Lymphoma: Better know these cancers of the lymphatic system
Introduction
Hodgkin's disease or Hodgkin's lymphoma: symptoms, treatment, prognosis
Non-Hodgkin's lymphoma: symptoms, treatment, prognosis
Follicular lymphoma, a form of non-Hodgkin's lymphoma
Mantle lymphoma, a form of non-Hodgkin's lymphoma
Burkitt's lymphoma
The lymphoma of MALT
Lymphoma: symptoms, screening and diagnosis
Introduction
Lymphoma is a cancer of the lymphatic system that makes more than 10,000 new cases each year in France. The disease is better known and better treated today, but its mortality has declined in recent years.
In 2011, lymphomas ranked 5th among the most common cancers. With more than 10,000 new cases each year in France, these diseases have shown an increasing incidence in recent years in both men and women. If these types of cancer remain unknown to the public, scientists have multiplied the research to better know them and to determine potential risk factors.
This has allowed better screening and more appropriate management of lymphomas, lowering the mortality rate. In 2011, these cancers ranked 8th among the deadliest cancers with about 4,000 deaths. As the name implies, lymphomas attack the lymphatic system, which includes bone marrow, spleen, thymus, lymph nodes and lymph vessels. However, not all lymphomas have the same characteristics since there are two main types: Hodgkin lymphomas and non-Hodgkin lymphomas, which are themselves divided into different subtypes.
To shed light on these diseases, Gentside discoveries decided to dedicate a dossier in order to explain in particular the differences between the lymphomas. The SUMMARY:
-Hodgkin's disease or Hodgkin's lymphoma
-Non-Hodgkin's lymphoma
-Follicular lymphoma, a form of non-Hodgkin's lymphoma
-Mantle lymphoma, a form of non-Hodgkin's lymphoma
-Burkitt's lymphoma
-Malt lymphoma
-Lymphoma: symptom, screening and diagnosis
Hodgkin's disease or Hodgkin's lymphoma: symptoms, treatment, prognosis
Hodgkin's disease or Hodgkin's lymphoma: symptoms, treatment, prognosis
Hodgkin's disease is the first lymphoma to have been well characterized. It is manifested by an abnormal development of certain immune cells, lymphocytes, most often in the lymph nodes of the neck or armpits.
Described for the first time in 1830, Hodgkin's disease is now rarer than other types of lymphoma, with just over 1,800 new cases per year. It reaches mainly the young adult, between 20 and 40 years old, and the subject aged over 60 years. On the other hand, it is more rare in children under the age of 10.
This cancer develops from the lymphatic system, most often in the lymph nodes of the neck and armpits, a little more rarely, at the level of the groin glands. From there it is characterized by an abnormal development of certain cells of the immune system, lymphocytes type B.
These cells have become malignant and have a large size and a larger nucleus with several lobes: this is called Reed-Sternberg cells which are characteristic of Hodgkin's disease. But there is also another variant called Hodgkin's cell.
A cancer that spreads in the lymphatic system
The presence, number and characteristics of these cells will determine the lymphoma subtype. Without treatment, the disease will evolve progressively in the lymph system reaching a ganglion (stage I) then two then several connected via the lymph vessels. The cancer can then also spread to the spleen, the thymus and the bone marrow or even other organs. This is the last stage of the disease, stage IV.
Today, the causes of Hodgkin's lymphoma remain unknown. But some risk factors have been identified: this is the case of Eipstein-Barr virus, which is responsible for infectious mononucleosis and which seems to play a role in the onset of certain forms of Hodgkin's disease. On the other hand, a deficiency or an immune deficiency could promote the occurrence of this cancer, perhaps again through viral factors.
Another hypothesis also evokes the possible existence of predispositions related to sex or certain hormones. But it has not been confirmed.
A long-term survival rate between 70 and 95%
Hodgkin's lymphoma has benefited from numerous scientific breakthroughs in recent years that have allowed for better diagnosis and better treatment. While it was very often fatal before, the long-term survival rate now reaches between 70 and 95% depending on the extent of the disease's spread, according to the League Against cancer figures.
The classical treatment combines radiotherapy and chemotherapy, the intensity of which will vary depending on the stage of the cancer. Given the likelihood of healing, doctors generally opt for the least aggressive treatments possible. In some cases, however, autografting of cells taken and re-implanted in the bone marrow may be necessary after treatment.
Non-Hodgkin's lymphoma: symptoms, treatment, prognosis
Non-Hodgkin's lymphoma: symptoms, treatment, prognosis
Like Hodgkin's disease, non-Hodgkin lymphomas are cancers of the lymphatic system that develop from lymphocytes. But they have different characteristics.
With more than 10,000 new cases per year, non-Hodgkin lymphomas represent the most common cancers of the lymphatic system. They are characterized by an abnormal and anarchic development of cells of the immune system, lymphocytes. But they do not present the same characteristics as Hodgkin's disease, hence their name.
Unlike the latter, non-Hodgkin (NHL) lymphomas can develop in the lymph nodes as in other organs of the system and can reach several types of lymphocytes (B or T). There are some thirty forms and subforms that will differ in particular in the degree of malignancy. This is why we are talking about the NHL indolent (low malignancy) and aggressive NHL (high malignancy).
B or T lymphomas with different evolution
The former grow slower and have fewer symptoms while the latter grow faster. On a smaller scale, these lymphomas differ according to the appearance of tumor cells and their mode of evolution. In many cases, these are B lymphocytes that are affected. But depending on the lymphoma, these can evolve into large cells as in small cells.
The stages of evolution are the same as Hodgkin's disease. We're talking about stage I when a single ganglionic area is reached, stage II when the cancer extends to at least two areas on one side of the diaphragm, stage III when the areas are on both sides and finally stage IV when there is diffuse impairment of one or Several viscera.
A survival rate of 70 to over 90%
The incidence of different lymphomas varies depending on the age of the patients. Among the most common, however, are large B-cell diffuse lymphoma, lymphoblastic lymphoma and Burkitt lymphoma. Being better known today, these different cancers show a much better survival rate than a few decades ago. Depending on the shape and stage of the disease, it varies from 70 to over 90%.
As well as the characteristics, diagnosis and treatment of the disease will differ from one form of lymphoma to another. But chemotherapy and radiation therapy remain the two main treatments.
Follicular lymphoma, a form of non-Hodgkin's lymphoma
Follicular lymphoma, a form of non-Hodgkin's lymphoma
Follicular lymphoma is one of the most frequent non-Hodgkin lymphomas. of low malignancy, it usually occurs in adults and very rarely in children.
Follicular lymphoma, classified as indolent lymphoma, is characterized by an abnormal proliferation of B lymphocytes. It usually manifests itself at the level of the lymph nodes but can reach the bone marrow, spleen and peripheral blood.
In half of the cases, it can cause a splenomegaly, i.e. an increase in the volume of the spleen caused by the multiplication of tumor cells showing a variable size. Today, this lymphoma is one of the most common non-Hodgkin lymphomas (more than 20%) but occurs in most cases in adults with a median age around 60-65 years.
Several triggers
The number of cases rises to more than 4,000 per year in France. In children it is extremely rare. Moreover, the indolent nature of this tumor often leads to a relatively late diagnosis, when lymphoma is already disseminated, which may explain why it is more detected in older subjects.
In studying tumors, scientists discovered that these lymphomas were linked to a genetic translocation, i.e., a material exchange between two non-homologous chromosomes. This irreversible genetic incident would occur in the bone marrow inside some blood cells. But this alone would not be enough to trigger cancer and could be added to other genetic and even environmental factors.
Chemotherapy to control the evolution of
Follicular lymphoma cannot be completely cured. On the other hand, its evolution can be mastered because it responds well to the treatments chosen according to the stage of the disease, the condition of the patient and his age. If the lymphoma is located, it is possible to use radiation therapy. If it is already at an advanced stage, chemotherapy will be offered. It can be associated with a monoclonal antibody-based immunotherapy directed against tumor cells.
This can lead to a major remission but there is a significant risk of relapse. The condition of the patients must be regularly monitored. The 5-year survival rate is between 80-90% and median survival is about 10-12 years.
Mantle lymphoma, a form of non-Hodgkin's lymphoma
Mantle lymphoma, a form of non-Hodgkin's lymphoma
Mantle lymphoma represents 2 to 10% of lymphomas and in most cases affects men aged at least 50 years.
Of the non-Hodgkin type, mantle cell lymphoma is classified as malignant and aggressive lymphomas. Affecting the lymphatic system, it is characterized by an abnormal development of certain immune cells, the B lymphocytes present in a region of the lymph node, named "Zone of the mantle". Hence his name.
However, there are several forms of mantle tumor depending on the characteristics of abnormal cells. Currently, mantle lymphoma represents less than 10% of lymphomas and affects more middle-aged adults, especially around 65 years. In contrast, it is very rare in children and has a distinct male predominance, with four times as many men as women.
A relatively late diagnosis
To the extent that this lymphoma is painless and difficult to detect on a blood test, it is diagnosed at relatively late stages. In many cases, the cancer is already extended to other ganglion areas, to the bone marrow or to other particularly digestive organs. It is then associated with generalized adenopathies (ganglion inflammation) (in 90% of cases) and digestive disorders (60%).
By studying tumors, scientists have succeeded in highlighting the existence of a chromosomal translocation. This corresponds to an exchange of genetic material between two non-homologous chromosomes (here, 11 and 14) and causes some cells to manufacture in excess a protein called cyclin D1. A production that leads them to develop abnormally. However, the origin of this translocation remains unknown and no genetic predisposition has been identified.
5-year survival rate from 50 to 70%
The current treatment includes intensive chemotherapy associated with monoclonal antibodies directed against molecules present on the surface of tumor cells. This helps to increase the effectiveness of chemotherapy but also has an effect on the body's healthy blood cells. This treatment is often followed by the autografting of cells taken and re-injected into the bone marrow.
Depending on the case, radiation therapy can also be offered. But there is a significant risk of relapse and resistance to treatment. Currently, the 5-year survival rate is in the order of 50% for advanced-stage lymphomas and 70% for early stages. The reported global medians of survival are generally between 3 and 5 years.
Burkitt's lymphoma
Burkitt's lymphoma
Burkitt's lymphoma is a non-Hodgkin's type tumor that is characterized by abnormal evolution of B lymphocytes. Its origin is most often viral.
Burkitt's lymphoma was described for the first time in Uganda in the years 1950. But it will take several years for the disease to be classified as lymphoma. Today, this form of tumor is the most common in children living in equatorial Africa. However, it also appears in the countries of Europe and North America, in children and adolescents. It is more rare in adults.
Like all lymphomas, this form of cancer attacks the lymphatic system but there are many forms. The endemic (African) Form and the sporadic (non-African) Form that do not have the same characteristics are distinguished. The first is most often characterized by localized swelling at the jaw level while the second appears more regularly at the tonsil or abdomen level.
Viral lymphoma
Whatever the form, lymphoma is manifested by an abnormal development of B lymphocytes that will propagate in the organism. In general, tumor growth is fast. Without treatment, it can be extended to the bone marrow, the central nervous system and various organs.
Unlike other lymphomas, the origin of Burkitt tumors is much better known. This is at least the case for the African form that has been clearly linked to the Epstein-Barr virus (EBV). This microorganism is part of the Herpesviridae family and is one of the most common human viruses in the world. He is responsible for several diseases including infectious mononucleosis.
In Africa, most Burkitt lymphomas are caused by a succession of infections usually initiated by the EBV virus followed by another microorganism, such as the malaria parasite, for example. A genetic translocation would then occur, i.e., an exchange of material between non-homologous chromosomes (8 and 14) that would lead to abnormal lymphocyte development. This cancer is the first to have been associated with a viral cause.
A survival rate that exceeds 70%
However, other viruses may also be responsible for them. Very often, sporadic forms observed in Europe or the United States are therefore not linked to EBV. In addition, there is a third form of Burkitt lymphoma that appears in immunocompromised individuals and particularly infected with the AIDS virus.
As with all cancers, the more the tumor will be treated early, the more effective the treatment will be. In general, it consists of chemotherapy combined with radiation therapy. As evolution is rapid, patients need to be closely monitored, but the long-term survival rate is now much better, between 70 and over 90%. There is however a risk of relapse with a less good prognosis.
The lymphoma of MALT
The lymphoma of MALT
The lymphoma of the MALT for "lymphoid tissue associated with mucous membranes" is a malignant lymphoma of non-Hodgkin's type. It is however rather rare and affects mostly adults over 60 years.
Representing about 5% of non-Hodgkin lymphomas, MALT lymphoma is a rare form of malignant tumor that attacks the lymphatic system. It is particularly characterized by an abnormal development of B lymphocyte at the level of the lymphoid tissue associated with the mucous membranes ( "MALT"). This tissue is diffuse in several organs such as the digestive tract, the lungs or even the skin.
Today, the disease essentially affects adults over 60 years of age with a greater incidence among women. On the other hand it is very rare in the child. In many cases, lymphoma appears first at the level of the digestive tract, especially the stomach. But it can also develop at the level of the lungs, glands such as lacrimal glands, thyroid or breasts, or even, although more rarely, at the level of the lymph nodes.
The damage will therefore depend on the organs affected: it may be chronic gastritis in the event of a stomach attack, respiratory infections in the event of affected lungs or a visual deficiency if the lacrimal glands are affected. Lymphoma is then identified by observing the lymphoid tissue and the cells in it.
Lymphoma often of bacterial origin
Unlike most lymphomas, the causes of MALT lymphoma are a little better known. In most cases, it would be associated with an autoimmune disease or chronic infection. Indeed, there is a well established link between this form of gastric tumor and the bacterium Helicobacter pylori which causes 80% of gastro-duodenal ulcers. Nevertheless, it would not be responsible for all the lymphomas of MALT and other bacteria would also play a role in its appearance.
On the other hand, the observation of tumor cells has made it possible to highlight in the majority of cases a chromosomal translocation, that is, an exchange of genetic material between two non-homologous chromosomes, in this case between the Chromosomes 11 and 18. This would lead to the merging of two genes, involved in the regulation of apoptosis, the process that allows cells to trigger their self-destruction. Would then result in an anarchic multiplication of the cells.
Antibiotics for localized lymphoma
When gastric impairment is localized and due to H. pylori, antibiotic treatment may allow the lymphoma to regress (in 60 to 80% of cases). In others, chemotherapy and/or radiation therapy must be used.
However, the presence of translocation may lead to resistance to treatment and require the use of monoclonal antibodies to increase its efficacy. Surgery can also be considered according to the condition of the patient, his age and the area affected.
With low-scalable lymphoma, it can be supported at a relatively early stage. The survival rate at 10 years after treatment is then 75%. But there is a risk of relapse, calling for regular patient follow-up.
Lymphoma: symptoms, screening and diagnosis
Lymphoma: symptoms, screening and diagnosis depending on tumor forms, lymphomas can cause a variety of symptoms ranging from non-specific signs like fever to pain in the affected organs. This must then lead to further examination and analysis.
Lymphomas are not simple cancers to be screened, as they can grow in any organ containing lymphoid tissue. But tumors can lead to the onset of some suspicious symptoms, capable of suggesting an attack. Depending on the forms of lymphoma, these will vary however.
In 80% of cases, the circumstances of discovery will involve a lymphadenopathy. This corresponds to an inflammation of a lymph node which then starts to grow and becomes palpable. This can occur at the neck, groin or over the clavicle with a rapid onset and a large volume (greater than 1 to 2 centimeters).
Sometimes, however, the lymphadenopathy is discreet because it is profound or non-existent in some cases. The discovery may then take place during a routine X-ray examination or as a result of specific signs such as respiratory discomfort.
Fever or persistent fatigue
In other cases, these are quite general but persistent symptoms that can lead to a doctor's visit. In fact, according to the forms, lymphomas can cause fever, fatigue, sweats, significant weight loss or pruritus on the skin. When organs are reached, other more serious symptoms may alert such as gastritis, respiratory infection or the perception of a mass at the abdominal level (this may be a large liver, a large spleen or a ganglionic mass abdominal).
Of course, these signs can also be associated with other less severe pathologies that have nothing to do with lymphoma. If they can guide a diagnosis, then other tests are imperative to be able to rule out other diseases and confirm the presence of a tumor. The principal is to observe the cells by removing tissue (biopsy) in one or more lymph nodes (in most cases) or in another suspicious area.
Confirm lymphoma and determine its type
Studied under a microscope, the tissue will make it possible to affirm or not the presence of tumor cells and if this is the case, to determine the form and characteristics of the lymphoma. This review will then be completed with a full clinical record as well as other tests to assess the stage of cancer and the affected areas.
This includes blood tests, X-rays, chest and abdomen scanners, or a bone marrow examination. The information obtained through these tests will make it possible to establish a precise diagnosis and to opt for the treatment best adapted given the age and the condition of the patient.
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