Lymphoma - What Is It

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Lymphoma - What Is It
Lymphoma - What Is It

Video: Lymphoma - What Is It

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Video: Lymphoma: What is lymphoma? | Norton Cancer Institute 2023, January
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Lymphoma: what is it?

Lymphoma in the strict sense means "swelling of the lymph nodes". This can be a harmless infection or inflammatory process or a malignant disease. In everyday parlance, the term lymphoma is mainly used for the latter: It is understood to be a malignant disease of the lymphatic system.

Lymphomas are caused by abnormally changed lymphocytes, a form of white blood cells. They can settle in different parts of the body and cause discomfort. Lymphomas are not uniform clinical pictures; there are around 100 different subtypes, some with very different courses.

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Lymphomas are rare compared to other cancers (such as breast cancer, colon cancer or lung cancer). They can occur at any age, but are mainly found in (older) adulthood. In children, the disease is sometimes different, and the treatment options also differ; the information provided relates to adult lymphomas only.

What is the lymphatic system?

The lymphatic system (lymphatic system) is part of the body's own defense system. It consists of the lymphatics as well as organs that contain lymphoid tissue. These include the lymph nodes, and lymphatic tissue can also be found in the bone marrow, the spleen, the gastrointestinal tract and the throat.

The lymph vessels run parallel to the blood vessels throughout the body. They take up the so-called lymph from the spaces between cells and transport waste products of metabolism as well as foreign bodies, pathogens and dead cells to the venous system.

In this way, the lymph also flows through the lymph nodes. There harmful substances are filtered out and combated. This task is carried out by so-called lymphocytes, a subgroup of white blood cells.

Lymphocytes

Lymphocytes - like all blood cells - are largely formed in the bone marrow. Through complex steps of division and maturation, different types of blood cells, including lymphocytes, are created from a common stem cell. They develop into B cells, T cells, and natural killer cells. The mature lymphocytes accumulate in lymphatic tissue and have the task of fighting pathogens, forming antibodies and setting immune reactions in motion.

All blood cells have a limited lifespan and must be constantly replicated. Normally there is a natural balance, which means that as many new blood cells are produced as are needed. The processes of blood formation are monitored and controlled by various complex control circuits.

How does a lymphoma develop?

In the case of lymphoma, these control loops are disturbed: The formation of the lymphocytes takes place in an uncontrolled manner, the maturation processes are not (fully) carried out. There is an excess of pathological blood cells that cannot fulfill their actual function.

Depending on which maturation step of the lymphocytes is interrupted, different pathological cells develop. They accumulate in the lymph nodes and other lymphatic tissue. At the same time, insufficiently mature, functional blood cells are formed, which leads to the various symptoms of the disease.

The cause of the uncontrolled cell division are changes in the DNA (mutations). How these mutations come about is not fully understood. It is assumed that genetic factors, increasing age, a permanent weakening of the immune system, chronic infections, smoking or certain autoimmune diseases can be risk factors. In many cases, however, none of these circumstances can be detected and the mutations occur randomly in the course of life.

What types of lymphoma are there?

All lymphomas start from altered lymphocytes. The division into different forms of lymphoma is based, among other things, on the cell type, on special gene changes and on molecular biological properties and surface features of the cells. Today around 100 different types of lymphoma are known, some of which are associated with very different disease courses.

One differentiates roughly:

  • Hodgkin lymphoma (Hodgkin's disease, lymphogranulomatosis): This type of lymphoma arises from progenitor cells of the B lymphocytes; they make up about ten percent of all lymphomas. Under the microscope, characteristic giant cells with several cell nuclei (Sternberg-Reed giant cells) are found for the disease. Hodgkin's lymphoma can occur at any age, the peak of the disease is around the age of 30 and 70. Two to four people per 100,000 inhabitants fall ill every year.
  • Non-Hodgkin lymphomas: As the name suggests, this includes all lymphomas that do not have the typical characteristics of Hodgkin lymphomas. This group of lymphomas includes a large number of diseases, some of which are very different. They occur more frequently at an older age (peak around the age of 70); there are five to ten new cases per 100,000 inhabitants per year. Non-Hodgkin lymphomas are roughly divided into:

    • B-cell lymphomas: They develop from preliminary stages of B-lymphocytes and make up around 80 percent of all non-Hodgkin lymphomas. This group includes multiple myeloma and CLL (see below).
    • T-cell lymphomas develop from precursors of T-lymphocytes.

The individual non-Hodgkin lymphomas sometimes differ significantly in their clinical course. Some lymphomas are slow to grow and are called indolent or low-grade; they make up around 70 percent of all non-Hodgkin lymphomas. 30 percent of non-Hodgkin lymphomas show rapid growth (aggressive lymphomas) or even very rapid growth (very aggressive forms).

Examples of non-Hodgkin lymphomas

Diffuse large B-cell lymphoma (DLBCL)

Diffuse large-cell B-cell lymphoma is the most common of all non-Hodgkin lymphomas and is one of the aggressive forms. Pathologically altered lymphocytes accumulate in a lymph node and multiply unchecked. As with many other forms of lymphoma, the symptoms are initially rather unspecific (lymph node swelling, fatigue, susceptibility to infection, etc.). Within a relatively short time, the diseased cells can spread through the body via the lymph and blood vessels, and the state of health deteriorates. Without appropriate treatment, diffuse large-cell B-cell lymphoma can quickly become life-threatening.

Follicular lymphoma

Follicular lymphoma is the most common low-grade lymphoma and accounts for around 25 percent of all non-Hodgkin lymphomas. It belongs to the group of B-cell lymphomas, the mean age of onset is between 60 and 65 years. Usually the only symptom is a lymph node swelling, which progresses slowly and does not cause any further symptoms for a long time. At a very advanced stage, the course of the disease can take on an aggressive form and lead to a rapid deterioration in health.

Chronic lymphatic leukemia (CLL)

Despite the term leukemia, CLL is counted as a lymphoma because the disease is associated with an excessively high number of lymphocytes (B cells). On the one hand, these are found in the blood (like in leukemia), but they also settle in the lymph nodes.

CLL is one of the most common forms of lymphoma in adults (20 percent of all non-Hodgkin lymphomas). It occurs from the age of 50. The first signs are often swelling of the lymph nodes and an enlarged spleen and liver. The disease can persist for years without causing any major symptoms. The diagnosis is made by chance in many cases. CLL is one of the low-malignant forms of lymphoma.

Multiple myeloma / plasmacytoma

Multiple myeloma is also a low-grade non-Hodgkin lymphoma. Overall, it is rare, but is one of the most common cancers of the blood-forming and lymphatic systems.

Multiple myeloma does not occur in the lymph nodes, but mainly in the bones. It develops from abnormal plasma cells (a subtype of B cells). These accumulate in the bone marrow, the place where blood is formed, and produce defective antibodies or antibody fragments. The plasma cell clusters are typically found in multiple locations in the bone marrow. A single tumor made up of plasma cells, on the other hand, is called a plasmacytoma.

In addition to the possible general symptoms of lymphoma, multiple myeloma and plasmacytoma often cause bone pain (e.g. in the spine, pelvic bones and ribs) and a weakening of the bone structure with an increased tendency to break (osteoporosis). The level of calcium in the blood is increased due to the breakdown of bones. The excess of antibodies can also lead to circulatory disorders and kidney failure.

Skin lymphomas (cutaneous T-cell lymphomas)

Some lymphomas develop predominantly in the skin and are called skin lymphomas or cutaneous lymphomas. The diseased cells settle in the skin and initially remain limited to it. As they progress, they can spread to the lymph nodes and further into the body.

There are different forms of skin lymphoma (B-cell and T-cell lymphoma, low malignant and aggressive forms, etc.), which can differ in terms of symptoms and course. A typical feature of skin lymphomas in general are changes in the skin that do not occur in other forms of lymphoma: they can range from reddening to flaking or crusting to dark, nodular changes. Itching is also common.

MALT lymphomas

Lymphomas can also develop in the lymphoid tissue of the mucous membranes; they are then referred to as MALT lymphomas (mucosa-associated lymphoid tissue). This form of lymphoma is most commonly found in the stomach, but it can also occur in the salivary glands, intestines, eyes or lungs.

MALT lymphomas usually grow slowly and hardly cause any symptoms. Possible symptoms of an attack on the gastric mucosa include stomach pain, heartburn, a feeling of fullness and weight loss. With regard to the development, a connection with Helicobacter pylori infections is known. Overall, MALT lymphomas are rare; they only make up around seven percent of all lymphoma diseases.

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