Lymphoma and Hodgkin's disease: Understanding
Words for EvilLymphoma is a cancer of the lymphatic system characterized by the uncontrolled growth of abnormal lymphocytes.
Cancerous lymphocytes develop in lymphoid organs that are located in many organs of the body in addition to the lymph nodes (lymphadenopathy) and spleen (splenomegaly).
Lymphoma is the first blood cancer in frequency and as it touches the immune system, it is accompanied by an immune depression (immunosuppression)
The treatment is based on a combination of chemotherapy (polychemotherapy), irradiation treatment (radiation therapy) and in some cases monoclonal antibodies (biotherapy) and bone marrow transplant.
What is lymphoma?Lymphoma is a cancer of the lymphatic system characterized by the uncontrolled growth of abnormal white blood cells that are usually responsible for controlling infections: lymphocytes.
When these lymphocytes become abnormal, they develop uncontrolledly in lymphoid organs that are located in many organs of the body (lung, liver, intestine) in addition to the lymphoid organs: lymph nodes, spleen, bone marrow, thymus. Due to the fundamental role of the lymphatic system in immune defences, lymphoma will be accompanied by an immune deficiency.
The lymphatic system is a network that makes and allows the circulation of the lymph throughout the body. Lymph is a clear transparent liquid that contains lymphocytes, essential cells of immune defense. Along this network are small bean-shaped organs called lymph nodes. The lymph nodes are responsible for the production and storage of lymphocytes to fight infections. The lymph nodes are particularly numerous in the neck, under the armpits, in the groin and in the abdomen.
Lymphoma and Hodgkin's disease: diagnosis
When should lymphoma be evoked?
The persistent and painless swelling of a ganglion of the upper body, i.e. the neck, the area of the clavicles, the armpits or the groin should evoke lymphoma (a swollen ganglion during lymphoma is normally not as Painful than an infected lymph node).
This lymph node is all the more suggestive of lymphoma as it is accompanied by signs showing a generalized process:
• Chills
• Changes in body temperature
• Fever (especially at night)
• Lower Appetite
• Unexplained weight loss
• Unusual General Fatigue
• Persistent cough
• Persistent itching of the whole body without any apparent cause or disease of the associated skin or liver
• headaches
• At a later stage, patients may experience pain in the lymph nodes after consuming alcohol.
How to diagnose?Because of the fairly common nature of lymphoma complaints and because the most suggestive signs of lymphoma do not appear in all cases, diagnosis is often problematic.
The signs of lymphoma are, in fact, frequently observed in other less severe illnesses such as influenza or any other viral infection. These symptoms are often neglected. But it is important to remember that in infectious diseases these signs do not last very long. In the case of lymphoma, these signs persist over time and cannot be explained in relation to an infection or other disease.
Most people who complain about the signs that we encounter during lymphoma do not have lymphoma. However, it is important for anyone with signs such as lymphoma who persists to consult a doctor to confirm the absence of lymphoma or serious illness.
The doctor will perform a complete physical examination and check that there are no swollen lymph nodes under the chin, neck, shoulders and elbows, under the armpits and groins. The doctor will also examine the other parts of the body to see if there is swelling of the tonsils or liquid in the chest or abdomen, which could be caused by swollen lymph nodes.
The doctor will ask if a pain is felt and where? And it will check that there is no sensory deficit or paralysis that could be the result of compression of the nerves or spinal cord by a hypertrophied ganglion.
It will examine the belly to see if no internal organ is hypertrophied (liver, spleen).
If the doctor suspects lymphoma, he or she prescribes a battery of additional tests including a biopsy, blood tests, X-rays and bone marrow assessment.
Should we do a ganglion biopsy?The biopsy of an inflated ganglion (lymphadenopathy) is one of the most important steps since it allows diagnosis.
A lymph node puncture may be a first step in distinguishing lymphoma from infection or even metastasis, but it does not provide a biopsy.
The quality of the biopsy is very important to be able to do an accurate study that will be: histologic, phenotypic, molecular and possibly karyotype (study of chromosomes in cell nuclei).
CT-guided biopsy may be useful if it finds lymphoblastic cells or Burkitt cells because, diagnosing the corresponding diseases, it will then dispense ganglion and marrow biopsy. On the other hand, the samples are often of lower quality and the material is insufficient to be able to precisely type lymphoma, which is not without therapeutic effect.
So the safest is to plan the complete removal of a ganglion by a surgeon under general anesthesia. The biopsy should be transmitted very quickly to the pathology laboratory.
If only the spleen is reached, the spleen must be removed for analysis.
Lymphoma is oriented when the Panleucocytaire antibody (CD45) is positive and the anti-cytokeratin antibody is negative (eliminating metastasis of carcinoma).
Second, examination of the biopsy orients to Hodgkin's lymphoma if Reed-Sternberg cells are found.
In their absence, it is directed towards non-Hodgkin's lymphoma type B or T, which is then possible to type using different markers on the surface of the cells (CD5, BCL-2, Cyclin D1, EBV...).
Are other exams needed?The remainder of the supplementary examinations is mainly of prognostic interest: they are used to assess the condition of the patient and especially the extension of the lymphoma in the body, which is of a major interest in the choice of treatment: it is the "balance of extension".
• X-rays: These are all the imaging exams in which low-dose × beams are used (lung, skeleton...).
• Thoraco-abdominal and pelvic tomography with contrast product injection to better separate the vessel-related images from those linked to the lymph nodes.
• Cerebral MRI (magnetic resonance imaging) for cerebral lymphomas. An MRI is similar to a CT scan, but it uses magnetic fields instead of X-rays.
• Pet (Positron emission tomography): This is a process that allows the visualisation of cancer in the body. Radioactive glucose (a sugar molecule used as a source of energy by the cells) is injected into the patient and preferably used by cells with high metabolic activity, such as cancer cells. A scanner then allows to visualize the areas of the body in which the radioactive glucose is concentrated.
• High endoscopy in the event of a call sign such as iron deficiency anemia or in the context of a MALT or mantle-type NHL.
• An ENT examination to investigate the damage to the lymph organs of the mouth and nose.
• Bone marrow biopsy Determines if lymphoma has invaded the bone marrow (if it has spread in it).
• Blood hepatic balance is used to seek associated liver damage.
• Other blood tests seek anemia, thrombocytopenia, or circulating lymphoma cells on the NFS. The assay of serum LDH and beta-2-microglobulin is essential for evaluating tumour mass. Blood Ionogram and creatinine measurement are important to seek renal impairment. The measurement of albumin on serum protein electrophoresis is a prognostic factor in Hodgkin's disease.
All of this information is used to establish a specific diagnosis and to classify lymphoma in several stages to which specific treatments (e.g. ANN Arbor classification) correspond.
Further examinations may be necessary before the start of treatment (Serology of hepatitis C and B, search for EBV and Helicobacter pylori in the NHL MALT, cardiac ultrasound to assess cardiac function before administration of Chemotherapy based on alkylating...). Finally, other prognostic tests may be available in specialized laboratories or can only be carried out for research purposes. Currently, treatment decisions are generally based on clinical outcomes (symptoms, physical examination and blood tests).
Finally a cryopreservation of sperm in young humans and ova in the young woman is necessary to preserve their chances of reproduction after treatment.
What are the signs of lymphoma?
The signs of lymphoma are not very specific and often similar to other diseases such as infectious diseases and digestive diseases.
The most common sign is a painless swelling of a lymph node (called "Lymphadenopathy" by doctors). This may be the reason for the consultation, but the patients often consult the doctor because they think they have a general malaise, a cold that does not want to pass or a digestive disorder.
At an early stage of development of lymphoma, there is usually no pain of adenopathies, which is atypical because, in case of infection and the fight of the immune system against bacteria or viruses, the lymph nodes are swollen and painful .
The areas of the neck and armpits are the sites where abnormal lymph nodes are most often observed first. But the swelling may appear on other parts of the body, such as the groin (which may result in secondary swelling of the legs or ankles) or abdomen (which can be associated with cramps and bloating).
However, some patients with lymphoma do not notice any swelling of their lymph nodes because they are in the belly or chest. Others complain only of night sweats, loss of weight, shivering, lack of energy.
Even more atypical, lymphomas that reveal themselves only by "sine materia" itching, i.e. without any signs of skin or liver disease.
In a few cases, Hodgkin's lymphoma may be a prolonged fever, with weight loss and inflammation on the blood test, without a large node or extra-ganglionic tumor.
Lymphoma at a more advanced stage can be accompanied by more suggestive signs:
• Patients may experience pain in the lymph nodes after consuming alcohol.
• If lymphoma touches lymphatic tissues inside the belly, a liquid can accumulate in the vicinity of the intestines, with abdominal pain, diarrhea, or stomach with digestive weights (such as indigestion).
• A highly inflated lymph node can sometimes compress a vein (which can cause swelling of an arm or leg) or a nerve (which can cause pain, numbness, tingling in a leg or arm). Some people complain of unexplained pain in the lower back that can be linked to hypertrophied lymph nodes that compress the nerve roots and nerves.
• As lymphoma evolves and cancer lymphocytes gradually invade the immune system and it loses its ability to fight infections.
• Some signs that appear as lymphoma develops (fever, shivering, anorexia, slimming, unusual fatigue, coughing...), depend on the synthesis of pro-inflammatory proteins (cytokines). They can be confused with signs of influenza, tuberculosis and other infections such as infectious mononucleosis or other cancers.
What are the different types of lymphoma?
The word "lymphoma" is a general term that is used to refer to the heterogeneous aggregation of over 50 different cancers of lymphoid origin T or B.
There are two general categories of lymphoma: Hodgkin's disease and non-Hodgkin's lymphoma (NHL). The main difference between Hodgkin's disease and the NHL is the presence of reed-Sternberg cells, which allows the diagnosis of Hodgkin, because the reed-Sternberg cell is a cell derived from a B lymphocyte that is present only in the Hodgkin. Non-Hodgkin's lymphoma develops from other B lymphocytes in 85% of cases and T lymphocytes in 15% of cases.
Many types of non-Hodgkin's lymphoma are distinguished, depending on the appearance of the cells affected by this condition (which results in many names), but are also classified according to their histological degree of aggression (low grade, intermediate grade and high grade), which is more useful for treatment. Overall, these lymphomas are classified into two major groups:
• Forms that evolve very quickly, so-called "aggressive". They affect 50 to 60% of people suffering from the disease, and must be taken care of as soon as possible.
• Forms of slow evolution, called "indolent". They concern 40 to 50% of patients and will develop over several years.
From one case to the next, the symptoms, the development of the disease and the reaction of the organism to the treatment are different, but many patients can now heal.
Are there any risk factors for lymphoma?
The exact causes of lymphoma are not known. However, the number of cases of non-Hodgkin's lymphoma reported has been multiplied by two in thirty years. The search for the causes of this increase has led to a lot of studies and some have concluded the possible influence of some factors studied:
• Chronic viral infections (Epstein-Barr virus in Burkitt lymphoma, HIV, HTLV-1, hepatitis C) or bacterial (in particular, prolonged presence of Helicobacter pylori in the stomach for MALT lymphoma),
• Prolonged immune deficiency (in the course of autoimmune diseases such as Sjögren's syndrome or rheumatoid arthritis), or during immunosuppressive treatments (e.g. in the case of grafting),
• Exposure to certain toxic substances (dioxins, pesticides, solvents, fertilizers), chemotherapy with drugs containing elements called "alkylating agents" or radiation therapy.
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