CONTENTS

What Is Lymphoma?

Lymphoma is a type of cancer that develops in the lymphatic system, which is part of the immune system that protects our body against infections and external agents. It is also commonly known as “lymph cancer.” The lymphatic system consists of colorless lymph fluid carrying B, T and natural killer (NK) lymphocytes circulating through lymphatic vessels; lymphatic vessels, which form a network of thin tubes that collect lymph fluid from different parts of the body and return it to the bloodstream; and lymph nodes (lymph glands), which are located in groups along the lymphatic network in areas such as the neck, armpits and mediastinum (chest cavity).

Lymph nodes are small, bean-shaped structures that filter lymph and store lymphocytes. The spleen, an organ that produces lymphocytes, stores red blood cells and lymphocytes, filters blood and destroys blood cells; the thymus, where T lymphocytes mature and proliferate; the tonsils; and the bone marrow, which is the production site for all blood cells, are also parts of this system.

Lymphatic tissue is also found in other parts of the body, including the stomach and intestines, thyroid gland, brain and skin.

Incidence and Causes of Lymphoma

Lymphomas rank third among childhood cancers after leukemia and central nervous system tumors and are encountered more frequently in developing countries such as Türkiye. Lymphomas account for approximately 15% of cancers in children.

Under normal conditions, the production, proliferation and lifespan of lymphocytes are controlled by the body. However, various genetic diseases—particularly certain immunodeficiency disorders—immunosuppressive medications or infections (EBV, HIV, HTLV), as well as changes associated with previous organ transplantation for any reason, may cause lymphocytes to proliferate uncontrollably and disrupt their normal life cycle.

Lymphoma may develop as a result of this process. Because lymphocytes are widely distributed throughout the body, the disease is not limited to lymph nodes in the neck, armpits or groin. The stomach and intestinal system, spleen, bone marrow, skin and other organs may also be affected by lymphoma.

What Are the Types of Lymphoma?

Lymphomas are a broad group of diseases that includes many different types. The type of lymphoma is determined by how the cells appear under a microscope. In the basic classification, lymphomas are divided into two main groups: Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Both groups are further divided into different subtypes.

Hodgkin Lymphoma

Hodgkin lymphoma is a type of lymphoma defined by certain characteristic cellular features. HL can occur in different age groups, from childhood and young adulthood to older age, but it is generally seen in adolescents over the age of 10.

HL is also divided into two subtypes: “nodular lymphocyte-predominant type” (NLPHL) and “classical” HL. NLPHL, which is more common in young males and is generally localized to a particular region, accounts for 10–15% of all HL cases. The classical type is divided into four subtypes: lymphocyte-rich, nodular sclerosis, mixed cellularity and lymphocyte-depleted. Nodular sclerosis is seen more often in adolescents and children with higher socioeconomic status, while mixed cellularity is more common in countries with lower socioeconomic status. The lymphocyte-depleted type is frequently seen in HIV-positive patients.

Non-Hodgkin Lymphoma

The three main types of childhood non-Hodgkin lymphoma are:

1. Aggressive Mature B-Cell NHL

Burkitt lymphoma, which develops from B lymphocytes, is a rapidly growing lymphoma most commonly seen in children, adolescents and young adults. It is associated with Epstein-Barr virus (EBV) infection and may develop in the abdomen, Waldeyer’s ring, testes, bone, bone marrow, skin or central nervous system.
Diffuse large B-cell lymphoma, the most common type, grows rapidly in the lymph nodes and frequently affects the spleen, liver, bone marrow or other organs. It is more common in adolescents than in children.
Primary mediastinal B-cell lymphoma, which is seen more often in adolescents than in children.

2. Lymphoblastic Lymphoma

Lymphoblastic lymphoma is a type of lymphoma that affects T-cell lymphocytes and closely resembles acute lymphoblastic leukemia. It usually develops in the mediastinum. This may cause shortness of breath, wheezing, difficulty swallowing, or swelling of the head and neck. It may spread to the lymph nodes, bone, bone marrow, skin, central nervous system, abdominal organs and other areas.

3. Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphoma is a type of lymphoma that affects T cells and usually develops in the lymph nodes, skin or bone, although it may sometimes occur in the gastrointestinal system, lungs and muscles. In patients with anaplastic large cell lymphoma, a receptor called CD30 is present on the surface of T cells, which is important for targeted therapy.

While some non-Hodgkin lymphomas may progress slowly, others may progress rapidly and require treatment within a short period of time. Therefore, a diagnosis of “non-Hodgkin lymphoma” alone is not sufficient to determine the treatment plan. Identifying the exact subtype of the disease is of great importance.

Symptoms of Lymphoma

Symptoms of lymphoma may vary depending on the type of disease, its location and extent. One of the most common findings is painless enlargement of the lymph nodes, particularly in the neck, armpit or groin. The presence of these symptoms does not necessarily mean that a person has lymphoma. Many different conditions, especially infections, can cause similar complaints. However, lymph node swelling that persists for a long time, increases in size or cannot be explained should be evaluated by a physician.

In addition, B symptoms such as unexplained and recurrent fever, intense night sweats and unintentional weight loss are seen particularly in HL. Persistent or marked fatigue, itching of the skin and loss of appetite may also occur.

A mass in the jaw or abdominal swelling and pain, together with intussusception (intestinal obstruction), which may occur in preschool-age children, may suggest Burkitt lymphoma. Symptoms such as cough, shortness of breath or a feeling of pressure in the chest should more strongly suggest mediastinal involvement.

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Diagnosis of Lymphoma

In patients with suspected lymphoma, the diagnostic process begins with a detailed medical history and physical examination. The characteristics of the lymph nodes, the patient’s complaints, the duration of symptoms and other accompanying findings should be evaluated.

Blood tests and imaging methods such as ultrasonography, magnetic resonance imaging (MRI), computed tomography (CT) or PET/CT may be used during the diagnostic process.

However, biopsy and pathological examination are generally of fundamental importance for the definitive diagnosis of lymphoma. A sample taken from an appropriate lymph node or affected tissue is examined in the pathology laboratory, and immunohistochemical and, when necessary, molecular/genetic examinations are performed to determine the lymphoma subtype.

Identifying the correct subtype is one of the most important stages in developing the treatment plan.

Treatment of Lymphoma

The treatment plan is developed by evaluating many factors together, including the type and subtype of lymphoma, the stage of the disease, the patient’s age, general health status, accompanying diseases and previously administered treatments. Hodgkin lymphoma is currently among the cancers that can respond very well to treatment, with response rates of 95–100%. In non-Hodgkin lymphoma, treatment response rates have now reached 85–90%. Nevertheless, the stage of the disease, the patient’s age, general health status, extent of the disease and certain biological characteristics are taken into account when planning treatment. Chemotherapy, radiotherapy when necessary, and different systemic treatment options may be used in suitable patients. In a patient with Burkitt lymphoma presenting with an acute abdominal attack, surgery should be considered first.

Chemotherapy

Chemotherapy is one of the main methods used in the treatment of many types of lymphoma. Treatment protocols may involve a single medication or combinations of different medications. The content and duration of treatment are determined according to the lymphoma subtype and the patient’s characteristics.

Targeted Therapies

Treatments aimed at enabling the immune system to recognize cancer cells or generate a more effective response against them have an important role in some types of lymphoma. Monoclonal antibodies exert their effects by binding to specific targets on the surface of lymphoma cells. In patients with relapsed HL or HL that has not responded adequately to standard treatments, anti-CD30 therapy (brentuximab), and in patients with CD20-positive NHL, targeted drugs such as rituximab may be used alone or in combination with chemotherapy.

Stem Cell Transplantation

Stem cell transplantation may be considered particularly in some lymphomas that relapse or do not respond adequately to standard treatments. Autologous stem cell transplantation, in which the patient’s own stem cells are used, and allogeneic stem cell transplantation, in which stem cells from a tissue-compatible donor are used, are different treatment approaches. The physician decides which method is appropriate by considering the type of disease, response to treatment and the patient’s general condition.

Cellular Therapies

One of the important developments in the treatment of hematological malignancies in recent years is CAR-T cell therapy. In this approach, the patient’s immune system cells are modified in a laboratory so that they can recognize and target specific cancer cells and are then given back to the patient. CAR-T cell therapy is not suitable for every patient with lymphoma; it is one of the advanced treatment options that can be applied in certain types of lymphoma and under specific clinical conditions.

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