On the occasion of World Lymphoma Day, Dr. Alcaraz, a specialist in Hematology in Murcia and member of Top Doctors, details the keys to understanding what lymphoma is, what symptoms it causes and what treatment options currently exist to combat it.
Lymphoma is a cancer that begins in the cells of the lymphatic system. The lymphatic system is part of the immune system, which helps the body fight infection and disease. Because lymphatic tissue is found throughout the body, lymphoma can arise anywhere in the body. The two general types of lymphoma are Hodgkin’s lymphoma and non-Hodgkin’s lymphoma (NHL), which can occur in both children and adults.
Most people with Hodgkin’s lymphoma have the classical type. In this type, there are abnormal large lymphocytes (a type of immune cell) in the lymph nodes called Reed-Sternberg cells. Hodgkin’s lymphoma can be cured.
There are many different types of NHL. Most types form from B cells, but some form from T cells or naturally occurring NK lymphocytes. NHL can be either indolent (slow-growing) or aggressive (fast-growing).
Lymphoma involvement and causes
Lymphoma affects between three and six people per 100,000 population each year. The average age at which the disease usually appears is 60 years.
To date, the causes that trigger lymphoma are unknown, except in some cases in which it is associated with an infection caused by bacteria such as helicobacter pylori and borrelia or by viruses such as Epstein-Barr virus (EBV).
Lymphoma is also associated with factors such as hair dyes, insecticides, and ionizing radiation, although no conclusive evidence has been drawn.
Symptoms of lymphoma
As a general rule, lymphomas present as enlarged lymph nodes which, when they appear in accessible areas such as the neck, armpits or groin, can be palpated to show their increased size. These lumps (adenopathies) are not usually painful. When they appear in less accessible areas (abdomen, mediastinum, etc.) they can go unnoticed, so diagnosis is more difficult and is only achieved when other symptoms appear that require more exhaustive studies.
These symptoms may include fever above 38 degrees, profuse night sweats to the point of soaking clothes and unexplained weight loss. Patients may also experience other local manifestations such as peripheral lymphadenopathy or enlargement of the spleen, known as splenomegaly.
Diagnosis of lymphoma
As in any blood disease, a good clinical history of the patient is crucial, inquiring about family history or looking for factors that could trigger the disease. In the physical examination we look for the presence of enlarged lymph nodes or lymphadenopathies, most of the time not painful. Blood tests including a complete blood count, biochemical profile and serology (hepatitis and HIV) also help the physician to have an alarm sign that facilitates the diagnosis, although analytical values are almost always normal, unless the disease is very advanced with bone marrow involvement.
The definitive diagnosis is made by performing a lymph node biopsy. This test is performed by puncturing the apparently affected lymph node in order to extract and analyze a tissue sample.
Depending on the results of the biopsy and the patient’s medical history, the specialist may also indicate other diagnostic tests such as chest X-ray and/or CT of the neck, chest, abdomen and pelvis. In addition, a positron emission tomography (PET) scan or bone marrow biopsy may also be recommended. The latter help the hematology specialist to diagnose the extent of the disease, which is very important as a prognostic factor when outlining an appropriate treatment plan.
Determining the stage of the disease is key to this. The Ann-Arbor system differentiates between four different stages of the disease:
- Stage I: The lymphoma affects a single nodal territory or a single extralymphoid location.
- Stage II: Two or more lymph node territories or lymphoid structures on the same side of the diaphragm are affected.
- Stage III: The patient has involvement of lymph node territories or lymphoid structures on both sides of the diaphragm.
- Stage IV: This is the most severe stage. The patient shows disseminated involvement of one or more extralymphatic sites (liver, skin, bone marrow, etc.), with or without lymph node involvement.
Treatment for lymphoma
As mentioned above, it is very important to make an accurate diagnosis and a good study of the extent of the disease in order to have all the necessary data to decide on the most appropriate treatment. At present, knowledge of lymphomas is increasing, as well as research with new molecules and other therapeutic modalities that offer encouraging expectations.
The most commonly used treatments are:
- Alkylating agents: directly attack DNA to prevent cancer cells from reproducing.
- Rituximab: a type of monoclonal antibody indicated for de novo or relapsed B-cell non-Hodgkin’s lymphoma, chronic lymphocytic leukemia-B and multiple myeloma, among other pathologies.
- Conventional chemotherapy.
- Radio – Chemotherapy.
- Hematopoietic transplant.
- Once treatment has been completed, three scenarios can occur: – The patient is refractory: the treatment does not work and the patient must go on to rescue treatment. – There is a partial response to treatment. – The patient has a complete response to treatment.
What’s new in Hodgkin’s lymphoma research?
Specialists are working to learn more about Hodgkin’s lymphoma, how to prevent it, what the best treatment is, and how to provide the best care for people diagnosed with the disease. It is advisable to always talk to your doctor about the best diagnostic and treatment options for each case. The new features are:
- Determining the effectiveness of treatment.
- New chemotherapy/targeted therapy
- Immunotherapy
- Genetic profiling
- Reduction of treatment intensity
- Palliative care
Prognosis of Hodgkin’s lymphoma
In general terms, more than half of the cases of lymphoma are cured, although the prognosis depends on a series of factors such as the age and general condition of the patient, the stage of the pathology at the time of diagnosis and, especially, the type of lymphoma in question.
- Follicular lymphoma is incurable by therapy but progresses very slowly (it is indolent) and the average life expectancy in these patients is 16 years from the time of detection.
- Burkitt’s lymphoma is cured by chemotherapy in a large percentage of patients.
- Hodgkin’s lymphoma responds positively to treatment with chemotherapy and radiotherapy in the vast majority of cases.
- HALT lymphoma, caused by a bacterium that affects the stomach mucosa, is easily cured with antibiotics.
- Mantle lymphoma has the worst prognosis and cannot be cured unless the patient undergoes a transplant.