Hodgkin's lymphoma - a type of a tumor, usually affecting the lymphatic system of the body, consisting of connected together in small vessels of the lymph nodes.As a result, a permanent division of the affected lymphocyte cells pass their new somatic organs and lymph nodes, while respecting their proper functioning.
Hodgkin disease was first described in 1832 by Thomas Hodgkin.Until 2001 it was called limfogranulёzom or Hodgkin's disease.This pathological process affects people of all ages.Moreover, statistics show that women suffer from this disease is much less than men.
Hodgkin's lymphoma affects two age categories - people from 14 to 40 years and after 50 years.Although now it is known that people over fifty are not always fall into this reporting.
Hodgkin's lymphoma causes
The cause of the disease, which forms the malignant cells in this disease, until now unknown.
Hodgkin's lymphoma has some features that bring it to inflammation (leukocytosis, undulating feve
But with such a diverse flora found in patients with Hodgkin's disease and absolute inactivity anti-inflammatory therapy can not be entirely attributed to the disease of infectious etiology.But on the basis of epidemiological and histological studies have provided evidence linking Hodgkin's to a viral origin, which indicates an increased risk of developing the disease in people who have had mononucleosis infectious nature.
causative agent of the disease is considered to be virus Eptayna - Barr virus (EBV), which was originally isolated from a cell culture of Burkitt's lymphoma.The fact that these diseases have a certain connection and EBV plays an important role in the causes of Hodgkin's lymphoma, says the similarities cells Berezovsky - Sternberg cells with those that make up the fabric when lifridnaya mononucleosis.
were also found to have high titers of antibody to the antibody humoral virus Eptayna - Barr virus in patients with neoplastic disease.
's disease having mixed cells and lymphoid depletion, which is characterized by a poor prognosis, it has been found high levels of antibodies to the virus.
Thus, at the moment it can be assumed, and viral and infectious nature of occurrence of Hodgkin's lymphoma.Although there is no absolute proof that these agents play a direct role in tumor development.Perhaps the formation of tumor cell lymphoma preceded by a viral infection.
There are certain assumptions that Hodgkin's lymphoma is associated with chronic conflict in the body's immunity.It's all based on the coincidence of the clinical symptoms of Hodgkin's lymphoma and lesions limfogranulёmy morphological manifestations of nature, which are observed in certain immune reactions of the body.
Hodgkin's lymphoma is presented in the form of the autoimmune process that develops as a result of changes blastomatous lymph cells under the influence of prolonged irritation antigens.
When using immunophenotyping and cytogenetic methods of investigation was set clone tumor Hodgkin's lymphoma, which is formed of B cells, and cells from the Berezovsky-Sternberg only 1% of the cell population.Not only are these cells limfogranulёme but mononuclear cells Hodgkin that the intermediate ring is a malignancy.Besides cytogenetic studies show that lymph nodes are Hodgkin's lymphoma cells with an abnormal karyotype and these changes concern the quantity and structure of the chromosomes.
Hodgkin's lymphoma symptoms
Pathological tumor disease is characterized by the diversity of the clinical picture, which is caused by lesions of various groups of lymph nodes and organs.Symptomatology Hodgkin consists of two groups.These are common symptoms of the disease and the local manifestations of the pathological process.
Almost every patient with Hodgkin's lymphoma, these common symptoms are observed as the temperature climbs body, fatigue and weakness throughout the body, night sweats, itching, joint and headaches, and pain in the bones.The rise in body temperature refers to the first symptoms of Hodgkin's lymphoma.At the beginning of the pathological process, the temperature is in the subfebrile numbers, but increased it in the evening.
As the disease begins to progress, then the temperature reaches high levels - 39-40 degrees with night chills the body and profuse sweating.Also, patients constantly complain of general weakness, which leads to a decrease in efficiency.
Itching is not always present in Hodgkin's lymphoma, but sometimes occurs long before the changes in the organs and lymph nodes, and a very long time is the only symptoms of the disease.This itch can spread both across the body surface, and localized on the anterior surface of the chest, upper or lower limbs, scalp, palms of the hands and feet.
for Hodgkin lymphoma is characterized by the local manifestations of the location and size of the changed nodes and pathological lesions in different tissues and organs.Basically more of affected lymph nodes above the diaphragm (90%), as observed in other podtsiafragmalnyh collectors (10%).
most cases of Hodgkin lymphoma begins with enlarged lymph nodes in the neck (50-75%), which are painless palpation, have nothing in common with the other tissues are not connected with each other and are provided as elastic consistency.The skin over the tumor has no infiltration, no redness and changes.
In 25% of affected supraclavicular lymph nodes are localized in the inner corner of the supraclavicular fossa and do not have large forms.
In 13% of pathological change are subject to the lymph nodes in the axillary regions.There is localized plexus of lymphatic vessels that connects them to other bodies, contributing to the rapid penetration of tumor in lymph nodes and mediastinal lymph nodes, which are located under the chest muscle on the breast.
15% affected mediastinal lymph nodes, which are subjected to strong pressure and germinate in the next underlying tissues and organs.After that, patients have early clinical manifestations of the disease in the form of a slight dry cough and chest pain.They can be of varying intensity, from dull and constant pain of growing up with a deep breath, and coughing.
Sometimes patients with Hodgkin's lymphoma experience a distension in the chest and tingling in the heart.This is a result of pressure enlarged lymph nodes in the nerves, heart, or with invasion of tumor formation in the bronchi, lungs and pericardium.At the same time in the pleural cavity and pericardial effusion appears non-specific nature, which causes patients initially slight shortness of breath when walking or exercising.And in the future, with the progression of the process, shortness of breath becomes much stronger, and mediastinal lymph nodes acquire a considerable size and this causes the syndrome of the upper genital vein.But sometimes such a defeat mediastinal lymph node runs completely asymptomatic and discovered accidentally when rengenologicheskom of the chest.
rare clinical manifestation of Hodgkin's lymphoma is a retroperitoneal lymph node lesion (1.7%).Initially, these changes occur without symptoms, and then when the lymph nodes increased, the lumbar zone appears numbness, pain, bloating and constipation.And even worse pain after taking small doses of alcohol (30-40 g).This symptom in medicine is called "alcoholic pain."
in rare lesions and iliac lymph nodes pahovobedrennyh (3-5%), Hodgkin lymphoma is characterized by malignant course and poor prognosis.The clinical picture is marked permanent or cramping pain in the abdomen, a violation of lymphatic drainage, weight in the lower extremities, puffiness and pasty stop, decrease the sensitivity of the front and inside of the thighs.
In the primary lesion of the spleen with Hodgkin's lymphoma, the disease is benign, and the life of patients with this form for a long time prolonged.In the pathological process involved the spleen from 65 to 86%.This body is the only symptom that signals the defeat of this increase in size, which is determined by Ultrasonic or radionuclide study.This symptom is considered very important to diagnose the disease and has a certain relevance.
primary form of Hodgkin's lungs can be seen rarely, but, nevertheless, always light attached to the pathological process (20-40%).The affected cells are entered in lung tissue by hematogenous.And clinical symptoms of pulmonary Hodgkin's lymphoma is characterized by diversity.This form of the disease manifests cough, chest pain, severe shortness of breath and sometimes hemoptysis.If the lung tissue damage affects a limited, the cough is negligible or absent.The specific nature of the changes in the lung combined with the affected pleura, which could result in fluid in the pleural cavity.
Patients with bone disease Hodgkin's lymphoma occur in 15-30% of cases.Existing primary and secondary changes as a result of tumor metastasis lesions or other structures it spread through the blood to distant organs.In this case, very often exposed to the pathological process of the spine, then the ribs, sternum and pelvis.
rare phenomenon is the localization of Hodgkin's lymphoma in the long bones and the skull.But with the defeat of the bones appear to severe pain radiates character increases during pressure on the spinal processes.Numbness in the feet, twitching and weakness occurs after changes verhnepoyasnichnyh and lower thoracic vertebrae.The progression of the spinal cord causing paralysis and paresis of the lower limbs and dysfunction of organs in the pelvis.
When the liver is affected by Hodgkin's lymphoma is 5-10% after the initial diagnosis, and pathologic data of - to 30-77%.The liver may be changes in the form of multiple and single foci.Besides, they can be smaller and larger sizes.Patients with liver of affected mainly complain of nausea, heartburn, specific breath and heaviness in the right upper quadrant.Patients with Hodgkin's lymphoma are all manifestations of parenchymal, mechanical or hemolytic jaundice, which only worsens the prognosis of the disease.
Bone marrow is affected Hodgkin lymphoma without specific symptoms and diagnosed to 4%, and at autopsy - 30%.
Hodgkin's lymphoma are sometimes affects the skin, nervous system, thyroid, oral cavity, heart muscle, genitourinary tract and mammary glands.
Hodgkin's lymphoma stage
clinical staging of the disease undertaken more in 1902, but only in 1965 at an international conference in New York, was adopted and set the classification of the clinical picture, which became the basis of modern stages of lymphoma.She shared the stage Hodgkin's lymphoma, gave the designation of pathological and clinical stage and extranodal dissemination (E).
According to current clinical classification of tumor diseases, which takes into account the anatomical distribution of the pathological process and all the symptoms of intoxication, adopted in 1971, the disease has four stages: I, II, III, IV.
At first (I) stage Hodgkin's lymphoma is one amazed limfozona or limfostruktura (I) or individually affected in a single amount ekstralimfatichesky organ or tissue (I E).
In the second (II) stage Hodgkin's lymphoma is affected two lymph region on one side of the diaphragm (II), or locally affected unit ekstralimfaticheskaya tissue or organ and regional lymph nodes, affecting or not the other lymph nodes on the same side of the diaphragm (NE).
When the third (III) stage Hodgkin's lymphoma affects the lymph nodes on both sides of the diaphragm (III), which are combined with the localized change in one ekstralimfaticheskoy tissue or organ (III), and perhaps with changed spleen (III S), and sometimes thatand the other (E and E + S).
At the fourth stage (IV) stage Hodgkin's lymphoma disseminated affects one or more ekstralimfoorganov, changing or not components of the lymphatic system, or isolation affected ekstralimfatichesky body changing neregionarnyh lymph nodes.
Ekstralimfaticheskim spread of the disease (E) is considered a pathological process in which involves one lobe or the root of the lung at the same time amazed lymph nodes on one side, or one-sided effusion of the pleura with joining or not a light in the process of destruction, but with the changes here inhilar lymph nodes.
For extranodal diffuse spread of the disease (IV) include the destruction of the bone marrow and liver.
In addition, all the stages of Hodgkin lymphoma have or do not have common symptoms.And denote their absence (A) and presence (B).To the common symptoms may include: weight loss, unexplained nature, within six months by 10%;for no apparent reason ups, up to 38 degrees, the body temperature for 3 days and profuse night sweats.
in 1989 were adopted in Kostvolde complement notation at stages of the affected regions, with (II 2, III 3, etc.).The third stage can share at the III 1, that affects the spleen, lymph nodes and the celiac nodes gate spleen.For 2 III characteristic lesions inguinal-femoral, iliac, para-aortic and mesenteric lymph nodes.
With a huge lymph node (X) reaches a pathological focus in diameter and more than ten centimeters.
Hodgkin's lymphoma treatment
cure this disease is the greatest achievement of XX-th century.With modern treatment programs Hodgkin complete remission is obtained in 70-90% of patients with primary forms of the disease, and 60% are patients who have had a twenty-year recurrence-free survival after first use of treatment programs.
Among the main treatments for Hodgkin's lymphoma emit radiation and drug therapy, as well as their combined method.In order to conduct radiotherapy on the affected lymph nodes of tumor dose is used in an amount of forty-five or forty-Gy and prevention - 35 Gy.For example, the Russian, in some cases using widefield method of exposure.
Currently, patients with Hodgkin's lymphoma treatment methods are used, taking into account the risk factors and stages of the disease.By
significant risk factors include the affected lymph region in an amount of three or more;if indicated in step B SOE - 30 mm / h and 50 mm / h in step A;when ekstranodulyarnom defeat;massive changes in the mediastinum;when mediastinal-thoracic index;In the presence of the huge spleen lesions with diffuse infiltration of the body;available at the lymph nodes in diameter and 5 cm or more.
However, radiotherapy Hodgkin's lymphoma are not satisfied with the results, so doctors use chemotherapy.Today monochemotherapy appointed rarely, and then only with the aim of palliative treatment of frail elderly patients or the substitution of one tissue to another bone marrow after administration of multiple-dose chemotherapy treatment.When monochemotherapy appointed any anticancer drug: Natulan - 100 mg daily;Chlorambucil - 10 mg every day for five days, rates of up to 450-500 mg;Vinblastine 6 mg / kg 1 time a week.
Basically, the treatment of Hodgkin lymphoma begins with the appointment of chemotherapy, a total dose of 36 Gy to the center for the first stage A patients with no risk factors and prevalence of lymphoid histological variant.