Primary mediastinal lymphoma with large B cells
Primitive mediastinal lymphoma with large B cells (LMPGB) can also be called B-cell mediastinal lymphoma or mediastinal lymphoma (thymic) with large B-cells. It is a subtype of diffuse large cell lymphoma B (LDGCB) that evolves rapidly (aggressive). It appears in the thymus or in the lymph nodes located in the center of the thorax (mediastinum).
The LMPGB is not common and it most often affects the elderly between 30 and 40 years and a little more the woman than the man.
Symptoms
People with a LMPGB often have a large mass in the thorax. It can cause symptoms as it grows like these:
Shortness
Cough
Chest Pain
Partial blocking of the main vein that circulates the blood from the upper part of the body to the heart (upper Vena cava), which may cause the compression syndrome of the upper vena cava
If the LMPGB reappears (recurrence) after treatment, it often does so in organs or tissues located outside the lymph nodes (extraganglionnaires seats). These organs and tissues are among other things the kidneys or the brain and the spinal cord (which together form the central nervous system, or CNS).
Treatments
The treatment options for the LMPGB are as follows.
ChemotherapyChemotherapy is often offered as a treatment for LMPGB. A frequently administered Chemotherapeutic association is the CHOP Protocol:
Cyclophosphamide (Cytoxan, Procytox)
Doxorubicin (Adriamycin)
Vincristine (Oncovin)
Prednisone
When we add rituximab (RITUXAN) to this association, the protocol is called R-CHOP.
Targeted treatmentTargeted treatment uses drugs to target specific molecules (such as proteins) located on the surface of cancer cells. These molecules contribute to the sending of signals that tell cells to grow or divide. By targeting these molecules, medications interrupt the growth and spread of cancer cells while limiting damage to normal cells.
Rituximab (Rituxan) is a targeted drug that can be associated with chemotherapy to treat LMPGB.
RadiotherapyExternal radiation therapy is often administered to the chest mass after chemotherapy.
The lymphatic system
Non-Hodgkin's lymphoma (NHL) is a type of cancer that originates in the lymphatic system. The lymphatic system consists of a network of lymph, lymphocytes, lymphatics, lymph nodes and lymphatic organs. These organs are spleen, thymus, tonsils, adenoids vegetation and bone marrow. Lymphatic tissue is also found in other parts of the body, including the stomach, intestine and skin.
The organs and tissues of the lymphatic system manufacture and store cells that fight infections and diseases. The lymphatic system works in conjunction with the circulatory device, the heart and blood circulation, and other parts of the immune system to keep the body healthy.
Diagram of the lymphatic system
Structure
The lymphatic system is composed of the following organs and tissues.
Lymph
Lymph is a clear, yellowish liquid that carries lymphocytes, macrophages and other white blood cells. It also carries protein and nutrients. The lymph circulates throughout the body through a network of lymphatic vessels and the tissues bathe in it. Lymph can also be called lymphatic fluid.
Lymphocytes
Lymphocytes are a type of white blood cells. They fight against viruses, bacteria, foreign substances and abnormal cells, including cancer cells. The main types of lymphocytes are:
B lymphocytes (b cells), which manufacture antibodies to fight bacteria, viruses and other foreign substances such as fungi;
T lymphocytes (T cells), which fight infections, destroy abnormal cells and control the immune response;
Natural killer Cells (NK), which attack any abnormal or alien cell.
All lymphocytes begin to develop in the bone marrow. B lymphocytes remain in the bone marrow until they reach maturity. T lymphocytes migrate to the thymus to further their development. Once matured, lymphocytes move to the lymph nodes and spleen.
Lymphatic vessels
The lymph circulates throughout the body through the lymphatic vessels. There are 3 main types of lymphatic vessels.
The lymph capillaries are tiny tubes in cul-de-sac through which the tissue fluid enters the lymphatic system.
The lymph vessels are tubes that bring the lymph into the lymph nodes and evacuate it.
The collecting tubes return the lymph to the bloodstream.
lymph nodes
The lymph nodes are small bean-shaped organs that follow the lymph vessels and filter the lymph. The size of the lymph nodes varies, but they usually measure less than 1 cm. They can reach up to 1.5 cm in the groin.
We find a lot of lymph nodes scattered all over the body. The number of lymph nodes varies from one part of the body to the other. The lymph nodes are grouped in the following main regions:
Neck (cervical ganglia)
Thorax (thoracic ganglia and mediastinal)
Armpits (axillary lymph nodes)
Abdomen (para-aortic, peri-aortic, and mesenteric ganglia)
Groin (inguinal ganglia)
Lymph nodes filter harmful particles from the lymph before returning it to the bloodstream. These include bacteria, viruses and foreign substances. The other main function of the lymph nodes is to activate the immune system.
The lymph nodes contain 2 types of white blood cells that fight the invasive micro-organisms. Lymphocytes attack viruses, bacteria and other micro-organisms. Macrophages surround and destroy foreign substances, damaged cells and cellular debris.
A lymph node or group of lymph nodes a large number of particles may or may become swollen and sensitive to the touch – a sore throat, for example, can cause swelling of the lymph nodes located under the jaw and in the neck.
Rate
The spleen is the biggest lymphatic organ. It is located in the upper left part of the abdomen.
The spleen filters the blood by removing the old red blood cells, lymphocytes and invaders, such as viruses and bacteria. The spleen also stores red blood cells and lymphocytes.
Thymus
The thymus is located in the thorax, behind the sternum. This is where the lymphocytes reach maturity and multiply.
Tonsils
The tonsils are small masses of lymphatic tissue that contain lymphocytes. The palatine tonsils are at the back of the mouth. We also have another pair of tonsils at the base of the tongue called lingual tonsils.
Tonsils help the body fight infections by trapping bacteria and viruses. They also produce antibodies.
Adenoids vegetation
Although we often talk about adenoids vegetation in the plural, there is only one. It is a single small mass of lymphatic tissue located at the back of the nose that contains lymphocytes. It is also sometimes called tonsil pharyngeal.
Adenoids vegetation helps the body fight infections by trapping bacteria and viruses.
Present in infants and children, adenoids vegetation begins to decrease in volume just before puberty, which is why most adults do not.
Marrow
The bone marrow is the soft and spongy tissue located in the center of most bones. It contains the immature blood cells called stem cells. These stem cells are transformed into red blood cells, white blood cells or platelets. Red blood cells bring oxygen to the tissues and remove carbon dioxide. White blood cells help the body fight infections and diseases. Platelets help the blood clot.
Function
The lymphatic system:
Removes excess fluid from the tissues and returns it to the bloodstream;
Helps the body fight disease
Helps circulate hormones and nutrients throughout the body
Helps to remove waste from tissues.
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