Lymphogranulomatosis - a pathological-granulomatous lesions of the lymphatic system, which is also called Hodgkin's lymphoma.Hodgkin's disease was first described in 1832 by the physician Thomas Hodgkin, explain a few moments of the disease.Initially, usually increase the lymph nodes and spleen , then a fever and cachexia, subsequently leading to the death of patients.And already in 1875 Coutard spent the first histological study of lymph node that was removed during the life of the patient.
In 1890, Russian scientist Boris Berezovsky has been described histology of Hodgkin's disease.They were revealed pathognomonic giant cells characteristic of the disease.A Viennese pathologist has in the years 1897-1898 was described by polymorphonuclear cell granuloma, which has giant multinucleated cells, which later became known as Berezovsky-cell Reed-Sternberg.
need to examine the lesion is due to Hodgkin's disease patients, starting from a young age (mostly - from 15 to 40 years
Lymphogranulomatosis ranks tenth among oncological pathologies, in which the largest percentage of injuries falls on men than on women.
Until recently lymphoma was seen as a disease of infectious origin.It was believed that it could be the causative agent of tuberculosis bacillus.Less often, this role was prescribed streptococcus, E. coli, Treponema pallidum, and diphtheria bacillus.It is also speculated that the virus etiology of Hodgkin's disease, but this is not confirmed.
Currently established that a tumor (gematosarkomy and leukemias) is considered certain disorders of the hematopoietic system and malignant cells Berezovsky - Sternberg cause of Hodgkin's disease.
also not fully clarified certain factors of life that may contribute to the disease.These include lifestyle, bad habits, diet and occupational hazards.Some studies provide data about the possible risk of occurrence of Hodgkin's disease in persons who had been ill with infectious mononucleosis or skin diseases, working on sewing or woodworking industry, in agriculture, as well as among chemists and doctors.
Cases of Hodgkin's disease among several members of one family, or in a team.This makes the assumption of the existence of the role slabovirulentnoy infections of viral etiology and genetic predisposition of the body, however, the final proof of this yet.Thus, specific and exact cause of Hodgkin's disease is not found.
clinical picture of Hodgkin's disease is characterized by two types: natural and chronic.
In the natural course of Hodgkin's disease is an alternation process of exacerbation and remission, but sometimes the disease becomes rapid, acute onset of a high rise in temperature, heavy sweating and spread of tumors in various organs.Without care, these patients may die within a few weeks of the onset of a pathological process or in the coming months.
But basically 90%, chlamydia is a chronic, with periodic exacerbations, resulting in the pathological process of adopting new lymph nodes, tissues and organs.
In the course of Hodgkin's disease adversely affected by some factors such as solar radiation, pregnancy and physiotherapy.
for clinical symptoms of the disease are characterized by local and general symptoms.While diagnosing the pathology of great importance may have common symptoms.Among them are various groups of nodes increase and the appearance of pathological lesions in tissues and internal organs.Primarily affects the peripheral lymph nodes, which are characterized by an increase in size without reason, or it may coincide with colds.These lymph nodes palpation are unequal in round or oval shape with elastic consistency.As a rule, they are not connected with each other and with adjacent tissues, and are movable painless.The skin has no changes and it is possible to freely assemble in the fold, fistulas do not appear.
In 80% of Hodgkin's disease affects the lymph nodes located in the neck.Its circumference is increased, the contours become uneven and sometimes visually noticeable protrusions lymph nodes if they are affected in the plural number.At the time of visits to the doctor almost 25% of patients at the same time with palpable cervical lymph nodes and supraclavicular significantly increased.
Very rarely diagnosed with primary Hodgkin axillary and inguinal lymph nodes.More often, there is a simultaneous loss of the cervical lymph nodes with or mediastinal lymph nodes.Sometimes the disease can begin with an inflammation of the occipital, submandibular and parotid glands.
About 20% of patients with Hodgkin's disease affects the lymph nodes in the mediastinum.Diagnosing this period of the disease is possible with random X-ray examination of the lungs.But the clinical picture for a long period of no symptoms, but when the lymph nodes are increased by large size, the patients complain of shortness of breath, wheezing, chest pain and cough.
When percussion is impossible to determine the small size of the lymph nodes.But increased conglomerates dull percussion sound, breathing and weakened in both large and small parts of the chest.In the later stages of the disease, after compression of the thoracic duct lymph nodes or accession infection of the second plan, pleura.Sometimes the patient has mediastinal compression syndrome of lung atelectasis or character.
In rare cases, can be found retroperitoneal lymph node failure or iliac in the primary form of Hodgkin's disease.Such damage occurs without any apparent clinical symptoms.Only when multiple lesions appear constant pain or recurrent nature in the lumbar region, abdomen, lower limbs;bloating and upset the chair.
With a significant increase in the lymph nodes in the inguinal region is a violation of lymphatic drainage of the limbs, which is the result of swelling of the distal part of the foot and lower leg.
Lymphogranulomatosis strikes and internal organs.Second place after the defeat of the lymph nodes of the spleen takes.However, the clinical symptoms it does not manifest itself.On palpation of the spleen is almost normal and does not reach large sizes, so it is difficult to probe and manages to do so only when there are multiple and major organ damage.
much rarer Hodgkin other bodies with the primary manifestations of the disease.And basically this pathological process is combined with lymph node and manifests as pain and symptoms associated with dysfunction of these organs.
With the defeat of Hodgkin's disease of bone, patients complain of pain of varying intensity and swelling of the affected by the outbreak.
Sometimes the disease spreads and skin.In this case, there are small rounded infiltrates and ulcers, acquiring a dark red color, or change in the form of rashes, hives, eczema associated with immunocompromised patients.
Almost 50% of patients had symptoms of intoxication in which the body temperature rises, there is a night sweats, itchy skin, and there is a dramatic reduction in weight.Many patients complain about the state of general weakness, aches in the joints, muscles and bones, as well as headaches.
There are three main clinical symptoms, which worsen the prognosis of Hodgkin's disease.Firstly, it is weight loss, without specific reasons for six months up to ten percent.Secondly, it is copious night sweats.And, thirdly, unexplained rise in body temperature above 39˚S for three days.
manifestation of generalized pruritus also refers to the symptoms of intoxication.
Lymphogranulomatosis affects the bone marrow with no specific features and is diagnosed in 5%, and at autopsy in 30% of cases.
Lymphogranulomatosis usually affects the lymph nodes, much less a pathological process extends to the spleen, liver, lungs and other organs.Therefore, symptoms of Hodgkin's disease is classified as lymph and ekstralimfaticheskie.
When "lymph" affects the lymph nodes, spleen, thymus, pharyngeal lymphoid ring, appendix and Peyer's patches.All other lesions are ekstralimfaticheskim manifestations of the disease.
Today, the most widely used classification of Hodgkin's disease, which was proposed in 1966 by Lucas.It distinguishes four histological type of Hodgkin's disease: lymphohistiocytic, nodular-sclerosing, mixed cellular and reticular.
believed that developing lymphoma unitsentricheski.The first one is subjected to lesion site of lymphoid tissue in a lymph node, and then the cancer cells spread through the lymphatic system to the new homes, and there are striking new lymph nodes.Therefore, it is this different from Hodgkin's disease and other pathological entities, as may affect the lymphoid tissue in the remote areas of the main focus of malignancy.At the time, it is difficult to develop in the division at the stage of Hodgkin's disease, which was finally adopted by the international classification in 1971.It considers four stages of the disease.
At the first stage of Hodgkin's disease, or local area affected one lymph node or one ekstralimfaticheskaya tissue, and can be, and the organ.
In the second stage of Hodgkin's disease or regional affects two areas of lymph nodes, and sometimes more localized on one side of the diaphragm.But it may be involved in the pathological process of different zones of the lymph nodes at the same time one defeats ekstralimfaticheskoy tissue, organ, located on the same side of the diaphragm.
When the third or generalized stage Hodgkin's disease affected areas of lymph nodes in the singular or plural form on both sides of the diaphragm.This process may include stunned by the spleen, one ekstralimfaticheskaya tissue or organ.
At the fourth stage of Hodgkin's disease, or disseminated diffusely affected ekstralimfaticheskih one or more tissues or organs with the simultaneous defeat or without lymph node.
In addition, when available to the patients symptoms of intoxication, the index added to the stage - "B" if no - "A".It is also known that the prognosis of Hodgkin's disease worsening of symptoms upon accession to the stages of the pathological process.
this malignant pathology is common among children in the ratio of 1: 100,000.This is much lower than among adults.Hodgkin's disease also affects children up to the first year of life.The main peak of the disease accounts for pre-school age.In addition to ten years of this age group among boys in the incidence prevails over girls.And from 15-16 years the relationship between the sexes is aligned.
Children malignant lymphoma is characterized by changes in the lymphoid tissue and spread to the lymph nodes limfogranulёm and somatic organs.Usually, chlamydia goes from one of the affected area to another.
Children generally diagnosed cervical lymphadenopathy, which is characterized by a variety of inflammatory processes in the nasal and oropharynx.As a rule, these changes align defeat verhnesheynyh and submandibular lymph nodes.And now with the defeat of supraclavicular and low-neck there is a suspicion about Hodgkin's disease.Sometimes (in 20% of cases) in the pathological process involved axillary lymph nodes, and less than 5% - inguinal.
If a large number of affected lymph nodes in the mediastinum, the affected tissue and lung and pleura and pericardium, and chest with possible development of a compression syndrome.At this time, the child's face becomes puffy with signs of cyanosis, develops a cough and shortness of breath tormenting character with little physical exertion and tachycardia.
in rare defeat subdiaphragmatic lymph nodes children do not impose specific complaints.Then, in the pathological process joins the spleen, and in rare cases - the liver.The child appears high fever, fatigue, night sweats, appetite decreases, it becomes persistent drowsiness and apathetic.In the analysis of the blood may increase the ESR.At the fourth stage of Hodgkin disease (disseminated) is determined by the reduction of red blood cells and platelets in the blood.Therefore, when these manifestations mandatory appointed by biopsy.More
note certain signs of activity of the pathological process.These include an increased level of haptoglobin, the presence of an increased amount of ceruloplasmin, fibrinogen and lactate dehydrogenase.
When Hodgkin lesions of intrathoracic lymph nodes in the child a cough, cough and starting rolling in the attacks.The child constantly complains of pain in the chest or the heart, worse during the next fit of coughing.
in children with Hodgkin para-aortic lymph nodes marked the defeat, which are located next to the vessels in the area of the gate of the liver, kidneys and spleen.When retroperitoneal lymph node lesions in combination with spleen children complain of recurrent abdominal pain of varying intensity.
Lymphogranulomatosis little changes all tissues and organs in the body of the sick children.For extranodal lesions include pulmonary tissue, bone, pleura and liver.Much less Hodgkin alters the bone marrow.
to diagnose in children given malignant lesion in the United States in 1971, were adopted by certain types of surveys that are necessary for the proper staging of Hodgkin's disease and its treatment of choice.First of all, when the child's medical history is collected, concentrated attention on all symptoms.Then, if necessary, and biopsy is carried out inspection of the affected areas.Then prescribe laboratory tests to determine the biochemical parameters.Mandatory study is a chest X-ray in different projections both forward and side, as well as computed tomography.If those cases where there is suspicion of pathological changes of bone marrow biopsy is appointed, and bone disease, kidney and liver - scanning.
Among the main methods of treatment of the pathological process in the lymphatic system include medicinal method, radiation therapy, and their combination.
However, in the past few decades one of the treatments for Hodgkin's disease had surgical excision of the affected lymph nodes.But in the nineteenth century it was found that surgery leads to the spread of a pathological process.Therefore, treatment of Hodgkin's disease have limited the fortifying agent.
first irradiation of the lymph nodes were made in 1901 in two patients with Hodgkin's disease.And in 1906 this treatment appeared in Russia.But only with the 1940 to the radiation therapy was added to chemotherapy.The first chemotherapy drug became Mustargen belonging to group hloretilaminoz.Since 1947, Larionov uses an analogue - embihin.However, until the sixties of the last century chemotherapy to treat Hodgkin's disease were used irregularly.