Hodgkin's disease: Information & Hodgkin's disease specialists

Leading Medicine Guide Editors
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Leading Medicine Guide Editors

Hodgkin's disease is a relatively rare cancer of the lymphatic system. In Germany, around 160 children and adolescents and around 2,500 adults are diagnosed with the disease every year. The term Hodgkin's disease is less common today, and the disease is usually called Hodgkin's lymphoma. The prognosis is usually very good.

Here you will find further information and selected Hodgkin's disease specialists and centers.

ICD codes for this diseases: C81

Article overview

Definition

Hodgkin's disease is a cancer that affects the B cells of the immune system. These cells, also known as B lymphocytes , are altered in such a way that they multiply uncontrollably.

Initially, only the lymph nodes are affected. However, as the disease progresses, the cancer can spread to the whole body.

Fortunately, Hodgkin's disease is usually curable nowadays. This disease is one of the cancers with the best cure rates.

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

Forms

Hodgkin's lymphoma should not be confused with non-Hodgkin's lymphoma.

Non-Hodgkin's lymphomas are a group of different types of cancer of the lymphatic system. However, they do not have the Reed-Sternberg giant cells typical of Hodgkin's disease. The Reed-Sternberg giant cells develop through the fusion of degenerated B cells.

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

  • classical Hodgkin's lymphoma
  • nodular lymphocyte-predominant Hodgkin's lymphoma

Classic Hodgkin's lymphomas account for over 90 percent of Hodgkin's lymphomas. They can in turn be divided into four types:

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

Symptoms

Swelling of the lymph nodes

In most patients with Hodgkin's disease, the lymph nodes in the neck area are swollen. The swollen lymph nodes are painless and usually feel firm and rubbery. Under certain circumstances, the lymph nodes

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

are also affected.

Die Lymphknoten des Oberkörpers
Illustration of the lymph nodes in the upper part of the human body © mybox | AdobeStock

The swollen lymph nodes behind the breastbone can press on the windpipe or blood vessels if they are large enough. This leads to further symptoms such as

  • shortness of breath on exertion or even at rest with a corresponding reduction in performance, or
  • noises during breathing.

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

B symptoms

Other, rather unspecific symptoms may also occur. These are known as B symptoms. These include

  • Fever above 38 degrees Celcius,
  • night sweats,
  • unintentional weight loss,
  • itching and
  • painful lymph nodes after consuming alcohol.

All 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. He or she should clarify the cause of these symptoms.

Causes and risk factors

The reasons for the development of Hodgkin's disease are still largely unknown. However, studies have shown that the Epstein-Barr virus (EBV) could play a significant role. People who have been diagnosed with the Epstein-Barr virus have a 2.5 times higher risk of developing the disease.

There are also indications that the following groups of people have an increased risk:

  • Smokers
  • HIV-infected persons
  • People who have had an organ transplant
  • Possibly also alcohol consumers

Examination and diagnosis

Medical questioning (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 illnesses you have had,
  • cancer in your family and
  • your medication.

Physical examination and tissue sample

After taking your medical history, the doctor will palpate the lymph nodes in your

  • Neck,
  • neck,
  • groin and
  • armpit

armpit. He also examines the abdomen.

Hodgkin's disease could be the cause of swollen lymph nodes over a long period of time if other causes are out of the question. 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 removes a swollen lymph node completely. Alternatively, a biopsy can be taken from another suspected 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's lymphoma can be made.

Diagnosis of spread

Once the diagnosis has been made, it is important to find out how far Hodgkin's disease has spread in the body. Using various imaging techniques, doctors can determine exactly how the other lymph nodes or other organs are affected.

The following procedures can be used:

Not everyone can tolerate the contrast agents used in CT examinations. Magnetic resonance imaging(MRI, magnetic resonance examination) is then possible as an alternative.

If necessary, the internal organs can be assessed even more precisely using ultrasound(sonography).

Depending on the results of the previous examinations, further examinations may follow. If the result of the PET-CT is unclear, a bone marrow biopsy may be necessary.

Further examinations

The doctor will obtain a more detailed picture in order to plan the therapy optimally with as few side effects as possible. He assesses the patient's physical condition.

Further examinations are necessary for this purpose, such as

  • an electrocardiogram (ECG),
  • a heart ultrasound(echocardiography),
  • pulmonary function tests, and
  • a blood test with blood count, erythrocyte sedimentation rate and determination of various blood values such as the thyroid hormone TSH and creatinine, as well as numerous other values

The patient's ideas regarding family planning are also important for treatment planning.

General information on treatment

The chances of a cure for Hodgkin's disease are very good. In the vast majority of cases, this cancer is curable.

In principle, the therapy depends on the extent of the spread in the body. Doctors use the Ann Arbor classification for this purpose. The more extensive the spread in the body, the higher the stage.

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

Further risk factors are taken into account when planning treatment, such as

  • the number of affected lymph node areas,
  • the size and location of the affected lymph nodes,
  • the involvement of organs 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 at an early, intermediate or advanced stage .

Chemotherapy and possibly radiotherapy

A decision is made depending on the stage of the disease,

  • which type of chemotherapy is most suitable for you,
  • how many treatment cycles are necessary and
  • whether radiotherapy (also known as radiotherapy) is also required.

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

The late effects often only become noticeable many years after the therapy.

Be aware of the desire to have children

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

There are various ways to prevent infertility:

Therapy for recurrence

In the event of a relapse of the disease, also known as a recurrence, treatment is carried out in stages. Preliminary chemotherapy is followed by high-dose chemotherapy with autologous stem cell transplantation. For this purpose, the patient's own stem cells are removed before the start of therapy and then transplanted after the therapy.

If remnants of the Hodgkin's lymphoma are still detectable afterwards, these are irradiated.

If this procedure is not successful, further

  • further drugs of the so-called targeted cancer therapy as well as
  • immune checkpoint inhibitors

can be used.

Progression and prognosis

Life expectancy depends on whether

  • the therapy was successful,
  • a relapse occurs,
  • certain pre-existing conditions and
  • therapy-related late effects occur.

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

The 5-year survival rate after chemotherapy and any radiotherapy is now over 95 percent. In children, it is even better, with up to 100% of patients surviving the next 5 years. The therapy is equally successful in children and adolescents as well as adults.

Without therapy, however, Hodgkin's lymphoma leads to death.

Chemotherapy often responds so well that radiotherapy is no longer necessary. A PET-CT scan after chemotherapy is a very good way of showing whether the chemotherapy was successful. Radiotherapy may be necessary.

Recurrence rate of up to 20 percent

The disease recurs in 15 to 20 % of patients. However, such recurrences can be cured in many cases.

The 5-year survival rate is then around 50 percent.

Non-curable Hodgkin's lymphomas

Very rarely, the patient suffers from underlying diseases such as

  • ataxia teleangiectatica or
  • Nijmegen-Breakage syndrome.

These patients often tolerate chemotherapy very poorly and cannot be irradiated. The prognosis here is therefore very unfavorable.

Be aware of late effects

Hodgkin's disease therapy can cause damage to organs that only becomes noticeable after many years.

Some chemotherapeutic agents can cause damage to the lungs or heart, for example. Radiation can lead to hypothyroidism and late effects on the heart.

Typical late effects on the heart are, for example

So-called secondary malignancies are also feared late effects of radiotherapy. These are newly occurring cancers in areas that were originally irradiated.

Prevention

The causes of the development of Hodgkin's disease are not yet known. There are therefore no ways of specifically preventing the disease.

However, you should generally refrain from 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 of the end of treatment.

The guidelines therefore recommend that patients attend regular follow-up appointments after the end of treatment:

  • Every three months in the first year
  • Every six months in the second to fourth year
  • Annually from the fifth year onwards

During the follow-up examination, the doctors check

  • for a renewed outbreak of the disease and
  • the development of late effects due to chemotherapy or radiation.

In this way, appropriate therapies can be initiated at an early stage.

References

  • amboss (2021) Hodgkin-Lymphom. https://www.amboss.com/de/wissen/Hodgkin-Lymphom
  • Göbel R et al. (2018) Patientenleitlinie Hodgkin Lymphom. 2. Auflage. „Leitlinienprogramm Onkologie“ der Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e. V., der Deutschen Krebsgesellschaft e. V. und der Stiftung Deutsche Krebshilfe. https://www.leitlinienprogramm-onkologie.de/fileadmin/user_upload/Downloads/Patientenleitlinien/Patientenleitlinie_Hodgkin-Lymphom_v08-2020.pdf
  • Leitlinienprogramm Onkologie (Deutsche Krebsgesellschaft, Deutsche Krebshilfe, AWMF): Diagnostik, Therapie und Nachsorge des Hodgkin Lymphoms bei erwachsenen Patienten, Kurzversion3.0, 2020; AWMF Registernummer: 018/029 OL, https://www.leitlinienprogramm-onkologie.de/leitlinien/hodgkin-lymphom/
  • Mauz-Körholz C, Körholz D (2021) Morbus Hodgkin. In: Schmoll H-J et al. Kompendium Internistische Onkologie. Springer, Berlin.
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