Non-Hodgkin Lymphoma Treatment

What is lymphoma?

Overview of the lymphatic system and lymphocytes

 

 B and T lymphocytes arise from the bone marrow (which is a primary lymphoid organ) and are initially precursors and then develop into mature lymphocytes in the same bone marrow for type B, while T cells develop in thymus.

The mature B cells then migrate to the lymph nodes (secondary lymphoid organs) where they gather as follicles in the lymph node cortex.

Mature T cells congregate in the paracortex.

 

On the surface of B lymphocytes, there are protein complexes called CD (cluster of differentiation) with different numbers that act as markers that identify the body's cells and help your body distinguish different types of cells.

B cells in the normal state express CD20 and CD45.

 

In the event that an antigen binds to the surface of the B cell, this cell activates, and some of it develops into plasma cells that secrete IgM, which are usually found in the middle of the lymph nodes in the so-called medulla, and some of them travel to the center of the lymphatic follicle and develop into a centroblast. The centroblast then divides and multiplies and forms what's called the germ center, where a rearrangement occurs in the immunoglobulin genes of these cells and they develop into centrocytes, some of which secrete IgA and others IgG.

 

The centrocytes then differentiate into either plasma cells or memory cells, and the latter circulates in the blood, where part of it remains, or settles in the spleen or in the mucosa-associated lymphoid tissue known as MALT.

While the development and differentiation of T lymphocytes begins in the thymic gland.
Mature cells express either CD4, in this case, helper T cells, or CD8, called killer or suppressor T cells.

Non-Hodgkin's lymphoma is a type of hematological neoplasm that specifically affects lymphocytes, and is sometimes abbreviated and called NHL.

It is the other more common type of lymphoma, as it is the seventh most common cancer affecting adults around the world.

The incidence of this cancer is estimated at about 72,000 cases annually, with the number expected to increase significantly in upcoming years.

 

Non-Hodgkin's Lymphoma is not a single disease as some think, but rather a group of diseases that affect lymphocytes, as it has been estimated that there are more than 86 types of Non-Hodgkin's Lymphoma.

It can be distinguished by the location of the tumor and its accompanying symptoms, but the main difference on which the diagnosis is based is the microscopic characteristics, where in Hodgkin's lymphoma there are tumor cells called Reed-Sternberg cells with a distinctive appearance, As they look like an owl's eye.

Also what helps distinguishing between the two types is that Hodgkin's Lymphoma arises mostly in the lymph nodes and is rarely seen outside these nodes, unlike non-Hodgkin's lymphoma, which is often seen outside the lymphatic system.

 

In Non-Hodgkin's Lymphoma, there is a change or mutation (for several reasons we will mention later) in the genes of lymphocytes, where these cells become not subject to programmed cell death and divide randomly, and the immune system cannot control the division of these cells, giving these random divisions Nonfunctional tumor cells.

These cells can grow in any organ of the body and symptoms vary depending on the affected organ.

As we mentioned, Non-Hodgkin's lymphoma has many types (more than 86 types), but to make understanding and classification of the disease easier, Non-Hodgkin's lymphoma cancer types have been classified in two main categories:

This lymphoma constitutes about 85% of non-Hodgkin's lymphoma cases, and it is either slow-growing indolent or fast-growing aggressive and includes different types, including:

It is the most common type of lymphoma from B-cell lymphomas, and it accounts for approximately 22% of them, and it is diagnosed in one out of every three cases with Non-Hodgkin's lymphoma.

It is characterized by being rapidly growing, and it affects the elderly more, especially those in their sixties, and usually appears as a mass in the abdomen, chest, or lymph nodes and gives metastases to multiple sites such as the liver, digestive tract, and even the brain, spinal cord and thyroid gland.

Symptoms are usually B symptoms (will be mentioned later in the Symptoms and Signs section), and may be asymptomatic at first.

 

It is highly responsive to treatment and treatable despite its rapid growth and it is usually associated with metastases.

There are many types of Diffuse Large B cell Lymphoma, and we mention only one of them due to its importance, which is Primary mediastinal diffuse B lymphoma It often appears in young women behind the sternum and can cause respiratory symptoms due to pressure on adjacent structures, or even cause swelling of the face and neck when it compresses the superior vena cava.

It is the most common indolent lymphoma, with 1 in 5 patients diagnosed with Non-Hodgkin's lymphoma, and the average age of infection is in the sixties.

The tumor arises in the lymph nodes and bone marrow, and this type of lymphoma is a chronic disease that is often incurable, as patients live for many years despite having this lymphoma.

This lymphoma can develop into diffuse large B-cell lymphoma.

They are almost the same disease, as they arise at the expense of small lymphocytes, but the difference is in the location of the tumor, where CLL arises in the blood and bone marrow, while SLL arises in the lymph nodes and spleen.

Treating them is difficult, but the patient often lives for long periods of time.

It is a rare, fast-growing (aggressive) type of non-Hodgkin's lymphoma, named after the doctor who first described it in children in sub-Saharan Africa.

It has three sub-types:

• Endemic Burkitt: As we mentioned, it is spread in children and adolescents in Africa and is often associated with infection with the Epstein-Barr virus, and it often affects their jaw and facial bones, and it can also arise in the intestines, kidneys and other organs.

Burkitt's lymphoma under the microscope looks like a starry sky at night, due to the presence of Tingible bodies, which are large macrophages that have ingested the tumor cells.

It needs immediate and intensive treatment due to its rapid spread and growth, although half of the patients recover after intensive chemotherapy.

It is a rare type of non-Hodgkin's lymphoma, arises in the mantle zone of the lymph nodes, and is often fast growing (can be indolent).

Often this lymphoma is not diagnosed until late stages and is often difficult to treat and cure.

There is an increase in the synthesis of the protein Cyclin D, which makes it easy to diagnose this lymphoma with a biopsy.

It is slow growing and often appears after the age of sixty and is divided into three sub-types:

• Extra-Nodal MZL: the most common, It arises in areas outside the lymph nodes, often in MALT, where this type can be called MALT lymphoma, and it is either gastric or Intestinal, this type appears with a medical history of chronic infection (mostly with H.Pylori).
• Nodal MZL: it arises in the lymph nodes or bone marrow.
• Splenic MZL: it arises in the spleen, blood, or bone marrow.

It usually arises in the bone marrow, lymph nodes, and spleen.

It is characterized by the secretion of immunoglobulins by tumor cells called immunoglobulins M, which accumulate in the blood at high levels. This accumulation leads to an increase in the thickness and viscosity of the blood, and this case is calledWaldenstrom Macroglobulinemia.

They account for approximately 7% of non-Hodgkin's lymphoma cases and have many types, but they are rare.

To make it easier, it is divided into two parts, depending on the degree of differentiation of the T cells from which the tumor arises:

It is rare and arises at the expense of T cells in its initial stages, and it first arises in the thymus gland and grows to large sizes that can affect breathing and cause swelling in the face and neck.

The disease is rapidly spreading and growing, but responds well to chemotherapy and is curable if detected early.

They arise at the expense of more mature T cells, a general term that includes many subtypes depending on where the tumor originates, and we will mention them briefly:

• Anaplastic Large Cell Lymphoma: ALCL can be systemic (occurring all over the body) or cutaneous (limited to the skin). Systemic ALCL is usually at an advanced stage when diagnosed and is fast-growing.
  The systemic type is further categorized into two types based on ALK (Anaplastic Lymphoma Kinase) positivity or negativity. Systemic ALCL responds well to treatment, especially ALK-positive disease, but ALK-negative patients may require more aggressive treatments, and relapse occurs more frequently than ALK-positive disease. .
• Angioimmunoblastic T-cell lymphoma: It is a rare, severe type that accounts for about seven percent of all patients with T-cell lymphoma in the United States.
  Most patients are middle-aged to elderly and are diagnosed at an advanced stage, and there is some evidence that AITL develops from a persistent immune response possibly due to an underlying viral infection (eg Epstein-Barr virus).
  Initial symptoms often include fever, night sweats, rash, itching, some autoimmune disorders such as autoimmune hemolytic anemia and immune thrombocytopenia and with elevated levels of gamma globulin in the blood.
• Cutaneous T-cell Lymphoma: It is a general term that includes several under slow-growing types, most of which are specific to the skin, and one of these types is Mycosis Fungoides, which is the most common type and is characterized by the presence of papules, plaques, and itchy skin lesions on the course of the body. Another type is Sézary syndrome, a syndrome that affects the blood and skin and appears with red skin. Severely itchy in most parts of the body and tumor T cells called Sézary cells can be seen.
• Lymphoblastic lymphoma: It is rare and arises from lymphoblasts and is more common on the expense of T-cell lymphoblasts.

There are many other types, but they are not mentioned due to the lack of their clinical importance.

The cause of non-Hodgkin's lymphoma has not yet been clearly established.

As we mentioned earlier, the mechanism of cancer formation through different genetic mutations in the genes of lymphocytes is agreed upon, but no real and clear reason for the emergence of these mutations has been discovered.

There are some risk factors that often have a role in the emergence of these mutations in non-Hodgkin's lymphoma patients, including:

• Age: The older the patient, the higher the incidence of Non-Hodgkin's lymphoma, as more than a third of cases were diagnosed in the elderly over 75 years.
• Gender: Where there is a slight tendency for lymphoma to occur in males more than in females.
• Race: It is believed that white people have a tendency to develop non-Hodgkin lymphoma more than people with black skin, but this belief needs more detailed studies.
• Family History: The incidence of Non-Hodgkin's lymphoma increases when there is a case among first-degree relatives (brother, father, son...).
• Obesity Recent studies have shown that obese people have an increased risk of some types of Hodgkin lymphoma.
• Hereditary Immune diseases: Such as hypogammaglobulinemia or Wiscott-Aldrich syndrome.
• History of previous exposure to radiation: Such as those who underwent previous radiotherapy or, rarely, survivors of nuclear and chemical disasters.
• Autoimmune diseases: Such as rheumatic fever, Sjögren's disease, systemic lupus erythematosus (SLE) or celiac disease have all been linked to non-Hodgkin's lymphoma, and AIDS, caused by HIV, may also have a role in this cancer.
• EBV and Human T-lymphotrophic Virus type I: It has been proven that they have a significant role in the occurrence of genetic mutations in patients with non-Hodgkin lymphoma.
• Chronic infections: Such as infection caused by H. pylori, hepatitis virus and Chlamydia psittaci.

Symptoms and signs of non-Hodgkin lymphoma vary greatly, due to the presence of many types and the possibility of cancer arising in almost any organ of the body.
Lymphoma may not have any symptoms until the tumor has grown to a certain size.

The most common presentation in patients with non-Hodgkin's lymphoma is Painless swelling of the lymph nodes Especially in the cervical, axillary and groin lymph nodes.

Other symptoms include:

• Profuse night sweats
• Unexplained weight loss
• Rashes or lumps with itchy skin all over the body
• Unexplained fever
• Constant feeling of tiredness and fatigue
• Increased possibility of bleeding, such as nosebleeds and heavy menstruation
• Breathlessness
• Feeling full after eating a small amount of food
• Frequent infections

Symptoms (night sweats, weight loss, fever) are called B symptoms.

The appearance of some other symptoms of lymphoma depends on the place of metastases from non-Hodgkin's lymphoma. The symptoms are often mechanical. Intestinal obstruction occurs if there is metastases to the digestive system, or a lack of blood cell formation occurs in the case of bone metastases. It may also cause compression of the spinal cord if there is a metastasis next to it.

The Diagnostic and investigative methods has evolved significantly in Turkey, especially in the field of oncology.

Non-Hodgkin's lymphoma patients often present with an enlarged lymph node history and sometimes some other symptoms, but the lymph nodes enlarge for other reasons that are more common than Non-Hodgkin's Lymphoma and the most common cause of Lymph nodes swelling is Infection.

 

Diagnosis depends mainly on the doctor's skill and experience, and many costly procedures can be dispensed with if the diagnosis is directed in the right direction.

First, the clinical history must be taken in detail, and then a clinical examination of the patient is conducted, and then the doctor proceeds to other, more specific procedures.

To help diagnose Non-Hodgkin's Lymphoma, the following may be done:

• Medical-Based Imaging: Such as ultrasound, CT, positron emission tomography (PET-Scan), simple chest radiograph, and magnetic resonance imaging (MRI).
• Blood tests: Such as a complete blood count (CBC), and blood chemical tests, as well as titration of certain enzymes in case of suspicion of a particular type of non-Hodgkin's lymphoma.
• Bone Scan.
• biopsy: It is the most important and most accurate diagnostic method, but it is not requested until after other causes and their types are denied, and it includes bone marrow biopsy.

If you have any problem or question then Bimaristan medical center will answer all your questions, feel free tocontact us,Bimaristan center is your family in Turkey.

Classification of non-Hodgkin's lymphoma

Choosing Non-Hodgkin's lymphoma treatment depends on several factors, including the patient's age and the type and stage of non-Hodgkin's lymphoma.

It is not always necessary to resort to treatment if non-Hodgkin's lymphoma is diagnosed, as it can be monitored if there are no symptoms or the patient's life is not affected.
Among the most common treatments in Turkey:

It is the most common treatment for non-Hodgkin's lymphoma, and Chemotherapy in Turkey has evolved significantly until it became one of the leading countries in the field of chemotherapy.
Chemo drugs target any cell that is constantly dividing in the body, including cancer cells, and as a result, they have many side effects. Cancer cells are not the only cells that renew quickly and constantly in the body (hair cells, skin, blood cells… are also constantly renewed).
Treatment is usually given in the form of drug combinations between several drugs to get a better result and includes many combinations of drugs used in Chemotherapy for non-Hodgkin's lymphoma include the monoclonal antibody rituximab (Rituxan), which is usually abbreviated with the letter R and placed at the beginning or end of the abbreviation for a co-administration, such as R-CHOP or CHOP-R consisting of cyclophosphamide, doxorubicin, vincristine, prednisone.

Intrathecal (into the spinal canal or subarachnoid space) chemotherapy may also be used to treat primary lymphomas that originate in the testicles or sinuses, diffuse large B-cell lymphoma, Burkitt's lymphoma, lymphoblastic lymphoma or some Aggressive (rapidly growing) T-cell lymphomas, given to reduce the spread of lymphoma cells to the brain and spinal cord.

An estimated 81,560 people in the United States will be diagnosed with non-Hodgkin's lymphoma in 2023, according to the National Cancer Institute.
Hodgkin's and non-Hodgkin's lymphomas can occur in children, adults, and at any age.

September is Leukemia and Lymphoma Awareness Month as it spreads awareness campaigns and urges people to get regular checkups, and shows that the disease is treatable.

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