Berkitt's lymphoma – the tumor of high degree of a zlokachestvennost relating to group of nekhodzhkinsky lymphoma. Primary center can be localized not only in lymph nodes, but also in various bodies and fabrics: in bones of a facial skeleton, a stomach, intestines, mammary glands etc. Aggressive local growth and bystry metastasis is characteristic. Berkitt's lymphoma is shown by deformation of the person and bones of a skeleton, fever, jaundice, dyspepsia and neurologic frustration. The diagnosis is exposed on the basis of survey, the given a biopsy and other researches. Treatment – chemotherapy, radiation therapy, surgeries.
Berkitt's lymphoma – extremely aggressive malignant new growth, mainly ekstranodalny localization; kind of V-cellular lymphoma. Quickly extends out of limits of lymphatic system then internals, TsNS and marrow are involved in process. The endemic form of a lymphoma of Berkitt makes about 80% of total of cases, sporadic – about 20%. From an endemic form the children living in South Africa and New Guinea mainly suffer. The peak of incidence is the share of age of 4-7 years, the disease is found in boys twice more often.
The sporadic form of a lymphoma of Berkitt comes to light in the different countries of the world, makes slightly more than 1% of total of the lymphoma diagnosed in the USA and countries of Western Europe. Average age of patients about 30 years, a ratio of men and women – 2,5:1. Both forms of a lymphoma of Berkitt quite often arise against the background of AIDS. Most of patients are infected with Epstein's virus - Barrel. In Russia this disease practically does not meet. Treatment of a lymphoma of Berkitt is performed by experts in the sphere of oncology, hematology, otolaryngology, stomatology, maxillofacial surgery, ophthalmology, orthopedics, gastroenterology, abdominal surgery etc.
Etiology and pathogenesis of a lymphoma of Berkitt
The reasons of development of a lymphoma of Berkitt are definitely not found out. Despite a frequent combination of this pathology to infection with Epstein-Barre's virus, most of researchers consider that Berkitt's lymphoma arises under the influence of several factors among which – the low level of immunity, contact with carcinogens and the ionizing radiation. Experts note correlation between the level of social trouble of the region and quantity of WEB AND POSITIVE lymphoma of Berkitt, pointing to possible communication between adverse living conditions and a role of a virus of Epstein-Barre in development of this disease.
Epstein-Barre's virus concerns to family of gerpesvirus. According to modern representations, along with other viruses entering into this family takes part in emergence of tumor cells. It is widespread, comes to light at a half of children and at 90-95% of adult inhabitants of North America. In most cases it is not shown in any way. Include not only Berkitt's lymphoma, but also other nekhodzhskinsky lymphoma in the list of the diseases associated with this virus, , infectious , herpes, multiple sclerosis, a syndrome of chronic fatigue and some other diseases.
Epstein-Barre's virus contacts certain receptors on a surface of V-lymphocytes, acting as the mutation activator. Besides, this virus changes V-cages, stimulating their continuous proliferation. Scientists assume that such changed cages in the subsequent become a basis for formation of primary center of a lymphoma of Berkitt. It is noted also that the men having this disease often have hereditary anomaly of the H-chromosome that causes the inadequate immune answer at hit in an organism of a virus of Epstein - Barrel.
At an endemic form of a lymphoma of Berkitt the centers most often come to light in a jaw, at sporadic – in a stomach, intestines, a pancreas, kidneys, testicles, ovaries, brain substance, brain covers, other bodies and fabrics. When carrying out a histologic research of a lymphoma of Berkitt unripe lymphoid cages in which kernels fine grains of chromatin are visible are found. Because of a large number of lipids such cages are poorly painted that gives to material a characteristic type of "star sky".
Symptoms of a lymphoma of Berkitt
Emergence of one or several knots in a jaw is characteristic of classical option of a lymphoma of Berkitt. Knots quickly increase in a size, sprout nearby bodies and fabrics, causing visible deformation of the person. Berkitt's lymphoma destroys bones of a facial skeleton, affects a thyroid gland and salivary glands, causes loss of teeth, complicates breath and swallowing. Emergence of knot is followed by a hyperthermia and the phenomena of the general intoxication. In the subsequent Berkitt's lymphoma spreads, affecting bones and the central nervous system, becoming the reason of development of pathological changes, deformations of extremities and neurologic frustration. Paresis, paralyzes and malfunction of pelvic bodies are possible.
At an abdominal form of a lymphoma of Berkitt internals initially are surprised: stomach, pancreas, intestines, kidneys and so forth. This form of a disease differs in the rapid progressing current and presents considerable difficulties in the course of diagnostics. Cages of a lymphoma of Berkitt quickly extend on various bodies, causing belly-aches, dyspepsia, jaundice and fever. At damage of intestines intestinal impassability can develop.
At Berkitt's lymphoma massive bleedings, perforation of hollow bodies, a sdavleniye of mochetochnik with the subsequent dysfunction of kidneys and a sdavleniye of veins with a trombemboliya can be also observed. At formation of large conglomerates in zabryushinny space Berkitt's lymphoma can be complicated by damage of a backbone and spinal cord with development of the lower paraplegia. Over time the tumor spreads in the remote lymph nodes. At the same time lymph nodes of a sredosteniye and pharyngeal ring at both forms of a disease usually remain intact.
In 2% of cases leukemia becomes the first manifestation of a lymphoma of Berkitt. When progressing a disease the leukemic phase arises at every third patient (more often – at new growths of the big sizes). Involvement in process of the central nervous system is considered predictively an adverse sign. It is usually shown in the form of meningealny symptoms. Defeat of brain covers at Berkitt's lymphoma it is quite often combined with violation of work of craniocereberal nerves. Most often suffer a facial and optic nerve. In the absence of treatment Berkitt's lymphoma quickly progresses. The lethal outcome comes within several weeks or months.
Taking into account prevalence of process allocate the following stages of a lymphoma of Berkitt:
- The I stage – is surprised one anatomic area.
- IIA a stage – are surprised two adjacent areas.
- IIB a stage – is surprised more than two areas located on the one hand diaphragms.
- The III stage – defeats come to light on both sides of a diaphragm.
- The IV stage – is involved in process by TsNS.
Diagnostics and treatment of a lymphoma of Berkitt
The diagnosis is exposed on the basis of complaints, the anamnesis, results of external survey and these objective researches. To patients with suspicion on Berkitt's lymphoma carry out a biopsy of a lymph node with the subsequent histologic research. Appoint IFA, RT-PTsR and PTsR. Carry out a X-ray analysis of a skull, KT, MPT, ultrasonography of internals and other researches for assessment of damage of ENT organs, abdominal organs, other bodies and systems. According to indications carry out a sternalny puncture. Differential diagnostics is carried out with other malignant lymphoma.
The main method of treatment of a lymphoma of Berkitt is the poliokhimioterapiya. As this disease children have more often, carry usually out chemotherapy by short courses to reduce risk of emergence of the delayed toxic effects (violations of growth, infertility, development of other malignant new growths). In all cases, except Berkitt I's lymphoma of a stage and a state after radical removal of the tumor located in an abdominal cavity perform prevention of defeats of TsNS with use of a methotrexate, Prednisolonum and a tsitarabin.
The chemotherapy is supplemented with reception of immunomodulators and antiviral means. Appoint high doses of interferon, foskarnt and . The possibility of use of other antiviral medicines for treatment of a lymphoma of Berkitt is in a studying stage so far. In some sources report about use of a combination of chemotherapy and radiation therapy, however, researches show that radiation does not increase efficiency of therapy with use of medicines.
Radical surgical removal of the tumor located in an abdominal cavity or zabryushinny space is considered as the auxiliary method of treatment of a lymphoma of Berkitt which is improving quality of life of patients, but not exerting impacts on the forecast. Operations at tumors of jaws (even followed by the expressed deformation) are not shown because of a plentiful vaskulyarization of a lymphoma of Berkitt and high risk of development of massive bleeding. Sometimes timely bone marrow transplantation gives good effect.
The forecast depends on a stage of a lymphoma of Berkitt. At an early initiation of treatment perhaps absolute recovery. At distribution of process on TsNS, marrow and internals the forecast in most cases adverse. Patients perish from complications, secondary infections and violations of activity of vitals. Berkitt's lymphoma is inclined to a retsidivirovaniye. As a rule, a recurrence develops within a year after the end of treatment. Especially predictively recurrent tumors, resistant to chemotherapy are considered as adverse. Life expectancy of patients in such cases usually does not exceed 8-9 months. Prevention of a lymphoma Berkitta on is developed.