Childhood Cancers: Burkitt’s Lymphoma

Burkitt’s LymphomaLymphomas are cancers that develop when malignant cells infiltrate the lymphatic system.

Childhood Non-Hodgkin’s Lymphoma (NHL) develops in the lymph system, which is an integral part of the body’s immune system.

The lymph system consists of:

  • Lymph: A watery substance that contains white blood cells (lymphocytes) that the body needs to fight infections and the growth of tumours
  • Lymph Vessels: This is a network of tubes that collect lymph from different parts of the body and returns it to the blood stream
  • Lymph Nodes: These are small structures that look like beans which store the white blood cells that help the body to fight disease and infection. They are located all along the network of the lymph vessels and are mainly found in the abdomen, neck, pelvis, groin and the underarm.
  • Bone Marrow: This is the soft spongy tissue located in the centre of the large bones of the body; it makes red blood cells, white blood cells, and platelets
  • The Spleen: The spleen is an organ located on the left side of the abdomen near the stomach; it filters the blood, makes lymphocytes, stores blood cells and destroys old blood cells
  • The Thymus: The thymus is an organ in the chest, behind the breastbone, in which lymphocytes grow and multiply
  • The Tonsils: These are two small masses of lymph tissue found at the back of the throat that manufacture lymphocytes

A malignancy in the lymph system can spread very quickly throughout the rest of the body.

Lymphomas are divided into two basic types:

  •       Hodgkin’s Lymphoma
  •       Non-Hodgkin’s Lymphoma

Burkitt’s lymphoma is an aggressive form of Non-Hodgkin’s Lymphoma, also called small non-cleaved cell lymphoma. Non-cleaved means that the cells have no indentation s or folds when viewed under a microscope. It is a rapidly-growing cancer that spreads to other organs, including the brain.

It involves the B cells and occurs because of chromosome translocation of the Myc gene, which is disruptive and therefore creates abnormal cell growth.

The cell division in Burkitt’s lymphoma is one of the highest amongst tumours found in humans, and Burkitt’s has been linked to the Epstein-Barr virus.

Causes of Burkitt’s Lymphoma

According to the World Health Organisation, there are 3 variants of Burkitt’s lymphoma: endemic, sporadic, and immunodeficiency-associated:

  • Endemic Burkitt’s Lymphoma: This variant generally involves the facial bones, particularly the jaw, orbit and maxilla, and primarily refers to cases occurring in African children. The Epstein-Barr virus (EBV) is prevalent in more than 90% of endemic Burkitt’s lymphoma cases.
  • Immunodeficiency-Associated Burkitt’s Lymphoma: This variant occurs in transplant patients (most often those who have had a solid-organ transplant), patients affected with HIV, and those who have other immune deficiency disorders. HIV is not directly related to the formation of cancer cells, but approximately 30-40% of non-Hodgkin lymphomas diagnosed in HIV infected individuals are Burkitt’s lymphoma. The Epstein-Barr virus occurs in 30-40% of these cases.
  • Sporadic Burkitt’s lymphoma: This variant generally occurs in the abdomen, and in no particular climatic or geographic region. The Epstein-Barr virus I only found in approximately 20% of cases of sporadic lymphoma. Sporadic Burkitt’s lymphoma accounts for approximately 40-50% of childhood non-Hodgkin lymphomas.

Hereditary abnormalities that create an increased risk of developing Burkitt’s lymphoma include:

  • Ataxia Telangiectasia
  • Wiskott-Aldrich Syndrome
  • X-linked Lymphoproliferative Disease

Signs and Symptoms

Some of the typical signs and symptoms of Non-Hodgkin’s Lymphoma are:

  •       Abdominal pain or swelling
  •       Coughing or sore throat
  •       Night sweats
  •       Fever or recurring infection
  •       Painless swelling of the lymph nodes in the neck, chest, abdomen, groin, or underarm
  •       Trouble breathing or wheezing
  •       Trouble swallowing
  •       Unexplained high temperature/fever
  •       Unexplained weight loss
  •       Weakness, tiredness
  •       Itchy skin

Some of the above could also be a sign of a medical condition other than cancer, so please consult your doctor if your child exhibits any of these symptoms.

Tests and Diagnosis

Burkitt’s Lymphoma can be diagnosed or staged using the following tests and procedures:

  • A Biopsy: Doctors perform a small operation done under general anaesthetic whereby cells and tissues are removed and studied under a microscope by a pathologist; various laboratory tests are also done
  • Physical Exam and History: The doctor will perform an physical examination, checking your child’s general health as well as checking for anything unusual or  signs of cancer, and a complete medical history will be taken
  • Lumbar Puncture: A needle is placed into the lower back into the spinal canal and a bit of cerebral spinal fluid (CSF) is removed and tested, This is done to check the central nervous system for cancer cells.
  • Endoscopy: An endoscope (tube with a light and a lens for viewing) is inserted in a cut made in the skin or via a body opening such as the mouth, and the doctor looks at the tissues and organs inside the body to check for abnormalities. It may also contain a tool to remove lymph node or tissue samples for examination under a microscope

Other tests that may be done include blood and urine tests, x-rays, a complete blood count, blood chemistry studies, ultrasound scans, MRI scan, CT scan, a PET scan,  an immuno-histochemistry study,  a bone marrow aspiration,  or  a lymphangiogram.

Treatment options

The exact treatment option for childhood Burkitt’s lymphoma depends on the type and staging of the cancer and will be determined by your child’s doctor.

  • Chemotherapy: Most times NHL is treated with a combination of Chemotherapy drugs. These anti-cancer drugs destroy or shrink cancer cells. B-cell NHL is generally treated with about 4–8 courses of intensive chemotherapy and T-cell NHL is treatment lasts about two years.
  • Radiotherapy:  In very rare cases radiotherapy is also used. In radiotherapy, high-energy rays are used to destroy cancer cells without doing damage to the normal cells
  • Surgery: Surgery may play a limited role and is usually only done to remove a tumour that is compressing the heart, airway or other major vessels.
  • Stem Cell Transplant: This is generally combined with high-dose chemotherapy and possibly also radiation treatment to destroy all of the bone marrow, stem cells and cancerous cells. An allogeneic transplantation of healthy stem cells from a compatible donor is then done.  Stem cells may be collected from the patient, prior to intensive treatment, treated in a laboratory to kill any remaining cancer cells, put into cold storage, and later used to supplement the recovery.

Some of the treatment options may result in after-effects such as nausea, vomiting, irritation or soreness of the skin from radiation, hair loss, risk of infection, fatigue, bruising and bleeding or diarrhoea. The doctor should explain all of this to you, but if they do not, please ask them about side effects.

Awareness Ribbon Colour

The awareness ribbon colour for Burkitt’s Lymphoma is Lime Green

This article was written on behalf of Little Fighters Cancer Trust by Billi du Preez of Red Feather Scribes. Please feel free to share the article, but please respect copyright by sharing the article in its entirety, as is, including this paragraph with links at the bottom of the article. Thank You!

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