Definition and Overview

Follicular malignant lymphoma is a type of non-Hodgkin lymphoma (NHL) that most commonly presents as painless, slow-growing lymph nodes tumours.

Lymphoma is a cancer of the lymphatic system. It starts in white blood cells called lymphocytes, which play a crucial role in the immune system and responsible for fighting diseases. When abnormal lymphocytes multiply and collect in one area, they cause the lymph nodes to swell and impair the immune system.

There are two types of lymphomas; Hodgkin and non-Hodgkin. The main difference between the two is the specific lymphocyte involved. The condition is Hodgkin lymphoma if a specific type of abnormal cell, called Reed-Sternberg cell, is present. Otherwise, it is classified as NHL.

20% of NHLs are follicular malignant lymphomas. The condition typically does not produce signs or symptoms in the early stages and is thus often diagnosed when it has already progressed. There is no cure for it, but many patients experience long-lasting remission with radiation and chemotherapy.

Causes of Condition

Follicular malignant lymphoma occurs when the body makes too many abnormal lymphocytes that grow and divide until they form tumours and cause the lymph nodes to enlarge. In general, all types of cancer develop due to DNA mutations that turn on oncogenes (genes that help cells grow and stay alive) or turn off tumour suppressor genes (genes that make cells die at the right time and help control cell division).

In the case of follicular malignant lymphoma, the risk of DNA mutations and impaired cell replication can be increased by the following:

  • Viruses, including human T-cell lymphotropic virus type 1, Epstein-Barr virus, and herpes virus associated with Kaposi sarcoma – It is believed that such viruses force the immune system to constantly produce new lymphocytes to fight infections, which increases the chances for mistakes in their DNA.

  • Congenital immune deficiencies and infection with the human immunodeficiency virus (HIV).

  • Immune deficiencies caused by diseases and medical procedures, such as organ transplantations and treatment with certain drugs. Lymphomas that develop in patients with a compromised immune system are mostly diffuse or high-grade.

Other risk factors include older age (most people diagnosed with the condition are aged 60 and older) and exposure to certain chemicals including insect killers.

Key Symptoms

Follicular malignant lymphoma is mostly asymptomatic in the early stages. Aside from painless swelling in the groin, armpit, and neck, no other signs or symptoms are usually observed.

As the condition progresses, various systemic symptoms, as well as signs of bone marrow dysfunction, become noticeable. These include:

  • Fever

  • Weight loss

  • Drenching night sweats

  • Anaemia

  • Leukopenia, or reduced number of white cells in blood

  • Thrombocytopenia, or abnormally low amount of platelets

  • Fatigue

  • Chronic infection

  • Shortness of breath

Who to See and Types of Treatments Available

Follicular malignant lymphoma is diagnosed with routine laboratory tests, imaging tests, and an excisional biopsy.

  • Complete blood cell count (CBC) and peripheral blood smear - Used to check for abnormalities in blood cells.

  • Liver function tests

  • Uric acid, lactate dehydrogenase, and creatinine level tests

  • Positron emission tomography (PET) and computed tomography (CT) scan of the chest, pelvis, and abdomen - Used to look for tumours and determine if they are causing any obstructions. These tests are also used to determine the number of affected lymph nodes and whether cancer cells have spread to other parts of the body.

  • Excisional biopsy - Involves the surgical removal of an enlarged lymph node and checking it for lymphoma cells. It is performed either under local or general anaesthesia.

  • X-rays

The results of the above tests can confirm a follicular malignant lymphoma diagnosis and the stage of the disease. The stage of cancer describes how many groups of lymph nodes are affected, their location in the body, and whether the liver and bone marrow are also affected.

  • Stage 1 - One group of lymph nodes is affected

  • Stage 2 - Two groups of lymph nodes located on either upper or lower half of the body are affected

  • Stage 3 - Affected lymph nodes are found above and below the diaphragm

  • Stage 4 - Cancer has spread to other organs, such as the lungs, bones, bone marrow, and liver

Stages 1 and 2 follicular malignant lymphomas are classified as localised cancer while stages 3 and 4 are classified as advanced or widespread lymphomas.

Treatment is almost always not necessary in the early stages of the condition. If the patient shows no or very few symptoms, an approach referred to as “active surveillance” or “watchful waiting” is recommended. With this approach, patients are closely monitored through regular check-up visits and periodic laboratory and imaging tests. Active treatment is started when signs that the disease is progressing begin to show.

The standard treatments for follicular malignant lymphoma are radiation and chemotherapy; both are proven to provide long-lasting remission especially in patients with limited disease. Those with widespread cancer are commonly treated with a combination of chemotherapy drugs and monoclonal antibodies.

Many patients experience relapse several years after successful treatment. In such cases, they may undergo the same treatment they had before or their doctor may use additional therapies to improve treatment outcomes. Stem cell transplantation is also used but is generally reserved for younger patients with relapsed, recurrent, or refractory disease.

The overall five-year survival rate for patients with follicular malignant lymphoma is up to 77%. However, the rate goes down to just 36% for older patients and those who have other medical conditions.

References:

  • Tan D, Horning SJ, Hoppe RT, Levy R, Rosenberg SA, Sigal BM, et al. Improvements in observed and relative survival in follicular grade 1-2 lymphoma during 4 decades: the Stanford University experience. Blood.

  • Press OW, Unger JM, Rimsza LM, Friedberg JW, Leblanc M, Czuczman MS, et al. Phase III Randomized Intergroup Trial of CHOP Plus Rituximab Compared With CHOP Chemotherapy Plus 131Iodine-Tositumomab for Previously Untreated Follicular Non-Hodgkin Lymphoma: SWOG S0016. J Clin Oncol.

  • Ekström Smedby K, Vajdic CM, Falster M, et al. Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. Blood. 2008 Apr 15. 111 (8):4029-38.

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