Hodgkin lymphoma

Clinical picture

Hodgkin lymphoma, also known as Hodgkin's disease, lymphogranuloma malignum and Hodgkin's morbus, is a type of cancer of the lymphatic system (lymphoma). The disease usually develops in the lymph nodes, but can also originate in the spleen, liver or bone marrow. Lymphoma means a tumour of a lymph gland. Hodgkin lymphoma spreads from one lymph node via the lymph vessels. In more than 98% of the cases, Hodgkin lymphoma develops from a certain type of white blood cell, the B lymphocyte.

There are two types of Hodgkin lymphomas:

  • Classical Hodgkin lymphoma: occurs in more than 95% of patients. This disorder is characterised by abnormal lymphoid cells, mostly B-lymphocytes. Under the microscope, these abnormal B lymphocytes resemble owl eyes, and are referred to as Reed-Sternberg cells.
  • Nodular paragranuloma (nodular lymphocyte predominant Hodgkin lymphoma or NLPHL): occurs in less than 5% of patients. This type is usually located in the lymph nodes of the neck and armpit. Distinctive for this type of lymphoma are the large popcorn-like cells; a variant of the Reed-Sternberg cells.

The British doctor Thomas Hodgkin described the disease in the 19th century as a disease where the lymphocytes began to divide malignantly. Since then, more than 50 other forms of lymphoma have been discovered, known as 'non-Hodgkin' lymphomas. Hodgkin lymphoma and non-Hodgkin lymphoma are considered as two different types of cancer because both diseases have a different course.

Symptoms

Patients diagnosed with Hodgkin’s lymphoma do not always have symptoms. Sometimes the disease is discovered by accident in someone who has no symptoms. Often the first symptom is swelling of the lymph nodes in the neck, the armpit or the groin. Usually these swellings are not painful but can be sensitive when pressed. Swelling of lymph nodes around the lungs may also occur, resulting in a feeling of tightness or pain in the area of the sternum. The ongoing presence of these complaints is characteristic for Hodgkin lymphoma.

Patients may also suffer from:

  • periods of fever, alternated by periods with normal body temperature
  • weight loss and lack of appetite
  • severe fatigue without a clear reason
  • sweating, especially at night
  • itching all over the body
  • uncommon: pain when drinking alcohol, at the site of the affected lymph nodes

Cause

Hodgkin lymphoma originates in one lymph gland because of the abnormal cell growth and division of a lymph node cell. Usually, Hodgkin lymphoma develops in a lymph node in the upper half of the body. This type of cancer is relatively rare. Every year, about 350 people in the Netherlands are diagnosed with this disease, mostly people between the ages of 20 and 35 and above 50. In men, Hodgkin lymphoma is 1.5 times more likely to occur than in women. It is not yet clear what causes the development of a Hodgkin lymphoma.

Diagnosis

The diagnosis of Hodgkin lymphoma can be confirmed by removing and examining a piece of tissue (biopsy) and a blood test. Medical professionals will focus on the presence of malignant Reed-Sternberg (RS) cells.

When the suspicion for Hodgkin lymphoma is confirmed, further research is needed to determine how far the disease has spread. Further research to determine the stage and the location of the cancer is necessary. This may consist of:

  • Detailed medical history, taking into account the symptoms of the patient.
  • Physical examination, in particular of the lymph nodes.
  • Complete blood count.
  • Ear, nose and throat (ENT) examination of the lymph nodes in the throat and nasal cavities.
  • Bone marrow examination, to determine whether there are abnormal lymphocytes in the bone marrow.
  • CT scan, to determine the extent of the disease.
  • PET-CT scan, to determine the activity of the disease.
  • MRI scan, depending on the location of the lymphoma.
  • Laboratory research into the functioning of the liver and kidneys.

Treatment

The treatment of Hodgkin lymphomas is usually curative. However, in some cases the disease will have already progressed to an advanced stage. In those cases, treatment is focused on reducing the symptoms or inhibiting the progression of disease, and is known as a palliative course of treatment. The course of the treatment is determined on the basis of the stage of Hodgkin lymphoma, the prognostic factors, age and condition.

The most commonly used treatments for Hodgkin lymphoma are:

  • Chemotherapy (treatment with cell division-inhibiting medicines)
  • Radiation (radiotherapy)
  • Immunotherapy
    • Monoclonal antibodies that target the protein CD20.

Almost all patients with Hodgkin lymphoma are treated with chemotherapy. After chemotherapy, radiation may also be required at the tumour site. In 2006, a study focussed on an experimental treatment with heavier chemotherapy that is not followed by radiation. This treatment provided such good results that since 2009, it has been the most chosen treatment option.

Hodgkin lymphoma was one of the first cancers that was cured by radiation and later on the first to be cured by combination with chemotherapy. The degree of healing is about 93%, making it one of the best cured cancers.

Additional information

Patient organisations

Clinical picture

Symptoms

Cause

Diagnosis

Treatment