission tomography (PET) scan

For a PET scan, a form of radioactive sugar (known as fluorodeoxyglucose or FDG) is injected into the blood. Because cancer cells in the body are growing quickly, they absorb large amounts of the radioactive sugar. After about an hour, you will be moved onto a table in the PET scanner. You lie on the table for about 30 minutes while a special camera creates a picture of areas of radioactivity in the body. The picture is not finely detailed like a CT or MRI scan, but it can provide helpful information about your whole body.

PET scans can help show if an enlarged lymph node contains Hodgkin disease or is benign. It can also help spot small areas that might be lymphoma, even if the area looks normal on a CT scan.

PET scans are often used to tell if Hodgkin disease is responding to treatment. Some doctors will repeat the PET scan after 2 or 3 courses of chemotherapy. If it is working, the lymph nodes will no longer take up the radioactive sugar. PET scans can also be used after treatment in helping decide whether an enlarged lymph node still contains cancer or is just scar tissue.

In looking at patients with Hodgkin disease, a machine that combines the PET scan with a CT scan is often used. This allows the doctor to compare areas of higher radioactivity on the PET scan with the more detailed appearance of that area on the CT. PET/CT scans often can help pinpoint the areas involved with lymphoma better than a CT alone.

Gallium scan

This test can find tumors that might be Hodgkin disease in lymph nodes and other organs. Gallium scans are not used as much now as in the past, because most doctors do a PET scan instead. This test can still sometimes be useful in finding areas of lymphoma that the PET scan might miss. It can also help distinguish infections from lymphomas when the diagnosis is not clear.

During this test, a small dose of radioactive gallium is injected into a vein. It is attracted to lymph tissue in the body. A few days later a special camera is used to detect the radioactivity, showing the location of the gallium.

Bone scan

This test is not usually done unless a person is having bone pain or has lab test results that suggest the Hodgkin disease may have reached the bones.

A different radioactive substance (technetium) is used for a bone scan. After it is injected into a vein, it travels to damaged areas of the bone. A special camera can then detect the radioactivity. Hodgkin disease sometimes causes bone damage, which may be picked up on a bone scan. But bone scans can’t show the difference between cancers and non-cancerous problems, which means further tests might be needed.

Other tests

Blood tests

Blood tests aren’t part of the formal staging system for Hodgkin disease, but they can help your doctor get a sense of how advanced the disease is and how well you might tolerate certain treatments.

Hodgkin disease cells do not appear in the blood, but a complete blood count can sometimes reveal signs of Hodgkin disease. Anemia (not having enough red blood cells) can be a sign of more advanced Hodgkin disease. A high white blood cell count is another possible sign, although it can also be caused by infection. Another test called anerythrocyte sedimentation rate (ESR) can help measure how much inflammation is in the body.

Blood tests may also be done to check liver and kidney function and to look for signs that that cancer might have reached the bones.

Your doctor may also suggest other blood tests to look for signs of certain infections:

HIV test: if you have abnormal symptoms that might be related to HIV infection

Hepatitis B virus test: if your doctor plans on using a drug called rituximab (Rituxan) in your treatment, which could cause problems if you have this infection

Bone marrow aspiration and biopsy

If Hodgkin disease has been diagnosed, these tests may be done sometimes to tell if it is in the bone marrow. The bone marrow aspiration and biopsy are usually done at the same time. The samples are taken from the back of the pelvic (hip) bone, although in some cases they may be taken from the sternum (breast bone) or other bones.

In bone marrow aspiration, you lie on a table (either on your side or on your belly). After cleaning the skin over the hip, the doctor numbs the area and the surface of the bone by injecting a local anesthetic, which may cause a brief stinging or burning sensation. A thin, hollow needle is then inserted into the bone and a syringe is used to suck out a small amount of liquid bone marrow. Even with the anesthetic, most patients still have some brief pain when the marrow is removed.

A bone marrow biopsy is usually done just after the aspiration. A small piece of bone and marrow is removed with a slightly larger needle that is twisted as it is pushed down into the bone. The biopsy may also cause some brief pain. Once the biopsy is done, pressure will be applied to the site to help stop any bleeding.

Most children having a bone marrow aspiration and biopsy either receive medicine to make them drowsy or have general anesthesia so they are asleep.

The samples are then sent to a lab, where they are viewed under a microscope to look for signs of Hodgkin disease.

Tests of heart and lung function

These tests are not used to help stage Hodgkin disease, but they may be done if certain chemotherapy drugs are going to be used that could affect the heart or the lungs.

Your heart function may be checked with an echocardiogram (an ultrasound of the heart) or a MUGA scan.

Your lung function may be checked with pulmonary function tests, in which you breathe into a tube connected to a machine.

Cotswold staging system

A staging system is a way for members of the cancer care team to sum up the extent of a cancer’s spread. The staging system for Hodgkin disease (HD) is known as the Cotswold system, which is a modification of the older Ann Arbor system. It has 4 stages, labeled I, II, III, and IV.

If Hodgkin disease affects an organ outside of the lymph system, the letter E is added to the stage (for example, stage IE or IIE). If it involves the spleen, the letter S may be added.

Stage I: Either of the following means that the disease is stage I:

Hodgkin disease is found in only 1 lymph node area or lymphoid organ such as the thymus (I).

The cancer is found only in 1 area of a single organ outside the lymph system (IE).

Stage II: Either of the following means that the disease is stage II:

Hodgkin disease is found in 2 or more lymph node areas on the same side of (above or below) the diaphragm — the muscle beneath the lungs that separates the chest and abdomen (II).

The cancer extends locally from one lymph node area into a nearby organ (IIE).

Stage III: Either of the following means that the disease is stage III:

Hodgkin disease is found in lymph node areas on both sides of (above and below) the diaphragm (III).

Hodgkin disease is in lymph nodes above and below the diaphragm, and has also spread to a nearby organ (IIIE), to the spleen (IIIS), or to both (IIIES).

Stage IV: Any of the following means that the disease is stage IV:

Hodgkin disease has spread widely through 1 or more organs outside of the lymph system. Cancer cells may or may not be found in nearby lymph nodes.

Hodgkin disease is found in organs in 2 distant parts of the body (and not in nearby lymph nodes).

Hodgkin disease is in the liver, bone marrow, lungs (other than by growing there directly from another site), or cerebrospinal fluid (the liquid that surrounds the brain and spinal cord).

Other modifiers may also be used to describe the Hodgkin disease stage:

Bulky disease

This term is used to describe tumors in the chest that are at least ⅓ as wide as the chest, or tumors in other areas that are at least 10 centimeters (about 4 inches) across. It is usually labeled by adding the letter X to the stage. Bulky disease may require more intensive treatment.

A vs. B

Each stage may also be assigned a letter (A or B). B is added (stage IIIB, for example) if a person has any of the symptoms listed below:

Loss of more than 10% of body weight over the previous 6 months (without dieting)

Unexplained fever of at least 100.4°F (38°C)

Drenching night sweats

If a person has any of these B symptoms, it usually means the disease is more advanced, and more intensive treatment is often recommended. If no B symptoms are present, the letter A is added to the stage.

Resistant or recurrent Hodgkin disease

These terms are not part of the formal staging system, but doctors or nurses may use them to describe what is going on with the lymphoma in some cases.

The terms resistant or progressive disease are used when the disease does not go away or progresses (grows) while you are still being treated.

Recurrent or relapsed disease means that Hodgkin disease initially responded well to treatment and went away, but it has now come back. If Hodgkin disease returns, it may do so in the area of the body where it first started or in another part of the body. This may occur shortly after treatment or years later.

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