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Lymphome de Burkitt
What is it ?
Burkitt’s lymphoma is a relatively uncommon type of lymphoma.
It is a so-called non-Hodkin lymphoma. The latter corresponds to a cancer developing from lymphocytes (white blood cells). These cells are part of the immune system in humans and are involved in many defense reactions of the body against bacterial, viral, parasitic infections, etc.
This pathology affects specific blood cells (white blood cells), so it is a hemopathy.
Burkitt’s lymphoma is a form of mature B-cell non-Hodkin lymphoma. These B cells form a special type of lymphocyte. The development of B lymphocytes takes place in the bone marrow from a stem cell (or mother cell): the hematopoietic cell. The mature B lymphocyte stage is the ultimate stage in the development of these cells in the bone marrow. This results in a migration of B cells to the secondary lymphoid organs: the spleen, the lymph nodes, lyphoid formations associated with the mucous membranes (MALT). (1)
This rare form of lymphoma is mainly found in Central Africa but can also affect other regions of the world.
Three forms of Burkitt’s lymphoma are present:
– the endemic form: found mainly in sub-tropical Africa. This form is very strongly linked to the Epstein Barr virus (EBV) and is characterized by a tumor of the jawbones (parts of the skeleton of the face).
– the sporadic form: this time, not linked to the EBV virus and developing mainly in the abdomen, more rarely in the ENT sphere or even in other regions of the Central Nervous System. In the latter case, the appearance of tumor meningitis or even neuronal paralysis may be observed. (2)
It is also possible to distinguish a third type: Burkitt’s lymphoma linked to immunodeficiency. This usually manifests itself in people affected by the Human Immunodeficiency Virus (HIV). The development of lymphoma is notably the consequence of antiretroviral treatment associated with HIV infection. (4)
In addition, children are more susceptible to the development of this blood disease.
Symptoms
The symptoms associated with Burkitt’s lymphoma are mainly abdominal (cecum, medenter). (3)
The symptoms of this type of lymphoma are different depending on the type.
– The endemic type is the cancer most frequently found in children in Africa. It affects more particularly the jaw and the facial bones (jawbone). Facial involvement represents 75% of endemic Burkitt lymphoma cases and results in indolent tumors.
– The sporadic type affects the abdomen, ovaries, testes, kidneys, pleura (membrane covering the lungs), the Central Nervous System or even the breasts. (4)
The origins of the disease
Again, the origins of the disease differ depending on the type of Burkitt’s lymphoma.
An Ebstein-Barr Virus (EBV) infection is often the main cause. Indeed, this infection is caused by a virus which causes harmful consequences at the level of the ganglia. As the lymph nodes are one of the secondary lymphoid organs into which mature B cells migrate, this infection can then be the source of the development of Burkitt’s lymphoma.
This origin of the disease is especially characteristic of the endemic type of Burkitt’s lymphoma. It can also be for the sporadic type, but of lesser incidence.
The Human Immunodeficiency Virus (HIV) may also be responsible for the development of this form of lymphoma. Indeed, subjects affected by HIV are more subject to the development of an EBV infection. In this sense, the majority is exposed to the development of Burkitt’s lymphoma.
Risk factors
The risk factors associated with the development of Burkitt lymphoma are: (5)
– infection with the Ebstein-Barr Virus (EBV);
– infection with the Human Immunodeficiency Virus (HIV);
– infection with the Hepatitis C virus (HCV);
– infection with Hepatitis B virus (HBV);
– infection with papillomavirus (HPV).
Prevention and treatment
The diagnosis for Burkitt’s lymphoma is a biopsy of a cell mass or bone marrow that can testify to the presence of tumor cells.
Associated with this biopsy, the use of medical imaging (ultrasound, scanner) is often essential for carrying out the assessment.
In addition, a prior diagnosis in children of other types of tumors, such as Wilms tumors or even neuroblastomas, may be closely linked to the presence of Burkitt’s lymphoma.
The management of this type of lymphoma must be effective as soon as possible. It is done in centers specializing in cancerology.
The treatment of Burkitt’s lymphoma is defined mainly by intensive chemotherapy for a few months.
The lack of a certain interest in the use of rituximab (chimeric monoclonal antibody) constitutes a major concern to date as to the therapeutic urgency for this type of lymphoma.
Support must be rapid and appropriate to the subject. In fact, at the start of anti-tumor treatment, metabolic problems are frequent in the subject suffering from lymphoma.
Despite the heaviness of the treatment, its duration is short and the long-term side effects are less. The current cure rate is on average 80-90%. (2)