The lymphatic system is an important system in the body that consists of lymph nodes and a vascular network, and the lymph fluid circulates within this vascular network. Lymph fluid contains white blood cells that fight microorganisms that have the potential to cause disease in the body. Lymph nodes (nodes) function like a filter, preventing the spread of viruses and bacteria in the body. Lymph cells forming the lymph system (lymphocytes) tend to proliferate uncontrollably and may lead to the development of lymph cancer.
The medical name for lymph cancer is lymphoma. When evaluated among all cancers, the detection rate of lymphoma is around 5%. It occurs as a result of excessive proliferation of blood cells called lymphocytes in the lymph nodes. Malignant lymphocytes can also multiply in the spleen, liver, bone marrow and other organs besides the lymph nodes. Lymph node cancer is medically divided into Hodgkin Lymphoma and Non Hodgkin Lymphoma. Non Hodgkin lymphoma is the more common type of lymph cancer. Both types of lymphoma have subtypes. These are important because they determine the course of the disease and treatment. Lymphoma can also be divided into groups as rapidly and slowly progressive. Lymphoma is more common in women than men.
Lymphoma, which occurs most frequently in young adults, is one of the most common types of cancer in childhood.
What is Hodgkin lymphoma (HL)?
Hodgkin lymphoma is a monoclonal (overgrowth of one type) cancer of lymphoid tissue cells, a condition with high cure rates. Biological and clinical studies on this disease have shown that the disease is classical Hodgkin lymphoma and nodular lymphocyte predominant Hodgkin lymphoma.
It has been evaluated by dividing it into two main categories. In pathological examinations of Hodgkin lymphoma, “Reed-Sternberg cells” originating from the characteristic B cells are detected.
Classical hodgkin lymphoma is the type of hodgkin lymphoma that is detected in approximately 95% of this lymph cancer type. These cancers tend to start in the lymph nodes, usually in the cervical (neck) region. Although the exact underlying cause of the disease is not known, there is an increased risk for the development of Hodgkin lymphoma in people infected with Epstein Barr virus, in autoimmune diseases and in people who are immunocompromised.
Hodgkin lymphoma, which usually occurs in the young adult age group, has a cure rate of approximately 80%.
What is Non-Hodgkin lymphoma (NHL)?
Non-Hodgkin lymphoma, another type of cancer that occurs in the lymphoid tissue, originates from mature B and T lymph cells in this tissue and stem cells that provide the formation of these cells.
The most common types of NHL arising from mature B lymphocytes include follicular lymphoma, Burkitt lymphoma and diffuse large B-cell lymphoma, Mantle cell lymphoma, marginal zone lymphoma, and primary central nervous system lymphoma. NHL arising from T cells includes adult T-cell lymphoma and mycosis fungoides species.
Treatment for these different types of non-Hodgkin lymphoma may vary depending on the stage and grade of the tumor, the type of cancer, and patient-related factors. Non-Hodgkin lymphoma is most commonly detected in people aged 65-74 years.
What are the symptoms of lymphoma?
Lymphoma may not always cause symptoms in the early stages. As the disease progresses, symptoms that mimic many diseases occur and these symptoms may change according to the stage of the disease. Sometimes a unilateral enlarged tonsil or soft subcutaneous nodules can be diagnosed as lymphoma. These lymph nodes can be detected in many areas of the body:
- Neck
- upper chest area
- underarms
- Gastric
- Groin
The diagnosis of lymphoma can sometimes be overlooked because the early signs of the disease are not selective. Many signs and symptoms may accompany lymph node enlargement at this stage:
- Cough and shortness of breath
- tonsil swelling
- High fever
- Night sweats
- Weakness
- Unexplained loss of weight and appetite
- Abdominal cramps
- Itching
- Bone pain
- enlargement of the spleen
- Pain after drinking alcohol
What are the causes of lymphoma?
In lymph cancer, cells called lymphocytes turn into lymphoma cells. These cells multiply uncontrollably in lymph nodes and other tissues, forming masses. In most cases, the cause of the disease is unknown. However, a relationship was found between EBV and HIV infections and lymphoma. When more than one person from the same family has lymphoma, it is also thought to be a genetic factor. Lymphoma is more common in people with compromised immune systems. Benzene and pesticides are also among the causes of the disease.
Various environmental, infectious and genetic factors may predispose people to develop lymphoma:
- Occupational Exposure
People working in the field of agriculture may be exposed to herbicides and pesticides used against weeds and pests. This exposure is associated with an increased risk of developing lymphoma.
- Infectious Causes
Diseases caused by various microorganisms have been associated with the development of different types of lymphoma. Bacteria called Helicobacter pylori MALT (mucous-associated lymphoid tissue) lymphoma, bacteria called borrelia burgdorferi, chlamydia psittaci, campylobacter jejuni, T cell lymphotropic virus adult T cell lymphoma, hepatitis C virus lymphoplasmacytic lymphoma or diffuse large b cell lymphoma, HHV8 may cause primary effusion lymphoma and Castleman disease.
Apart from these microorganisms and diseases, there is a risk of developing lymphoma in viruses such as Epstein Barr virus and cytomegalovirus, which cause long-term stimulation of lymphoid tissue.
- Immune (immune) Deficiency
Lymphoma may occur in people with HIV infection, drugs used to prevent rejection after organ transplantation (transplant), or people with genetically immune-related diseases.
- Autoimmune Diseases
Diseases in which the immune system produces antibodies against its own cells and tissues and causes damage to these structures are referred to as autoimmune diseases. Inflammatory bowel diseases (IBD), rheumatoid arthritis and Sjögren's syndrome are among the diseases included in the autoimmune disease classification. While enteropathy-associated lymphoma may occur in IBD, there is a risk for the development of diffuse large B-cell lymphoma in rheumatoid arthritis and Sjögren's syndrome.
How is lymph node cancer diagnosed?
Patients usually apply to health institutions because of enlarged lymph nodes. However, since lymphoma can mimic many diseases, it may be possible to miss the diagnosis of this disease in the early stages.
Although physicians request various blood tests, the main diagnostic one is lymph node biopsy. If lymphoma cells are seen in the biopsy sample, the diagnosis is made. In order to understand the stage of the disease, bone marrow biopsy and various radiological examinations are performed. Chest X-ray, tomography, MRI and PET are among the examinations performed. The number and distribution of enlarged lymph nodes and the presence of other organ involvement are very important in staging the disease.
If the tissue biopsy result is lymphoma, a PET/CT scan is performed to determine in which part of the body the disease is active. In this examination, radiologically labeled fluorodeoxyglucose (FDG) substance is used on the patient. Energy consumption is very high in the tissues where the disease is active, so you can have an idea about which part of the body has lymphoma with the images taken during the use of this marked substance, which contains sugar.
Following the completion of the diagnostic approach to lymphoma, staging of the disease is required before treatment planning can be started.
The Ann Arbor staging system is used for clinical staging of both hodgkin and non-hodgkin lymphoma. Persistent fever, called B symptoms, weight loss of more than 6% of body weight in the last 10 months, and the presence of night sweats are among the parameters evaluated in the clinical classification. Lactate dehydrogenase and the results of the biochemical analysis determined by examining the blood of the other patient, the patient's metabolic markers and uric acid value are also within the parameters examined during the staging process.
The stages of lymphoma are generally as follows:
- stage 1
There is involvement in a single lymph node region or in a single lymphoid structure in the spleen, thymus or nasal region, or in a single region other than lymph.
- stage 2
More than one lymph node region is involved on the same side of the diaphragm. Lymphomas involving a single organ other than the lymph node on the same side of the diaphragm or involving the lymph nodes in that area and surrounding can also be classified as stage 2.
- stage 3
Lymphoma involving lymph node regions on both sides of the diaphragm is classified as stage 3. This involvement may be accompanied by spleen involvement or a regional non-lymph node organ involvement.
- stage 4
There is very common involvement in tissues and organs. If one or more non-lymph node organ involvement is detected in more than one focus, the disease is classified as stage 4.
How is lymph cancer treated?
Treatment of lymph cancer is done by oncologists in hematology-oncology services. With modern chemotherapy, 70-80% of lymphoma patients can be cured. Factors affecting the course of the disease; The stage of the disease, whether the patient responds to the treatment, the type of lymphoma, the recurrence of lymphoma, whether there is diabetes or kidney disease together.
Chemotherapy drugs used in the treatment of lymphoma cancer can be administered alone or in various combinations. These drugs are used to eliminate cancer cells and prevent their proliferation. Chemotherapy drugs used in the treatment of lymphoma are usually given to patients through the large venous line located in the chest area. There are 3 basic chemotherapy regimens used for Hodgkin lymphoma.
- ABVD regimen is a lymphoma treatment regimen containing chemotherapy drugs with active ingredients doxorubicin, bleomycin, vinblastine and dacarbazine.
- The BEACOPP regimen includes the drugs bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone.
- Stanford V, another chemotherapy regimen used in Hodgkin lymphoma patients, uses mechlorethamine, doxorubicin, vinblastine, vincristine, bleomycin, etoposide and prednisone drugs. This combination of chemotherapy and drugs is preferred in advanced lymphoma cases.
There are various chemotherapy agents used for non-Hodgkin lymphoma as well as for Hodgkin lymphoma. When choosing these drugs, which are examined in various categories, the stage and type of the disease are taken into consideration.
- Alkylating agents destroy DNA, the hereditary material of constantly dividing cells. An important side effect of these drugs is that they increase the risk of developing leukemia.
- Corticosteroids inhibit the growth of cancer cells and are effective in controlling the symptom of nausea.
- Platinum-containing drugs act with a similar mechanism to alkylating agents, but there is no increase in the risk of developing leukemia after the use of these drugs.
- Purine analogs, another class of drugs used in non-Hodgkin lymphoma, slow down the metabolism of cancer cells and prevent their proliferation.
- Antimetabolite drugs have the feature of preventing the growth of cancer cells by replacing their DNA and RNA.
In patients with aggressive non-Hodgkin lymphoma, chemotherapeutic drugs administered as a combination therapy are called the R-CHOP regimen. Rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone are drugs included in this regimen.
Patients are treated with radiotherapy along with chemotherapy. The drugs used in chemotherapy can lower the patient's immune cells and blood cells. In this case, it may be necessary to apply supportive treatments such as blood transfusion to the patient.
Another treatment method used in the treatment of lymphoma, apart from chemotherapy and radiotherapy, is immunotherapy. In immunotherapy, antibodies produced in the laboratory and injected into the body through the vein aim to determine the location of cancerous cells and destroy them or prevent their development. Along with immunotherapy, nausea and vomiting, which are among the side effects of chemotherapy, can also be reduced.
Immunotherapy, which is considered within the scope of targeted therapy, directly targets cancer cells. Immune modulatory drugs, monoclonal antibodies, proteasome inhibitors, and small molecule therapies are among the immunotherapy drug classes used in non-Hodgkin's patients.
In case of recurrence of lymphoma, bone marrow and stem cell transplantation is also frequently applied. In case of recurrence of the disease, high-dose chemotherapy should be administered. Since this will damage the bone marrow, the bone marrow taken from the patient before chemotherapy is transplanted back to the patient after chemotherapy. In patients with bone marrow involvement, bone marrow transplantation can also be performed from family members.
Are there any side effects of lymphoma treatment?
Side effects that may occur after lymphoma treatment may vary depending on the chemotherapy drug used, radiation or surgical interventions. Chemotherapy drugs used in treatment planning generally suppress the bone marrow and this results in a decrease in the number of various blood cells. Multiple chemotherapy treatments can increase nausea and vomiting in patients. In order to prevent this situation, patients may be prescribed anti-nausea serotonin receptor antagonists or benzodiazepine-derived drugs. Doxorubicin is a drug with a predominant heart-related side effects and may cause side effects defined as cardiotoxicity. Chemotherapy drug with vincristine active ingredient is a drug that has toxic effects on nervous tissue.
As with chemotherapy drugs, some side effects may occur in lymphoma patients after radiotherapy applications. One of the most important side effects of radiotherapy is that it can cause fibrosis (connective tissue increase) in the inner tissue of the heart, causing the patient to enter the picture of heart failure. Hypothyroidism may occur as a side effect in patients receiving radiotherapy from the neck and mediastinum (middle part of the chest).
At the same time, loss of function in the tissues of the reproductive system of patients may occur after radiotherapy and cytotoxic (cell-killing) chemotherapy applications. Freezing of reproductive cells may be an important option for people who want a child after treatment, in order to use them in the future in patients undergoing treatment where this condition may develop.
Secondary cancers that occur after treatment for Hodgkin lymphoma are an important side effect of treatment in these patients. The most common cancer that develops after treatment in patients with this type of lymph cancer is lung cancer. Apart from lung cancer, breast, various soft tissue sarcomas, pancreatic cancer and thyroid cancer are among the cancer types that may occur secondary to these patients after Hodgkin lymphoma treatment.
The most common symptom in patients with successful lymphoma treatment is fatigue, which lasts for a long time. This condition is detected in 3 out of 2 patients diagnosed and treated with non hodgkin lymphoma. Fatigue usually tends to regress within 1 year after the end of treatment, but in some patients this may persist for longer periods.
The following symptoms may occur during and after lymphoma treatment:
- Low white blood cells (white blood cells, white blood cells)
- Low red blood cells (red blood cells) and related anemia
- sores in the mouth
- Nausea, vomiting, diarrhea
- Constipation
- Problems in the urinary bladder
- bloody piss
- Extreme tiredness and weakness
- Fire
- Cough
- Hair loss
- Lung, heart and nervous system problems
If you or your relatives have swelling in the lymph nodes, prolonged fatigue and any of the other findings we mentioned in the symptoms section, you should definitely get an expert opinion.
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