Hodgin Lymphoma

21.03.2024

HODGKIN LYMPHOMA DIAGNOSIS AND TREATMENT

Hodgkin Lymphoma is a type of cancer of the lymph system and specifically affects the lymph nodes. This type of cancer is characterized by the uncontrolled proliferation of abnormal lymphocytes (a type of white blood cell), leading to swelling in the lymph nodes. Hodgkin Lymphoma generally responds well to treatment, and many patients can remain disease-free for long periods of time after treatment. When diagnosed early, a high rate of recovery can be achieved with modern treatment methods. The recognition and treatment of this type of cancer is considered a significant achievement in cancer research, as more effective treatment strategies have been developed and patients' quality of life has been significantly improved. Various methods are used to treat Hodgkin Lymphoma, such as radiotherapy, chemotherapy and sometimes surgery. When detected in the early stages, treatment is often simpler and more effective.

WHAT ARE THE RISK FACTORS?

There are some risk factors that contribute to the development of Hodgkin Lymphoma, but the presence of these factors does not mean that the disease will definitely develop.

Age and Gender: Hodgkin Lymphoma is generally more common in young adults (especially those in their 20s and 30s) and older adults (over 55). Also, it is slightly more common in men than women.

Family History: The incidence of Hodgkin Lymphoma in the family is not high, but if one of the family members has this disease, the risk may increase slightly.

Immune System Weakness: Diseases that weaken the immune system, such as HIV/AIDS, or medications that suppress the immune system (for example, those used after organ transplantation) may increase the risk of Hodgkin Lymphoma.

Epstein-Barr Virus (EBV) Infection: EBV is the virus that causes mononucleosis. Having an EBV infection may increase the risk of developing Hodgkin Lymphoma.

Socioeconomic Status: There is some evidence that individuals with higher socioeconomic status have a slightly higher risk of Hodgkin Lymphoma, but this relationship is not fully understood.

Geographic Factors: Hodgkin Lymphoma may be more common in some geographic areas and ethnic groups than in others.

Each of these risk factors can contribute to the development of Hodgkin Lymphoma, but none is the definitive cause of the disease. Most people do not develop Hodgkin Lymphoma despite having one or more of these risk factors. On the other hand, people with no known risk factors can also develop this disease. Therefore, it is important to consult an oncology or hematology doctor if there are symptoms or concerns.

Image 1: The presence of Reed-Sternberg cells is the typical indicator for Hodgin lymphoma.

HOW DOES IT OCCUR?

Although the mechanisms of development of Hodgkin Lymphoma are complex and not fully understood, the basis of this disease is the abnormal and uncontrolled proliferation of normal lymphocytes (white blood cells that are part of the immune system). Hodgkin Lymphoma is characterized by the presence of large, abnormal cells known as Reed-Sternberg cells, which are specifically an abnormal form of B-type lymphocytes. These abnormal cells can be triggered by factors such as genetic mutations and environmental influences. These mutations disrupt the normal growth and death cycles of cells, causing them to proliferate uncontrollably. Additionally, these abnormal cells can affect other cells of the immune system, leading to disease progression and symptoms. Epstein-Barr virus (EBV) infection is also thought to play an important role in some cases of Hodgkin Lymphoma. However, much is still unknown about exactly why Hodgkin Lymphoma develops in some people and not in others. It is an active research topic for researchers in cancer biology and immunology.

WHAT ARE THE SYMPTOMS?

Signs and symptoms of Hodgkin Lymphoma often vary depending on the location and extent of spread of the disease.

The most common symptoms may include:

Lymph Node Swelling: Painless lymph node swelling in the neck, armpit or groin area are the most typical symptoms. These swellings are usually hard and mobile.

Fever and Sweating: Especially at night, intense sweating and fever attacks of unknown cause may occur.

Weight Loss: Unexplained and significant weight loss is another important symptom of Hodgkin Lymphoma.

Fatigue and Weakness: Constant fatigue and a general feeling of malaise may be a result of the body's fight against cancer.

Itching: A widespread and unexplained itching throughout the body may be a sign of Hodgkin Lymphoma, especially when accompanied by other symptoms.

Cough or Shortness of Breath: Enlargement of lymph nodes in the chest area can cause breathing problems such as cough or shortness of breath.

Pain After Alcohol: Although it is a rare finding, some patients experience pain in their lymph nodes after drinking alcohol.

Since these symptoms can also be seen in many other health conditions, a medical evaluation and necessary tests must be performed to diagnose Hodgkin Lymphoma. If you are experiencing any of these symptoms, it is important to see a physician.

HOW IS IT DIAGNOSED?

Diagnosis of Hodgkin Lymphoma typically involves a series of steps. First, the patient's medical history and physical examination are performed, where symptoms such as swollen lymph nodes, fever, night sweats and weight loss are examined. A lymph node biopsy is usually required to confirm the diagnosis; Here, a tissue sample is taken from the abnormal lymph node and examined under a microscope. The presence of Reed-Sternberg cells is a typical sign of Hodgkin Lymphoma. Additionally, imaging tests such as blood tests, chest X-ray, computed tomography (CT) scan, positron emission tomography (PET) scan, and sometimes bone marrow biopsy are performed to evaluate the spread of the disease. These tests help determine the stage of the disease and create the most appropriate treatment plan. The diagnosis of Hodgkin Lymphoma is made through a comprehensive combination of these tests and evaluations and should always be carried out by an experienced hematologist or oncologist.

WHAT ARE THE PATHOLOGICAL TYPES?

Hodgkin Lymphoma is basically classified into two main pathological types: Classical Hodgkin Lymphoma (CHL) and Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL). Both types have distinct morphological and biological characteristics and may require different treatment approaches.

Classic Hodgkin Lymphoma: This type accounts for the majority of Hodgkin Lymphoma cases. CHL is divided into four subtypes:

Nodular Sclerosis Type: It is the most common subtype and occurs especially in young adults. Its characteristic feature is the presence of bands of scar tissue called nodular sclerosis.

Mixed Cell Type: This type is characterized by the presence of various types of lymphoid cells and can be seen in all age groups.

Lymphocyte-Rich Classical Hodgkin Lymphoma: This subtype contains more normal lymphocytes compared to other types of CHL.

Lymphocyte Poor Hodgkin Lymphoma: The most aggressive subtype, less common and associated with a worse prognosis.

Nodular Lymphocyte Predominant Hodgkin Lymphoma: This type, which accounts for a small percentage of Hodgkin Lymphoma cases, generally has a better prognosis. NLPHL is characterized by nodules containing large, atypical lymphocytes (also known as L&H cells or "popcorn" cells). This type usually progresses more slowly and may require different treatment approaches compared to CHL.

Both major types require careful evaluation of pathological and clinical findings in diagnosis and treatment planning. Treatment is customized according to the stage of the disease, the patient's general health condition and subtype. Therefore, the role of experienced hematology/oncology specialists in the correct diagnosis and treatment of Hodgkin Lymphoma is vital. Some critical receptors and molecular markers are important in the diagnosis and treatment process of Hodgkin Lymphoma. These molecular markers play a key role in accurately identifying the disease and evaluating potential treatment options. Hodgkin Lymphoma cells are usually CD30 positive, which is an important indicator in diagnosis and evaluation of CD30-directed therapies. Additionally, most classical Hodgkin Lymphoma cells are CD15 positive, which is used in diagnosis. PAX5 is a factor involved in the development of B cells and is frequently positive in Hodgkin Lymphoma cells. Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) cells are usually CD20 positive, which can be used as a target on B cells. In cases associated with Epstein-Barr Virus (EBV), EBV-related markers (such as EBNA-1, LMP1) may be helpful in understanding the subtype of the disease. Additionally, Ki-67 is a marker that shows the proliferation rate of cells and can be found at high levels in Hodgkin Lymphoma cells, which provides information about the aggressiveness of the disease.

Image 2: In lymphoma, symptoms such as swelling in the lymph nodes, night sweats and fever may be observed.

HOW IS TUMOR STAGING DONE?

Tumor staging of Hodgkin Lymphoma is an evaluation process to determine how far the disease has spread in the body. This staging is important in determining the treatment plan of the disease and predicting its prognosis. The Ann Arbor Staging System is generally used for Hodgkin Lymphoma. This system divides the disease into four main stages according to the location of the disease, its spread, and the presence of certain symptoms:

Stage I: Lymphoma is confined to a single lymph node region of the body (I) or a single extra-lymphatic organ or region (IE).

Stage II: Lymphoma has spread to two or more lymph node regions and this spread is located on the same side of the diaphragm (II); or has spread to one or more extralymphatic organs or areas and at least one lymph node area (IIE).

Stage III: Lymphoma has spread to the lymph node areas on both sides of the diaphragm. This may have spread to extra-lymphatic organs or areas (IIIE), the spleen (IIIS), or both (IIIES).

Stage IV: Lymphoma has spread to one or more extra-lymphatic organs. This condition can involve distant organs such as the bone marrow, liver, lungs or brain and is the most advanced stage.

Moreover, the disease is classified as "A" or "B" according to the presence of symptoms:

A: There are no specific symptoms such as fever, night sweats, or significant weight loss.

B: The patient has certain systemic symptoms, such as fever, night sweats, or loss of more than 10% of body weight in the last six months.

This staging is based on the results of various medical tests, such as a physical exam, biopsy, blood tests, and imaging studies (PET scan, CT scan, etc.). Staging results play a critical role in determining treatment options for Hodgkin Lymphoma and evaluating the patient's prognosis. Therefore, it is important to have a comprehensive evaluation by experienced medical professionals for accurate staging.

HOW IS TREATMENT DONE ACCORDING TO STAGES?

Hodgkin Lymphoma treatment varies depending on the stage of the disease, the patient's general health, age, and other individual factors.

General treatment approaches according to the stages of Hodgkin Lymphoma:

Stage I and II (Early Stage): Early-stage Hodgkin Lymphoma is usually treated with radiotherapy, chemotherapy, or a combination of both. Standard chemotherapy regimens usually include the ABVD (adriamycin, bleomycin, vinblastine, dacarbazine) protocol. In some cases, less intense chemotherapy regimens or radiotherapy alone may be preferred. In early stage disease, treatment is generally quite effective and provides high rates of recovery.

Stages III and IV (Advanced Stage): Advanced stage Hodgkin Lymphoma requires more intense chemotherapy. Chemotherapy combinations such as the ABVD protocol or the more intensive BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone) regimen may be used. In advanced stages, treatment usually aims to control the disease and achieve remission. In some cases, high-dose chemotherapy and autologous stem cell transplantation may also be included in the treatment plan.

Relapsed or Refractory (Treatment-Resistant) Hodgkin Lymphoma: In cases of relapsed or treatment-resistant Hodgkin Lymphoma, different approaches than standard chemotherapy may be required. In this case, treatment options such as high-dose chemotherapy and autologous stem cell transplantation, next-generation targeted therapies (e.g., brentuximab vedotin), and immunotherapy agents (e.g., PD-1 inhibitors) may be considered.

During treatment, it is important to monitor patients' general health, manage side effects, and provide supportive care. Hodgkin Lymphoma treatment should be customized for each patient and managed by experienced oncology and hematology specialists. The treatment plan is personalized according to the characteristics of the disease, the patient's preferences and lifestyle. Regular medical follow-up and evaluations during the treatment process are an important part of treatment success.

Image 3: Hodgin lymphoma treatment may vary depending on the stage of the disease.

WHAT ARE THE SYSTEMIC DRUGS USED IN TREATMENT?

Various chemotherapy regimens, targeted (smart) drugs, immunotherapies and sometimes hormonal drugs are used in the treatment of Hodgkin Lymphoma. Here are some of these treatment options:

Chemotherapy Regimens

ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine): One of the most commonly used chemotherapy combinations for Hodgkin Lymphoma.

BEACOPP (Bleomycin, Etoposide, Doxorubicin, Cyclophosphamide, Vincristine, Procarbazine, Prednisone): It is a more aggressive treatment protocol and is used especially for advanced stage Hodgkin Lymphoma.

Stanford V: It is another treatment protocol that includes a combination of different medications.

Targeted (Smart) Drugs

Brentuximab Vedotin: It is an antibody-drug conjugate targeted to CD30 positive Hodgkin Lymphoma cells.

Immunotherapies

Checkpoint Inhibitors (Pembrolizumab, Nivolumab): As mentioned above, these drugs activate the immune system, helping it fight against cancer cells.

Supportive Treatments

G-CSF (Granulocyte Colony Stimulating Factor): Used to increase the number of white blood cells after chemotherapy.

These treatments are personalized according to the characteristics of the disease, the patient's general health condition and response to treatment. Treatment options and combinations are constantly evolving and should always be implemented in accordance with the most current medical guidelines and research. It is important that patients be evaluated by experienced oncology or hematology specialists before any treatment plan is initiated.

HOW SHOULD FOLLOW-UP BE CARRIED OUT AFTER RECOVERY?

Hodgkin lymphoma is a type of cancer that occurs in the lymph system. Post-recovery follow-up is vital for people struggling with this disease. Follow-up after recovery is usually done with regular doctor visits, blood tests, and imaging tests. During these checks, doctors evaluate the patient's general health condition and perform detailed examinations to ensure that lymphoma symptoms do not reoccur. In addition, the long-term side effects of the drugs used during treatment are also evaluated during these checks. It is also important for patients to exercise regularly, follow a healthy diet, and receive psychological support if necessary during the recovery process. In this process, communication between the patient and the doctor ensures that the patient is constantly informed about his health status and that any problems are detected and intervened early.