Non-Hodgkin lymphoma

Clinical picture

Non-Hodgkin lymphoma (NHL) is cancer of the blood and lymph system. This term covers all types of lymph gland cancer that cannot be classified as Hodgkin's disease.

Non-Hodgkin's lymphoma is caused by a certain type of white blood cells (lymphocytes) that starts dividing uncontrollably resulting in a tumour in the lymph gland (lymphoma). Lymphocytes play an important role in the control of pathogens and the production of antibodies. The type of the lymphoma is depending on the stage of the development in which the mutation occurs.

All types of lymphoma that are not diagnosed as Hodgkin lymphoma are automatically non-Hodgkin lymphomas. Non-Hodgkin lymphomas can be located everywhere in the body, in contrast to the Hodgkin lymphoma that often growths in the neck. The tumour cells can spread throughout the body via the bloodstream and therefore more glands at a greater distance can be affected. In approximately two-thirds of patients, a non-Hodgkin lymphoma develops in a lymph node. In more than a third of patients, the disease starts elsewhere in the body, mostly in other parts of the lymphatic system. For example, in the lymph tissue in the stomach, lungs, thyroid or bone marrow.

Aggressive non-Hodgkin lymphoma

Non-Hodgkin lymphomas are classified into aggressive and non-aggressive (indolent) lymphomas. An indolent non-Hodgkin lymphoma can sometimes turn into an aggressive lymphoma after a period of time. Although aggressive lymphomas behave more violently when compared to indolent lymphomas, patients with an aggressive tumour have a much longer survival and better chance on complete recovery.

Several aggressive forms of non-Hodgkin lymphomas are:

  • diffuse large B-cell lymphoma
  • primary mediastinal B cell lymphoma
  • primary lymphoma of the skin / mycosis fungoides
  • lymphoma in immune disorders / post-transplantation lymphoma
  • Burkitt lymphoma
  • peripheral T-cell lymphoma
  • other T cell lymphomas

Indolent non-Hodkin lymphoma

If a patient has an indolent non-Hodgkin lymphoma, they may have one of the below mentioned types:

  • follicular lymphoma (FL): this is the most common non-Hodgkin lymphoma. Follicular lymphoma usually occurs in a lymph gland, but can also develop in the spleen, liver or bone marrow.
  • MALT lymphoma: especially growths in the stomach and further in the salivary glands, lungs and eyes.
  • marginal zone lymphoma: lymphoma in the glands.
  • mantle cell lymphoma: a type of tumour classified between an aggressive and indolent lymphoma.

Chronic lymphocytic leukaemia (CLL / SLL) and Waldenström disease are classified as indolent non-Hodgkin lymphomas.

In the Belgium, non-Hodgkin is the 7th most common cancer, with around 2,000 new cases each year. Particularly people above the age of 55 are affected by this disease. In men, the disease is slightly more common than in women. Approximately half of the patients with non-Hodgkin lymphoma have the indolent, non-aggressive variant.

Symptoms

The first symptoms of non-Hodgkin lymphoma are often enlarged lymph nodes in the neck, armpits or groin. These swellings are not usually painful, but can sometimes become tender when pressed. If a non-Hodgkin lymphoma develops somewhere else in the body, it may cause damage or swelling of the surrounding tissue. Complaints can consist of:

  • fever episodes
  • weight loss and lack of appetite
  • severe fatigue without a clear reason
  • sweating, especially at night

Cause

The cause of non-Hodgkin lymphoma still remains unknown. In some cases, the development of the disease is initiated by a viral infection. Research also suggests that people with a congenital immune disorder, or those who take medications that supresses the immune system, have an increased chance of developing non-Hodgkin lymphoma. In all cases, these are malignancies from cells that descend from the lymphocytes.

Diagnosis

The diagnosis of non-Hodgkin lymphoma is made based on a physical examination and blood tests. Often, additional tests are needed to determine the severity of the disease. The below test can be necessary to collect all the required information about the tumour:

  • CT- scan
  • PET-CT scan
  • MRI scan
  • a comprehensive patient history, taking into account the signs and symptoms of the patient
  • physical examination, in particular of the lymph nodes
  • blood tests
  • tissue examination of the affected lymph gland
  • ENT examination of the lymph nodes in the throat and nasal cavities
  • bone marrow test to determine whether there are abnormal lymphocytes present in the bone marrow

Treatment

The effectiveness of a treatment and the prospects for each patient are different. An aggressive non-Hodgkin lymphoma can be cured more often than its indolent variant, and thanks to the development of new medicines, the chance of survival is increasing. In patients whose treatment is effective and survive the first year, the chance on total recovery increases. Approximately 60% of patients who received a diagnosis between 2008 and 2012 were still after five years.

The choice of treatment depends on the following factors:

  • disease stage of the non-Hodgkin lymphoma
  • type of lymphoma
  • location of lymphoma
  • prognostic factors (factors that can influence the course of the disease)
  • age and physical condition of the patient

The treatment will often consist of a combination of:

  • chemotherapy: to inhibit cell division.
  • radiation/radiotherapy: locally destroying the proliferating cells.
  • immunotherapy (with rituximab) with antibodies directed against the proliferating cells. This allows the own immune cells to destroy the cancer cells.
  • targeted therapy, such as:
    • Proteasome inhibitors.
    • HDAC inhibitors.
    • BTK inhibitors.
    • PI3K inhibitors.
    • EZH2 inhibitors.
    • Nuclear export inhibitors.

Additional information

Patient organisations

Clinical picture

Symptoms

Cause

Diagnosis

Treatment

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