Hodgkin's Disease

Definition
Hodgkin's disease — also known as Hodgkin's lymphoma — is a cancer of the
lymphatic system, which is part of your immune system.

In Hodgkin's disease, cells in the lymphatic system grow abnormally and may spread
beyond the lymphatic system. As Hodgkin's disease progresses, it compromises your
body's ability to fight infection.

Hodgkin's disease is one of two common types of cancers of the lymphatic system.
Non-Hodgkin's lymphoma, the other type, is far more common. Hodgkin's disease is
named after the British physician Thomas Hodgkin, who first described the disease in
1832 and noted characteristics that distinguish it from other lymphomas.

Advances in diagnosis, staging and treatment of Hodgkin's disease have helped to

make this once uniformly fatal disease highly treatable with the potential for full
recovery.

Symptoms

Many initial signs and symptoms may be similar to those of the flu, such as fever,
fatigue and night sweats. Eventually, tumors develop.

Hodgkin's disease symptoms may include:

 Painless swelling of lymph nodes in your neck, armpits or groin

 Persistent fatigue
 Fever and chills
 Night sweats
 Unexplained weight loss — as much as 10 percent or more of your body weight
  Loss of appetite
 Itching

Causes

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.

Risk factors
The following are risk factors for Hodgkin's disease:
  Age. People between the ages of 15 and 40, as well as those older than 55, are
most at risk of Hodgkin's disease.
 Family history. Anyone with a brother or a sister who has the disease faces an
increased risk of developing Hodgkin's, though this may be due to similar
environmental exposures rather than genetic factors.
 Sex. Males are slightly more likely to develop Hodgkin's.
 Past Epstein-Barr infection. People who have had illnesses caused by the
Epstein-Barr virus, such as infectious mononucleosis, are more likely to
develop Hodgkin's disease than people who haven't had a past Epstein-Barr
infection.
 Compromised immune system. Having a compromised immune system,
such as from HIV/AIDS or from having an organ transplant requiring
medications to suppress your immune response, also appears to put you at a
greater risk of Hodgkin's disease.

Tests and diagnosis

Many people with Hodgkin's disease experience no classic symptoms. Sometimes, an
abnormality found on a chest X-ray done for nonspecific symptoms leads to an
eventual diagnosis of Hodgkin's disease.

Because the symptoms of Hodgkin's are similar to those of other disorders, such as
influenza, the disease can be difficult to diagnose. Some distinctive characteristics
help diagnose Hodgkin's disease, and these include:

 Orderly spread. The pattern of spread is orderly, progressing from one group
of lymph nodes to the next.
 Only rare 'skipping.' The disease rarely skips over an area of lymph nodes as
it spreads.

Biopsy can reveal changes

A tissue sample (biopsy) of an enlarged lymph node is needed to make the diagnosis.
The pathologist looks for changes in the normal lymph node architecture and cell
characteristics, including the presence of Reed-Sternberg cells. The affected lymph
nodes may contain only a few of these malignant cells.

Other procedures that may be valuable in evaluating Hodgkin's disease include:

 X-ray
 Computerized tomography (CT) scan
 Magnetic resonance imaging (MRI)
 Gallium scan, which uses a radioactive substance given intravenously that
indicates areas in your body where Hodgkin's disease may be present
 Positron emission tomography (PET) scan
 Bone marrow biopsy
 Blood tests

If the disease is predominantly in your abdomen, you may need exploratory surgery to
help define the extent of the disease.
Staging Hodgkin's disease
Once the diagnosis is confirmed, doctors "stage" the disease. Staging is how doctors
judge the extent of the disease, which will likely affect your treatment options.

 Stage I. The cancer is limited to one lymph node region or a single organ.
 Stage II. In this stage, the cancer is in two different lymph nodes, but is
limited to a section of the body either above or below the diaphragm.
 Stage III. When the cancer moves to lymph nodes both above and below the
diaphragm, but hasn't spread from the lymph nodes to other organs, it's
considered stage III.
 Stage IV. This is the most advanced stage of Hodgkin's disease. Stage IV
Hodgkin's disease affects not only the lymph nodes but also other parts of your
body, such as the bone marrow or your liver.

Additional definitions of the cancer

Additionally, your doctor may use the letters A, B, E and S to help define the extent
of your cancer and the treatment needed:

 The letter A means that you don't have any significant symptoms as a result
of the cancer.
 The letter B indicates that you may have significant signs and symptoms,
such as a persistent fever greater than 100 F with no other known cause,
unintended weight loss of more than 10 percent of your body weight or severe
night sweats.
 The letter E stands for extranodal, which means that the cancer has spread
beyond your lymph nodes.
 The letter S designates a cancer that has spread into your spleen.

The letters B, E and S indicate potentially more serious disease.

Treatments and drugs

The most important factor in Hodgkin's disease treatment is the stage of the disease.
The number and regions of lymph nodes affected and whether only one or both sides
of your diaphragm are involved also are important considerations. Other factors
affecting decisions about treating this disease include:

 Your age
 Your symptoms
 Whether you're pregnant
 Your overall health status

The goal of treatment is to destroy as many malignant cells as possible and bring the
disease into remission. As many as 95 percent of people with stage I or stage II
Hodgkin's disease survive for five years or more with proper treatment. The five-year
survival rate for those with widespread Hodgkin's disease is about 60 percent to 70
percent.

Treatment options include:

Radiation
When the disease is confined to a limited area, radiation therapy may be the treatment
of choice. It's typical to radiate the affected nodes and the next area of nodes where
the disease might progress. The length of radiation treatment varies depending on the
stage of the disease. Radiation therapy may be used alone, but is commonly used in
conjunction with chemotherapy. If you relapse after radiation therapy, chemotherapy
becomes necessary.

Some forms of radiation therapy may increase your risk of other forms of cancer, such
as breast or lung cancer. The risk of breast cancer from standard-dose radiation is
even higher for girls and women treated when they were younger than 30; the risk is
now considered too high for standard radiation therapy to be considered in this group.
Most children with Hodgkin's disease are treated with combination chemotherapy, but
may also receive low-dose radiation therapy.

Chemotherapy
When the disease progresses and involves more lymph nodes or other organs,
chemotherapy is the preferred treatment. Chemotherapy uses specific drugs in
combination to kill tumor cells.

A major concern with chemotherapy is the possibility of long-term side effects and
complications, such as heart damage, lung damage, liver damage and secondary
cancers, such as leukemia.

Although severe effects aren't common, an ongoing effort is being made into finding
equally effective regimens with less toxicity. Drug regimens have been developed that
substantially diminish the likelihood of long-range, life-threatening complications,
including acute leukemia in people who have received multiple courses of
chemotherapy and radiation therapy.

Chemotherapy regimens are commonly referred to by their initials, such as:

 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.

Bone marrow transplant

If the disease recurs after an initial chemotherapy-induced remission, high-dose
chemotherapy and transplantation of your own (autologous) bone marrow or
peripheral stem cells may lead to prolonged remission. Peripheral stem cells are bone
marrow cells mobilized from the bone marrow into the bloodstream.

Because high doses of chemotherapy destroy bone marrow, your own marrow or
peripheral blood stem cells are collected before treatment and frozen. You'll undergo
chemotherapy, and then your own cells, which have been protected from the effects of
the treatment, are injected back into your body.

Coping and support

A diagnosis of cancer can be extremely challenging. Remember that no matter what
your concerns or prognosis, you're not alone. The following strategies and resources
may make dealing with cancer easier:

 Know what to expect. Find out everything you can about your cancer — the
type, the stage, your treatment options and their side effects. The more you
know, the more active you can be in your own care. In addition to talking with
your doctor, look for information in your local library and on the Internet. The
National Cancer Institute answers questions from the public and can be
reached at 800-422-6237. Or you can contact the American Cancer Society at
800-227-2345.
 Be proactive. Although you may feel tired and discouraged, don't let others
— including your family or your doctor — make important decisions for you.
Take an active role in your treatment.
 Maintain a strong support system. Having a support system and a positive
attitude can help you cope with any issues, pain and anxieties that might
occur. Although friends and family can be your best allies, they sometimes
may have trouble dealing with your illness. If so, the concern and
understanding of a formal support group or others coping with cancer can be
especially helpful.

Although support groups aren't for everyone, they can be a good source for
practical information. You may also find you develop deep and lasting bonds
with people who are going through the same things you are.

 Set reasonable goals. Having goals helps you feel in control and can give you
a sense of purpose. But don't choose goals you can't possibly reach. You may
not be able work a 40-hour week, for example, but you may be able to work at
least part time. In fact, many people find that continuing to work can be
helpful.
 Take time for yourself. Eating well, relaxing and getting enough rest can help
combat the stress and fatigue of cancer. Also, plan for the downtimes when
you may need to rest more or limit what you do.
 Stay active. Receiving a diagnosis of cancer doesn't mean you have to stop
doing the things you enjoy or normally do. For the most part, if you feel well
enough to do something, go ahead and do it. It's important to stay involved as
much as you can.