tumors

Burkitt lymphomas

Lymphocyte tumor

Burkitt's lymphoma is named after the surgeon D. Burkitt, who first described its symptoms around the middle of the twentieth century; a decade later, Burkitt's lymphoma was included in the class of malignant neoplasms.

Burkitt's lymphoma is considered a B-lymphocyte-derived tumor: it is a rare neoplasm with a high incidence among African children.

Causes

Burkitt's lymphoma is considered a form of non-Hodgkin's lymphoma, which is distinguished by its rapid progression; it appears, in general, following the infection caused by the Epstein-Barr virus, the same that causes the kissing disease transmitted by means of infected saliva (mononucleosis). It should be pointed out that the virus, in healthy subjects, is easily eradicated, although the hospitalization is relatively long; if the virus occurs in malnourished, disadvantaged or immunodeficient people, the consequences could have far more serious implications. In the latter case, in fact, the virus could trigger chain reactions that would cause the onset of Burkitt's lymphoma.

Risk factors

Statistics show that in the African equatorial groups Burkitt's neoplasm caused by Epstein-Barr virus occurs in 95% of lymphomas; in other cases, the same virus was found only in 20% of people with Burkitt lymphoma.

Another predisposing factor is the bone marrow transplantation in people suffering from leukemia : the sick are subjected to very consistent chemotherapy treatments, which cause a decrease and a weakening of the immune defenses. As a result, the risk of contracting Burkitt's lymphoma appears high.

The disease could also arise in case of congenital immunodeficiency, not only in the acquired one: AIDS and malaria, ataxia-telangiectasia and Wiskott-Aldrich syndrome (hereditary pathology linked to the X chromosome, which presents with eczema, infections, piarstrinopenia and diarrhea) could trigger Burkitt's lymphoma.

Dr. Burkitt formulated, at the time, another hypothesis that was (almost) immediately refuted, since he considered the malaria mosquito to be the most widely credited cause to cause lymphoma; the Anopheles mosquito transmits the protozoan that causes malaria, but it is certainly not the insect that causes a stage of immunodeficiency (in particular, inhibition of the activity of the T lymphocytes) in the affected subject. In fact, it is malaria itself that causes a progressive weakening of the immune defenses, which in turn sets the stage for Burkitt's lymphoma.

Every year, in Italy, 12, 000 people are affected by this neoplasm, which means that the disease has an incidence of 1: 5, 200 subjects; the puzzling fact is that the phenomenon is progressively increasing.

Symptoms of Burkitt's lymphoma

Burkitt's lymphoma usually begins in the maxillary and cervical lymph nodes; rarely, neoplasia also occurs at the level of the ileum, mesentery and cecum. As the lymphoma evolves, it could expand into the ovaries (or testicles), belly, marrow and brain, causing pain. In the initial stage, the disease is perceived as a consistent swelling, followed by a swelling of the jaw bones; later, lymphoma causes abundant sweating (a typical symptom of lymphomas in general), ulcers in the mucosa, malaise, sore throat and apathy.

Cellular and biochemical analysis

Following the lymphoma biopsy, we note that it is made up of macrophages that have incorporated cellular debris ( starry sky appearance): in fact, the maxillary area, analyzed by X-ray, appears jagged, and the dental roots, near the lymphoma, have been reabsorbed.

From the genetic point of view, Burkitt's lymphoma causes a translocation between chromosome 8 and chromosome 14, with subsequent involvement of the protoncogene gene MYC, a transcription factor whose target is represented by some genes that control cell replication. It is clear that, if an error occurs in the transcription, the cell cycle goes mad, causing a series of chain events that begin in Burkitt's lymphoma. To summarize the concept, Burkitt's neoplasm is a chromosomal rearrangement model that determines a cellular alteration following a new placement of a gene.

Chemotherapy and prognosis

Although Burkitt's lymphoma is quite aggressive and impetuous, it seems to respond positively to poly-chemotherapy therapies (mono-chemotherapy does not seem to be particularly indicated); it is estimated that the survival rate of Burkitt lymphoma patients treated with chemotherapy is very high and 75% of them recover completely.

Summary

To fix the concepts ...

Disease

Burkitt lymphoma, B-lymphocyte-derived tumor

Category of membership

Non- Hodgkin's lymphoma, a rare disease

Incidence

High incidence in African equatorial groups. In Italy 12, 000 new cases every year

Areas concerned

Maxillary, cervical, ileus, mesentery area, blind. It progresses evolving in the ovaries (or testicles), in the belly, in the marrow and in the brain, causing pain

Symptoms

Initial stage: swelling of the lymph nodes

Subsequently: swelling of the jaw bones, abundant sweating, mucosal ulceration, malaise, sore throat, apathy, pain

Trigger causes

Epstein-Barr virus, leukemia, chemotherapy treatments (induced immunodeficiency), AIDS and malaria, ataxia-telangiectasia and Wiskott-Aldrich syndrome (congenital immunodeficiency)

Genetic point of view

Translocation between chromosome 8 and 14 with consequent involvement of the MYC protoncogene

Therapies to combat Burkitt's lymphoma

Poly-chemotherapy. Survival rate after 5 years: 75%

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