Hodgkin's Disease: Information & Hodgkin's Disease Specialists

Hodgkin's disease is a type of cancer that affects the B cells of the immune system. These cells, also known as B lymphocytes, undergo changes that cause them to multiply uncontrollably.

Initially, only the lymph nodes are affected. As the disease progresses, however, the cancer can spread throughout the body. 

Fortunately, Hodgkin’s disease is usually curable today. It is one of the cancers with the highest cure rates.

The English physician Thomas Hodgkin first described the disease in 1832. It was therefore named after him.

Hodgkin lymphoma should not be confused with non-Hodgkin lymphomas.

Non-Hodgkin lymphomas are a group of various types of cancer of the lymphatic system. However, they do not contain the Reed-Sternberg giant cells typical of Hodgkin’s disease. Reed-Sternberg giant cells are formed by the fusion of degenerated B cells.

Hodgkin’s disease is classified into two main groups based on its appearance under the microscope:

• classical Hodgkin lymphoma
• nodular lymphocyte-predominant Hodgkin lymphoma

Classic Hodgkin lymphomas account for over 90 percent of all Hodgkin lymphomas. They can, in turn, be subdivided into four types:

• Lymphocyte-rich type
• Nodular sclerosing type
• Mixed-cell type
• Lymphocyte-poor type

Swollen Lymph Nodes

In most patients with Hodgkin’s disease, the lymph nodes in the neck are swollen. The swollen lymph nodes are painless and usually feel firm and rubbery. In some cases, the lymph nodes

• behind the breastbone,
• in the groin,
• in the armpits, and
• in the abdomen

.

If the swollen lymph nodes behind the sternum are large enough, they can press on the trachea or blood vessels. This leads to additional symptoms such as

• shortness of breath during exertion or even at rest, accompanied by a corresponding decrease in physical performance, or
• noises during breathing. 

However, Hodgkin’s disease can affect any part of the body. Therefore, completely different symptoms may also occur, such as

• skin rash,
• seizures,
• back pain, or
• bone fractures.

B-symptoms

In addition, other, more nonspecific symptoms may occur. These are referred to as “B symptoms.” They include

• a fever above 38 degrees Celsius,
• night sweats,
• unintentional weight loss,
• itching, and
• tender lymph nodes after drinking alcohol. 

All of these symptoms can also have other causes, but you should always take them seriously. If your symptoms do not improve within two weeks, you should see a doctor. The doctor should determine the cause of these symptoms.

The causes of Hodgkin’s disease are still largely unknown. However, studies have shown that the Epstein-Barr virus (EBV) may play a significant role. People who test positive for the Epstein-Barr virus have a 2.5 times higher risk of developing the disease.

In addition, there is also evidence that the following groups of people are at increased risk:

• Smokers
• People infected with HIV
• People who have undergone an organ transplant
• Possibly also people who consume alcohol

Medical Interview (Medical History)

At the beginning of the examination, the doctor will ask you about

• the nature and duration of your symptoms,
• your dietary and lifestyle habits,
• any past illnesses,
• a history of cancer in your family, and
• the medications you are taking.

Physical Examination and Tissue Sample

Following the medical history interview, the doctor will palpate the lymph nodes

• the neck,
• back of the neck,
• groin, and
• armpits

. He also examines the abdomen.

Hodgkin’s disease could be the cause of lymph nodes that have been swollen for an extended period of time if other causes can be ruled out. Lymph nodes can also swell as a result of an infection or for other reasons. 

If Hodgkin’s disease is suspected, a tissue sample must be examined. To do this, the doctor completely removes a swollen lymph node. Alternatively, the doctor may take a biopsy from another suspicious organ. The tissue sample is examined histologically under a microscope.

If the typical Sternberg-Reed cells or Hodgkin cells are found, a diagnosis of Hodgkin lymphoma can be made.

Staging

After the diagnosis, it is necessary to determine how far Hodgkin’s disease has spread throughout the body. Using various imaging techniques, doctors can precisely determine the extent of involvement in other lymph nodes or organs.

The following procedures may be used:

• A computed tomography (CT) scan of the neck, chest, and abdomen using contrast dye
• A chest X-ray
• A positron emission tomography-computed tomography (PET-CT) scan

Not everyone can tolerate the contrast agents used in CT scans. In such cases, magnetic resonance imaging (MRI) is a possible alternative.

If necessary, an ultrasound examination (sonography) can be used to assess the internal organs in even greater detail. 

Depending on the results of the previous tests, further tests may be required. If the PET-CT results are inconclusive, a bone marrow biopsy may be necessary.

Further Tests

To plan the best possible treatment with as few side effects as possible, the doctor will gain a more detailed understanding of the patient’s condition. The doctor assesses the patient’s physical condition.

For this purpose, additional tests are necessary, such as

• an electrocardiogram (ECG),
• a heart ultrasound (echocardiography),
• lung function tests, as well as
• a blood test including a complete blood count, erythrocyte sedimentation rate, and measurement of various blood levels such as the thyroid-stimulating hormone (TSH) and creatinine, as well as numerous other values

The patient’s plans regarding family planning are also important for treatment planning.

The prognosis for Hodgkin’s disease is very good. In the vast majority of cases, this cancer is curable. 

In general, treatment depends on the extent of the disease’s spread throughout the body. To determine this, doctors use the Ann Arbor classification. The rule is: the more the disease has spread throughout the body, the higher the stage.

• Stage 1: Only one lymph node region is affected, for example, only the lymph nodes in the neck
• Stage 2: Multiple lymph node regions on one side of the diaphragm are affected. Example: The lymph nodes in the neck and those behind the sternum
• Stage 3: Lymph node regions on both sides of the diaphragm, as well as one organ, are affected. Example: The lymph nodes behind the sternum and in the abdominal cavity, as well as the liver
• Stage 4: One or more organs are affected, including the liver and/or the bone marrow

When planning treatment, additional risk factors are taken into account, such as

• the number of affected lymph node regions,
• the size and location of the affected lymph nodes,
• organ involvement, and
• the erythrocyte sedimentation rate (ESR).

Based on the Ann Arbor stage and the risk factors, it is possible to determine whether the disease is in an early, intermediate, or advanced stage.

Chemotherapy and, if necessary, radiation therapy

Depending on the stage of the disease, a decision is made

• which type of chemotherapy is most suitable for you,
• how many treatment cycles are necessary, and
• whether additional radiation therapy is required.

In the past, the affected lymph node regions and organs were irradiated in addition to chemotherapy (radiochemotherapy). Today, radiation therapy is used more sparingly. Young patients, in particular, have an increased risk of late effects from radiation. These include

• treatment-related secondary malignancies (cancers)
• and cardiovascular diseases.

These long-term effects often do not become apparent until many years after treatment.

Consider the Desire to Have Children

Cancer treatment can also damage healthy cells such as eggs and sperm. If the patient still wishes to have children, they must be informed about the treatment’s impact on fertility.

There are various options for preventing infertility:

• Protective medications during treatment for women
• Retrieval and freezing of eggs and ovarian tissue
• Freezing of sperm following sperm donation or retrieval from testicular tissue

Treatment for Recurrence

In the event of a relapse, also known as recurrence, treatment is carried out in stages. Preparatory chemotherapy is followed by high-dose chemotherapy with autologous stem cell transplantation. For this purpose, the patient’s own stem cells are collected before treatment begins and reinfused after treatment.

If residual Hodgkin lymphoma is still detectable afterward, it is treated with radiation therapy. 

If this approach is not successful,

• additional drugs used in so-called targeted cancer therapy, as well as
• immune checkpoint inhibitors

may be used.

Life expectancy depends on whether

• the treatment was successful, 
• whether a recurrence occurs, 
• certain pre-existing conditions are present, and
• whether treatment-related long-term effects occur.

The course and prognosis for Hodgkin’s disease are very good

The 5-year survival rate following chemotherapy and, if necessary, radiation therapy is now over 95 percent. In children, the outlook is even better, with up to 100% of patients surviving the next 5 years. Treatment is equally successful in children, adolescents, and adults.

Without treatment, however, Hodgkin lymphoma is fatal.

Often, patients respond so well to chemotherapy that radiation therapy is no longer necessary. A PET-CT scan performed after chemotherapy is an excellent way to determine whether the chemotherapy was successful. In some cases, radiation therapy may be necessary. 

Recurrence rate of up to 20 percent

In 15 to 20% of patients, the disease recurs. In many cases, however, such recurrences can be cured.

The 5-year survival rate in these cases is approximately 50 percent.

Incurable Hodgkin lymphomas

Very rarely, the patient suffers from underlying conditions such as

• ataxia telangiectatica or
• Nijmegen Breakage Syndrome.

These patients often tolerate chemotherapy very poorly and cannot undergo radiation therapy either. Therefore, the prognosis in these cases is very poor.

Be aware of long-term effects

Treatment for Hodgkin’s disease can cause damage to organs that may not become apparent until many years later.

Some chemotherapy drugs, for example, can cause damage to the lungs or the heart. Radiation therapy can lead to hypothyroidism as well as long-term effects on the heart.

Typical long-term effects on the heart include, for example

• heart valve defects,
• coronary heart disease, and
• cardiac arrhythmias.

Another feared long-term effect of radiation therapy is the development of so-called secondary malignancies. These are new cancers that develop in areas that were originally treated with radiation. 

The causes of Hodgkin’s disease are not yet known. Therefore, there are no specific ways to prevent the disease.

However, you should generally avoid smoking and excessive alcohol consumption. This also reduces the risk of developing other cancers and cardiovascular diseases.

However, early detection of a recurrence is of the utmost importance.

• 70 percent of recurrences occur within the first two and a half years, and
• 90 percent occur within five years after the end of treatment.

Therefore, the guidelines recommend that patients attend regular follow-up appointments after treatment ends:

• Every three months in the first year
• Every six months in the second through fourth years
• Annually starting in the fifth year

During the follow-up examination, doctors check

• for any recurrence of the disease and
• for the development of long-term effects resulting from chemotherapy or radiation therapy.

This allows for the early initiation of appropriate treatments.