Currently, among adults and children gaining weight group such malignancies as lymphoma.This category is characterized by lesions of the lymphoid tissue and the appearance of neoplastic cells.It includes two types of tumors:
- limfagranulematoz (Hodgkin's lymphoma) - survival of about 80% of patients with non-Hodgkin's lymphoma
- - survival of 20-25%
These two diseases are very different in matters of the causes and course of the disease, and most importantly, patient survival.
General concepts about the lymphatic system
The lymphatic system is an integral part of the cardiovascular system consists of lymph vessels and lymph nodes.Lymph node contains specific lymphoid tissue through which the lymph and its decontamination occurs.
It performs several vital functions:
- cleanses the body of toxins and alien bacteria
- involved in metabolism (especially in fat metabolism)
- saturates the blood immune cells (lymphocytes)
Lymphogranulomatosis or Hodgkin's disease -neoplastic process is developing in the lymphoid tissue and is characterized by the emergence of new Berezovsky-Sternberg cells.This pathology is known since 1832, when an English doctor Thomas Hodgkin described seven cases of Hodgkin's disease.The disease affects mainly young people aged 20-25 years.pattern was found between the advent of Hodgkin's disease and carriage of Epstein-Barr virus.There are 4 histomorphological variant of the disease:
- lymphohistiocytic - 5%
- Nodular sclerosis - 30-45%
- Mixed-cell - 35-50%
- lymphoid depletion - 10%
clinical picture of Hodgkin's disease depends on the location of the primarysource tumor.Often the disease begins with hypertrophy of the cervical lymph nodes, supraclavicular, axillary and inguinal areas that blend with time in large groups and grow into the underlying tissues.Then the superior vena cava syndrome develop, pleurisy and pericarditis, respiratory failure, enlarged liver and spleen, loss of body weight up to cachexia.
main method of diagnosis of the disease is a needle biopsy of the affected lymph node.In preparation revealed Hodgkin's cells, multinucleated cells Berezovsky-Reed-Sternberg, lymphocytes, neutrophils, eosinophils, and others.
Treatment of Hodgkin's disease is a combined and includes radiation and chemotherapy.The prognosis for patients is beneficial, as Hodgkin's disease responds well to treatment.The five-year survival rate of patients after application of combined therapy is 87-90% (more details in the article about the symptoms and treatment of limfagranulematoza).
Malignant lymphoma - lymphosarcoma
This is a large group of lymphoproliferative disorders (30 diseases), affecting lymphoid germ of blood, characterized by different histological characteristics of blast cells, the symptoms of disease and prognosis of survival.
morbidity statistics At present, the incidence of this disease is about 45% higher than the occurrence of Hodgkin's disease.Very often there are lymphosarcoma in the US, Canada and Europe.Rarely, the disease occurs in Asia (Japan, Singapore, India).In Russia, the incidence lymphosarcomas is about 3%, there is a significant trend NHL growth in recent years.They occur at any age, men in 2 times more often, it is very rare in children up to 3 years, mostly diagnosed in older people.
lymphoma Why are there still remains a mystery to doctors and scientists.There is a particular trait found in all patients at the same time.But we know the following:
- Epstein-Barr virus causes Burkitt's lymphoma in HIV-infected patients and in patients receiving therapy immunnosupressivnuyu.Many
- NHL patients with genetic defects were found in chromosome 8.U.S.
- most patients was isolated human T-cell lymphotropic virus type 1 (HTLV-1), which is transmitted through a proven human biological fluids (semen, blood, saliva, milk).
Environmental factors also play a big role in causing the disease.There is a high risk of morbidity:
- prolonged contact with pesticides, herbicides
- when taking certain medications
- when exposed to radiation, ionizing radiation (X-rays, ultraviolet irradiation).
Division NHL REAL (The Revised European-American Classification of Lymphoid Neoplasms) is complex and is based on the molecular structure and biological characteristics of the tumors.
Types of Lymphoma:
|B-cell lymphoma||T-cell lymphoma|
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In malignant lymphosarcoma fall into three categories:
- low-grade - a small malignancy have plasmacytoma Valdenstrёma and follicular mixed cell NHL
- intermediate level observed during the development of large-follicular and diffuse large cell lymphoma.
- high degree - the most dangerous with a high degree of malignancy are considered Burkitt's lymphoma and NHL immunnoblastnaya.
clinical picture NHL
symptoms of lymphoma are quite diverse, each species has its own peculiarities.A common feature for all the NHL is a primary lesion of the lymph nodes (nodal location) or liver and spleen (extranodal).In the pathological process involved the lymph nodes of the neck, armpit, mediastinal, retroperitoneal and peritoneal (ie on the side of the neck, the armpits, the folds in the elbow, under the collarbone, in the groin).
At the beginning of the disease lymph nodes increase in size, become dense and painless, and then merge into larger groups (conglomerates) may ulcerate and form fistulous passages.Symptoms of lymphoma due to hypertrophy of nodes:
- cough, inspiratory dyspnea
- sensation of a lump in the throat, swallowing disorder
- stagnation of blood in the upper half of the body due to compression of the veins (cyanosis and puffy face)
- limb edema
eventually lymphoblastic cells spread throughout the body (of disseminated), affecting the bones, liver, spleen, nervous system, mammary glands, testicles, and so on.By ekstranodulyarnym (ie not related to lymph nodes) Symptoms include:
- appetite Deterioration, weight loss (for 6 months more than 10%), malaise, fever, increased night sweats, poor sleep, apathy, irritability
- Increase and consolidation breast symptom umbilikatsii (retraction of the nipple)
- appearance on the skin of dense, painful nodules, prone to mergers
- meningism (defeat blast meningeal cells), characterized by headaches, repeated vomiting, not bringingease, positive meningeal signs
- If it affects the bone marrow -. anemia, susceptibility to difficult flowing infections, bleeding tendency, frequent nosebleeds, prolonged heavy menstruation, bruises, etc.
- defeat of the musculoskeletal system is manifested by severe pain in the spine, bonespelvis, muscle atrophy, pain in the joints and bones, even at rest
- Enlarged lymph nodes are not painful on palpation, the skin is normal color, limfauzly welded together
- If there is a lesion of the tonsils - difficulty in swallowing, changes tone of voice, the tonsils are increased insizes
- We would like to see the symptoms of Burkitt's lymphoma, the most common tumors in the group.Burkitt lymphoma primarily affects the gastrointestinal tract, causing hypertrophy of lymph nodes.The tumor is accompanied by severe abdominal pain, bloating, constipation, intestinal obstruction.Perhaps the development of bowel perforation and peritonitis.
- If the tumor process involves the lymph nodes of the chest cavity, the respiratory tract, thymus - difficult swallowing, there is a dry hacking cough, tightness of the chest cavity, progressive dyspnea
- If it affects the central nervous system - convulsions, vomiting, severe headaches, paralysiscranial nerves
Lymphoma in children has its own characteristics.The most common tumor in the process involves the bone marrow blast cells inhibit the sheets of blood, causing anemia, increased bleeding, frequent inflammatory diseases.
The main disease diagnosis methods include:
- needle biopsy of a lymph node cytology punctate, which usually identifies the immature form of T- and B-lymphocytes.It is for the degree of maturity of tumor cells is determined by a malignancy NHL.Depending on the internal organ may conduct a biopsy of the liver, bone marrow, lumbar puncture.
- X-rays (CT scans, an overview scan), magnetic resonance imaging, ultrasound duplex scanning of the affected organs.
- clinical tests (complete blood count, urinalysis, biochemical analysis, coagulation).
- Laparoscopy and laparotomy trial to study ascitic exudate.
- immunological blood test to detect antibodies to the Epstein-Barr virus-specific markers of tumor (BCL-2, CD 19, 20, 22, HLA / DR).
The choice of tactics of treatment of NHL depends on the extent of tumor and morphological types of cells.In any event, treatment should be complex and objective.
- Surgical treatment - when single tumors of internal organs.It manufactures a complete excision of the affected tissue and nearby lymph node groups.When expressed splenizme (i.e. excess spleen when it destroys not only tumor cells, but normal red blood cells) are removed sezenku.
- Radiation therapy - used in combination with chemotherapy is an effective method at all stages of treatment, it can be used in the pre- and postoperative period.
- Chemotherapy - involves the use of cytotoxic drugs (cyclophosphamide, fluorouracil), monoclonal antibodies (rituximab), corticosteroids (methylprednisolone, budesonide).When lymphoma is often used monochemotherapy.
- transplantation (transplantation) of the bone marrow - the recovery of blood cell composition, reduces the symptoms of the disease.
main criterion for remission is the disappearance of lymphoma in punctate blasts.In recent decades, it is widely implemented this non-invasive method like PCR, revealing the body's lymphoid modified DNA clone.
prognosis of lymphomas
5-year survival in NHL varies depending on the morphological variants:
- good forecast of more than 70% - with follicular lymphoma, MALT- lymphomas, B-cell lymphoma, marginal zone
- At T-lymphoblastic, peripheral T-cell NHL - less than 30% survival rate.
- If the primary lymphoma of the orbit, lymphomas, lung, stomach, salivary glands, rings Pirogov-Waldeyer - more favorable forecasts - 5-year survival rate of over 60%
- Lymphoma mammary glands, bones, central nervous system lymphoma, ovarian, and testicular are highly malignantand a low survival rate.
also in lymphomas of high grade life expectancy of patients is directly dependent on the timely initiation of treatment and its results:
- If achieved complete remission, then 5 years-survival rate of about 50%
- In partial remission - 15%, therefore,it is important to conduct an active intensive care immediately after the diagnosis of lymphoma vysokozlokachestvennoy to achieve complete remission
- Five-year survival in low-grade lymphoma is 80% regardless of the outcome of treatment - complete or partial remission.