Berkitt's lymphoma

Description:

Berkitt's lymphoma is widespread in the countries of the Central Africa, Oceania, is preferential at children at the age of 3-7 years. Now the disease is registered in Europe, the USA not only at children, but also at adults with a heavy immunodeficiency, including AIDS.

Symptoms of the Lymphoma of Berkitt:

Classical option of a tumor - single or multiple new growths of a jaw which can diffuzno extend to sialadens, a thyroid gland and other bodies. The tumor quickly grows, infiltrirut soft tissues, destroys bones, causing deformation of a nose, disturbance of breath and swallowing, dedentition, deformation of jaws. As at any infectious disease at patients it is possible to allocate an all-toxic syndrome in an onset of the illness with fever and emergence on this background quickly increasing in volume of a tumoral node. The generalized course of a disease leads to damage of vertebras, femoral and haunch bones that is followed by pathological fractures, squeezing of roots of a spinal cord, disturbances of functions of pelvic bodies, paresis and paralyzes.

Especially it is necessary to allocate an abdominal form which differs in quickly progressing current and extremely difficult diagnosis. In this case can be struck: pancreas, liver, intestines, ovaries, kidneys. In all bodies the infiltrates consisting of tumor lymphoid cells come to light. Patients have abdominal pains, dispepsichesky symptoms; development of intestinal impassability, jaundice, exhaustion, fever is possible.

At the same time also favorable disease with long remissions, the proceeding months is observed. At timely diagnosis and intensive complex care perhaps full treatment of the patient.

One of options of a malignant course of infections, caused by Epstein-Barre's virus, the nazofarinzealny carcinoma - a malignant tumor which is localized on the lateral party of a nasal cavity is or in the field of the average nasal course, quickly burgeons in a nasopharynx and gives metastasises in submaxillary lymph nodes. Primary symptom - disturbance of nasal breath. Then there are mucopurulent allocations from a nose in which there can be a blood impurity. At a rinoskopiya the tumor has a hilly appearance or reminds a polyp. In process of growth of a tumor auditory acuity decreases, patients are disturbed by a sonitus, pains irradiate to the temporal area. At a tumor arrangement on a sidewall of a throat the hearing disorder, trifacial branch neuralgia are observed. Quickly also all-toxic symptoms develop. It should be noted that the nasopharyngeal carcinoma exclusively seldom creates the remote metastasises. As a rule, the tumor burgeons in a nasopharynx with destruction of a bone tissue and metastasises in lymph nodes. With disturbances of immune system Epstein-Barre's virus is capable to cause development of a leukoplakia of mucous membranes of language, an oral cavity in persons (especially to a campaign of a bite of teeth), a red border of lips (especially under lip). Also mucous membrane of a vulva can be affected, less often the centers are localized on a balanus at men, on a clitoris, in a vagina and on a neck of uterus at women. There are sites of white or whitish-gray color with a diameter of 2-3 cm. Border of these sites are quite accurately expressed. Folds of a mucous membrane smooth out, on affected areas the burning sensation, numbness, disturbance of flavoring feelings appears. When traumatizing these sites bleeding is noted.

There are data on participation of VEB in developing of other limfoproliferativny diseases.

Reasons of the Lymphoma of Berkitt:

The etiology of lymphoma remains to the unknown. Among etiological factors such, the general traditionally are considered for all neoplastic diseases, factors as the ionizing radiation, chemical carcinogens, unfavorable conditions of the environment. In addition it is necessary to stop for roles of viruses as in some cases development of lymphoma the expressed interrelation between influence of a virus and tumoral growth is traced. It was so shown that at children of patients with a local African lymphoma of Berkitt in 95% of cases infection with a virus Epstein - Barrel takes place.

Epstein-Barre's virus is DNA the containing virus for the first time found in 1964 in the patient from Africa suffering from Berkitt's lymphoma. Epstein-Barre's virus can is specific to contact a receptor on a superficial membrane of V-lymphocytes (a glycoprotein of CD-21 which is also a receptor for C3b - a complement component). Communication with a virus affects the V-lymphocyte as initial polyclonal mitogenetic stimulation, partly responsible for increase in an expression of a receptor (CD-23) for the V-cellular growth factor. Besides, the viral infection transforms some infected V-cells doing them capable to prolifelirovat continuously in culture. Possibly these cells are capable to a malignant progression.

Epstein-Barre's virus is the reason of an infectious mononucleosis, a state clinically similar to an abortal current of a lymphoma. The infectious mononucleosis represents evident model in which the proliferation of the V-lymphocytes capable induced by Epstein-Barre's virus to a malignant progression, is suppressed with the normal T-cellular answer.

It is shown that Epstein-Barre's virus induces development of a fatal lymphoma in the men coming from families with the traced anamnesis of insufficiency of immune system. Such patients have the genetic defect linked to X-chromosome (X q24-q27). This genetic defect of immunoregulation is the reason of deficit of the immune response directed against Epstein's virus - Barrel.

Thus, despite the visible monoetilogichesky nature of a tumor traced at some lymphoma, nevertheless by thorough consideration of a question the combination of several causative factors attracts attention.

Treatment of the Lymphoma of Berkitt:

Apply Cyclophosphanum intravenously on 30-40 mg/kg repeated courses (single introduction each 10-14 days, only 2-3 injections on a course). Introduction of Embichinum intravenously in a dose of 0,2-0,3 mg/kg/day within 4-5 days in a row with repeated courses in 3-4 weeks is shown. The short-term effect of Vincristinum, sarcolysine, a methotrexate is gained.

In complex therapy use also immunomodulators. So, in treatment of the malignant diseases caused by VEB usually apply high doses of recombinant interferon (for example, in therapy of lymphoma - роферон on 15 million ME intramusculary daily, then every other day, further 2 times a week not less than half a year).

In recent years the chemotherapy is combined with antiviral drugs (ганцикловир, foskarnt), which can improve results of treatment. The possibility of use of an adefovir, a brivudin, a tsidofovir, a lobukavir is studied.

Tests of immunoglobulins from blood of the person, supressiruyushchy WEB лимфопролиферацию are carried out.

At early purpose of chemotherapy perhaps absolute recovery. In far come stages of a disease and in particular at a nasopharyngeal carcinoma apply surgical methods of a flecheniye.