What is Lymphoma?
A lymphoma is a cancer of lymphatic cells in the lymphatic system. About 54 percent of the blood cancers that occur each year are types of lymphoma. Lymphomas are divided into two types - Hodgkin's lymphoma and non-Hodgkin's lymphoma. There are various types of non-Hodgkin's lymphoma which are divided into high-grade (fast growing) and low-grade (slow growing).It is important to know exactly what type you have. This is because the treatments and outlook (prognosis) can vary for different types of lymphoma. Majority of lymphoma can be cured with treatment
What is the lymphatic system?
The lymphatic system consists of lymph nodes (glands), a network of thin lymphatic channels (like thin blood vessels), and organs, such as the spleen and thymus.
The lymphatic system also forms a major part of the immune system. Lymph and lymph nodes contain white blood cells called lymphocytes and antibodies which defend the body against infection. The lymphocytes are made in the bone marrow. When they are mature they are released into the bloodstream and migrate into the lymphatic system.
There are three types of mature lymphocytes:
- B lymphocytes which make antibodies that attack infecting bacteria, viruses, etc.
- T lymphocytes (which finish off their maturing in the thymus gland) have various functions, including helping the B lymphocytes to make antibodies.
- Natural killer lymphocytes which also help to protect against infection.
What causes a Lymphoma and how does it develop?
The exact cause is not known. If your immune system is down (for example, if you have AIDS) your risk of developing a lymphoma is increased. A previous infection with a virus called the Epstein-Barr virus, HTLV may increase the risk slightly. However, many people have an infection with the Epstein-Barr virus, and the vast majorities do not develop Hodgkin’s lymphoma. However, this only accounts for a small number of cases and the cause is unknown for most people. It is not an inherited condition and does not run in families.
Origin of a cancer (such as a lymphoma) starts from one abnormal cell. Exactly why the cell becomes cancerous is unclear. It is believed to be something that damages or alters certain genes in the cell making the cell abnormal. These cells multiply and produce many more abnormal cells. The cancerous lymphocytes tend to collect in lymph nodes. The lymph nodes become bigger and form cancerous tumours. Some abnormal cells may travel to other parts of the lymphatic system, such as the spleen. So you may develop lots of large cancerous lymph nodes and an enlarged spleen. Cancerous lymphocytes can also form lymphoma tumours in places in the body outside of the lymphatic system. This is because lymphocytes can also travel in the bloodstream.
Who gets non-Hodgkin's Lymphoma?
Anyone can be affected. Most cases occur in people over the age of 60. Men are more commonly affected than women.
Who gets Hodgkin's Lymphoma?
Anyone can be affected, including children. However, most cases occur between the ages of 20 and 25 years, or after the age of 70. It is one of the few cancers where there is a peak of incidence in young adults.
What are the signs & symptoms of Lymphoma?
Enlarged Lymph Nodes: The most common early symptom is to develop one or more swollen lymph nodes in one area of the body - most commonly the side of the neck, an armpit or the groin. The swollen lymph nodes tend to be painless and gradually get bigger. If the affected lymph nodes are in the chest or abdomen, you will not be aware of them swelling in the early stages of the disease.
However, the most common cause of swollen lymph nodes is infection. A lymphoma may be suspected if lymph nodes remain swollen, or if there is no infection to cause the swelling.
Various other general symptoms
may also develop, e.g.:
- Recurrent fevers (greater than 38˚C)
- Night sweats (drenching)
- Weight loss (greater than 10% in less than six months)
- Fatigue (severe and persistent)
As a lymphoma develops you may feel generally unwell. If the lymphoma tumours become large and press on nearby parts of the body, various other symptoms can develop. For example, you may develop a cough or breathing problems if the tumour enlarges in the lymph nodes inside the chest, stomach pain if the lymphoma develops in the stomach.
How is Lymphoma diagnosed?
Lymphoma is generally diagnosed on biopsy of enlarged lymph nodes. A biopsy is when a small sample of tissue is removed from a part of these nodes. Sometimes an entire lymph node is removed. The sample is then viewed under a microscope to look for abnormal cells, patterns and grade of lymphoma. In Hodgkin's lymphoma, a cell called the Reed-Sternberg cell is seen under microscope.
The additional test like immunohistochemistry (IHC) is performed for identifying subtypes of lymphoma. Some cases this sample may be processed for cytogenetics or immunophenotype.
What are different types of non-Hodgkin’s Lymphoma?
Non-Hodgkin’s lymphoma is also divided as High-Grade (fast-growing) or Low-Grade (slow-growing) depending on grade and B cell or T cell depending upon cell of origin. The common types of non-Hodgkin’s lymphomas include: diffuse large B-cell lymphoma, lymphoblastic lymphoma, follicular lymphoma, anaplastic large-cell lymphoma, lymphoplasmacytic lymphoma and mantle cell lymphoma – but there are various other types. Different types of lymphoma are treated differently and different outlook.
What are different types of Hodgkin’s Lymphoma?
There are various sub-types of Hodgkin’s lymphoma. It is divided in classical Hodgkin’s lymphoma and lymphocyte predominant Hodgkin’s lymphoma as treatment of these two types different.
How is Lymphoma staging done?
The aim of staging is to find out how much the lymphoma has grown locally, and whether it has spread to other lymph nodes or to other parts of the body. For determining stage of lymphoma CT scan, blood tests, a bone marrow biopsy or other tests are performed. Nowadays PET/CT scans are considered superior to CT scans and preferred. All staging investigations are repeated at end of treatment for documenting complete clearance of lymphoma. PET scan can be done in interim after 2-3 cycles of chemotherapy for assessing initial response to chemotherapy.
Ann Arbor staging of Lymphoma
- Stage 1: The lymphoma is confined to one group of lymph nodes only.
- Stage 2: The lymphoma affects two or more groups of lymph nodes. However, they are all on the same side of the diaphragm. (The diaphragm is the large muscle that separates the chest from the abdomen and helps us to breathe. So, for stage 2, all the affected nodes will either be above or below the diaphragm.)
- Stage 3: The lymphoma affects nodes on both sides of the diaphragm.
- Stage 4: The lymphoma affects parts of the body outside of the lymphatic system.
Each stage is also divided into A or B. ‘A’ means that you do not have symptoms of night sweats fevers or weight loss. ‘B’ means that you do have one or more of these symptoms.
What are the different types of treatments for Lymphoma?
The treatment advised for each case depends on various factors such as the exact type and stage of the lymphoma. It depends on, whether it is high or low-grade, your age, your general health, the size of the affected nodes, and which parts of the body are affected. In general, the goal of treatment is to destroy as many lymphoma cells as possible and to induce a complete remission; that is, to eliminate all evidence of disease. Patients who go into remission are sometimes cured of their disease.
In general, chemotherapy and radiation therapy are the two principal forms of treatment for lymphoma. Although radiation therapy is not often the sole or principal curative therapy, it is an important additional treatment in some cases. Stem Cell Transplantation and a watch-and-wait strategy are also used to treat some lymphoma subtypes.
What is treatment of non-Hodgkin Lymphoma (nHL)?
Some early stage low grade NHL may just be observed and not need any treatment until they progress. Rest of NHL will need chemotherapy with or without radiotherapy.
In general R-CHOP chemo-immunotherapy is commonest chemotherapy used in this patient, however there other different protocols for some types. It is given as injections every 21 day cycle for 3-8 cycles depending upon stage of the lymphoma. Those patients who have heavy or bulky disease are given Involved Field Radiation Therapy (IFRT) which targets specific area without damaging the surrounding normal tissue.
What is treatment of Hodgkin Lymphoma (HL)?
Early stage favorable HL treated with 3 cycles of ABVD chemotherapy (most common protocol) followed by IFRT. Early stage unfavourable and advanced HL is treated with 6 cycles of ABVD chemotherapy with IFRT for those patient with bulky areas. Each ABVD chemotherapy cycle include ABVD chemotherapy to be given 2 times 2 weeks apart. That means for 6 cycles, the patient would come 12 times for chemotherapy
Do I need to get admitted for treatment?
Except for very aggressive lymphoma where high intensity chemotherapy given inpatient, majority of times you would be given chemotherapy in daycare facility where you come in morning on the day of chemotherapy and discharged on same day after chemotherapy.
What are common side effects of chemotherapy?
Most common side effect is nausea, vomiting, throat pain and mouth ulcers. You would loose your hairs temporarily with chemotherapy. Your WBC count and platelet count would fall after 7 days of chemotherapy during which you have to be careful about catching infections. They will start to recover before next chemotherapy cycles. There are other uncommon side effects of chemotherapy which your doctor would discuss with you.
What is role of stem cell transplant in treatment of Lymphoma?
Relapsed (disease coming back) or refractory (disease not responding to chemo) lymphoma can be given high dose chemotherapy followed by Autologous Stem Cell Transplants in which case stem cells from the patient (self) are collected after chemotherapy cycle and given back after high dose chemotherapy of transplant.
Some of young patient and refractory patient are treated with Allogenic Haematopoietic Stem Cell Transplant where stem cells are taken from HLA matched donor.
How do you follow-up in Lymphoma patients?
Follow up care is important with both aggressive and indolent forms of lymphoma because, even if the disease recurs, curative options are still available for many people. Follow-up care needs to be individualised and should be based on several factors, including how the disease initially manifested. Patients who are in remission should continue to be examined regularly by their doctors.
Periodic assessment of the patient's state of health, blood cell counts and, if necessary, marrow is important. Over time, the interval between assessments may be lengthened, but assessments should be continued indefinitely.