What is Lymphocyte Depleted Hodgkin Lymphoma?
Lymphocyte-depleted classic Hodgkin lymphoma is the least common type of classic Hodgkin lymphoma found worldwide, although it might be found more often in developing countries. The term ‘lymphocyte-depleted’ means that the lymph nodes have fewer immune cells, known as lymphocytes, than usual. This variant can be hard to accurately diagnose because it might be mistaken for more aggressive forms of lymphomas, which are cancers of the lymphocytes, either B-cell or T-cell types.
Patients who have lymphocyte-depleted classic Hodgkin lymphoma often show up with more severe risk factors, and typically have lower survival rates compared to patients with other types of classic Hodgkin lymphoma. Therefore, an accurate and early diagnosis is essential to improve their chances of survival.
What Causes Lymphocyte Depleted Hodgkin Lymphoma?
Even though certain viruses like the Epstein-Barr virus (EBV) and the human immunodeficiency virus (HIV) have been connected to this disease, no definite cause-and-effect relationship has been found yet.
Risk Factors and Frequency for Lymphocyte Depleted Hodgkin Lymphoma
Lymphocyte-depleted classic Hodgkin lymphoma is a very rare type of Hodgkin lymphoma, making up only 1% to 1.5% of Hodgkin lymphoma cases in Western countries. This subtype may be more prevalent in developing countries. The disease is often diagnosed in people aged 60 years and older, though it also impacts younger individuals between 18 and 39 years old. Men are diagnosed with this disease twice as often as women. At the time of diagnosis, most patients (64%) are found to be in the advanced stages (stages III-IV) of the disease.
- Lymphocyte-depleted classic Hodgkin lymphoma is known as the least common type of Hodgkin lymphoma.
- In Western countries, about 1% to 1.5% of all Hodgkin lymphoma cases are lymphocyte-depleted classic Hodgkin lymphoma.
- This subtype might be more prevalent in developing countries.
- Approximately 40% of people diagnosed with this disease are aged 60 years and above.
- Younger people aged between 18 and 39 are also affected, making up around 28% of the cases.
- It is more common in men, who get the disease twice as often as women.
- At the time of diagnosis, about 64% of patients are in the advanced stages, III-IV.
Signs and Symptoms of Lymphocyte Depleted Hodgkin Lymphoma
People with certain symptoms, often referred to as B symptoms, may experience unexplained fever over 100.4°F (38°C), drenching night sweats, and unexplained weight loss of more than 10% in 6 months. These individuals usually have extensive symptomatic disease that tends to impact parts of their body like the retroperitoneal lymph nodes (nodes located behind the peritoneum, the lining of the abdominal cavity), organs in the abdomen including those beneath the diaphragm, and bone marrow.
Testing for Lymphocyte Depleted Hodgkin Lymphoma
To confirm a diagnosis of a type of cancer called lymphocyte-depleted classic Hodgkin lymphoma, the doctors generally prefer to take a piece of a lymph node through a procedure called an excisional lymph node biopsy. This is considered more effective than a core-needle biopsy. A less invasive method called a fine-needle aspiration biopsy, however, is not adequate for a precise diagnosis.
After this, a procedure called immunophenotyping is performed. This helps to confirm the diagnosis by checking for certain markers on the Hodgkin Reed-Sternberg (HRS) cells, a cell type commonly associated with this kind of cancer. These cells typically test positive for certain markers named CD30, CD15, and PAX5. About 20% of the cases also show positive for CD20. They usually do not have CD3, CD45, and CD79a markers. Moreover, the cells in around 60% to 72% of the lymphocyte-depleted classic Hodgkin lymphoma cases may test positive for a marker named LMP 1 of EBV (Epstein-Barr Virus).
Once the diagnosis is confirmed, it’s crucial to determine the extent or ‘stage’ of the disease to plan the most effective treatment. Usually, this involves imaging scans like Positron Emission Tomography (PET) and Computed Tomography (CT), which help doctors see the cancer’s size and location. A procedure called a bone marrow biopsy, previously used for staging, is no longer typically required for Hodgkin lymphoma.
The 2014 Lugano classification describes the disease stages as follows:
- Stage I: A single lymph node, a group of neighboring lymph nodes, or a single organ is affected, but without lymph node involvement.
- Stage II: Two or more lymph node groups on the same side of the diaphragm (a muscle that helps with breathing) are affected, or there’s limited spread to nearby organs.
- Stage II bulky: This is similar to Stage II, but with one lymph node mass being larger than 10 centimeters or more than a third of the diameter across the chest.
- Stage III: Lymph nodes on both sides of the diaphragm are affected, or lymph nodes above the diaphragm along with the spleen are affected.
- Stage IV: The cancer has spread to one or more organs or tissues that are not directly next to the affected lymph nodes.
Treatment Options for Lymphocyte Depleted Hodgkin Lymphoma
Treatments for classic Hodgkin lymphoma have been progressing, including the use of chemotherapy, radiation therapy, and other targeted treatments. The approach to treat this condition varies based on several factors like the patient’s age, how far the disease has advanced, risk factors, other health conditions, potential side effects and the patient’s quality of life after treatment.
Doctors classify patients into three groups to decide the best treatment path. These groups include early-stage favorable, early-stage unfavorable, and advanced-stage disease. Unfavorable factors for early-stage disease can include B symptoms (symptoms that suggest a systemic rather than local disease), certain lab test values, enlarged lymph nodes in the chest, and more than three sites of disease.
For those with advanced-stage disease, there is a score system called the International Prognostic Score. The score calculated helps predict how the disease will progress and inform the doctor’s decisions about treatment.
One common treatment path for early-stage Hodgkin lymphoma is a specific chemotherapy regimen called ABVD, which stands for the four drugs used: doxorubicin, bleomycin, vinblastine, and dacarbazine. This is usually followed by a certain amount of radiation therapy. Each patient’s case varies and the number of chemotherapy cycles and radiation doses may be adjusted accordingly. However, one must be careful while using bleomycin, especially in elderly patients, due to a potential risk of lung toxicity.
For more advanced stages of Hodgkin lymphoma, six cycles of ABVD are often prescribed. But other regimens, which may include a greater variety of drugs and targeted therapies, are also considered.
After the first two cycles of ABVD chemotherapy, the doctor will assess the patient’s response to treatment using PET/CT scans. This helps to identify whether the disease is spreading or reducing in the body and to decide about further treatment options. The results are analyzed using a scoring system called the Deauville criteria.
At the end of treatment, further assessment takes place to confirm whether the patient is in remission, meaning that their disease symptoms have decreased or disappeared entirely. The doctor uses the Deauville score again to check the effectiveness of treatment.
If the disease does not respond to treatment or returns, a biopsy is performed for confirmation. In such cases, alternative chemotherapy regimens are considered, and an autologous stem cell transplant (using the patient’s stem cells) may be a standard option. The use of targeted therapies, such as brentuximab vedotin and PD-1 monoclonal antibodies, can also be considered.
What else can Lymphocyte Depleted Hodgkin Lymphoma be?
Lymph nodes can become swollen due to several conditions like infections, autoimmune diseases, and cancer. People who have swollen lymph nodes should go through medical tests to find out the cause. To figure out what’s wrong, a biopsy needs to be done. A biopsy is a procedure where a small piece of tissue is removed and examined under a microscope.
This is especially important if there’s a possibility the patient might have a type of cancer called Hodgkin lymphoma. Having enough tissue to examine is crucial to make a correct diagnosis.
What to expect with Lymphocyte Depleted Hodgkin Lymphoma
People who have a specific type of Hodgkin lymphoma, called lymphocyte-depleted classic Hodgkin lymphoma, face more risks compared to those with other types of the same disease. This makes their condition harder to manage. Moreover, these individuals have lower survival rates over 5 years without the disease progressing or deteriorating, and lower overall survival rates compared to those with other forms of classic Hodgkin lymphoma.
Possible Complications When Diagnosed with Lymphocyte Depleted Hodgkin Lymphoma
For those who survive Hodgkin lymphoma for a long time, the most severe late complications can include developing other cancers, heart diseases (especially if treated with chest radiation and/or a particular type of chemotherapy), underactive thyroid (resulting from chest radiation), and infertility especially related to a specific treatment plan named BEACOPP. It’s important to identify and properly manage these potential late complications among lymphoma survivors in order to decrease the risk of health problems and death related to their treatment.
Common Long-Term Complications:
- Secondary cancers
- Cardiovascular diseases, more common in patients treated with chest radiation and/or anthracycline-based chemotherapy
- Hypothyroidism, related to chest radiation
- Infertility, more often associated with the BEACOPP treatment plan
Preventing Lymphocyte Depleted Hodgkin Lymphoma
Being told you have lymphocyte-depleted classic Hodgkin lymphoma, a type of cancer, can have a big impact on your life and the lives of your loved ones. This can changes things dramatically on many levels, including your physical well-being, feelings, spiritual beliefs, and relationships. During each doctor’s appointment, your emotional and social wellbeing should be evaluated, as well as medical concerns. It’s crucial that you and your family members are fully informed about the possible treatment options – like combined therapy (which is more than one type of treatment together) and chemotherapy (cancer-killing drugs) – discussing advantages and disadvantages of each approach, potential side effects, what kinds of supportive care are available for you, what regular check-ups involve, and the long-term plan for your treatment.
In addition, certain cancer drugs can affect your ability to have children in the future. Because of this, your doctor may discuss options like sperm banking or egg freezing to help preserve your fertility. Additionally, regular check-ups with your general practitioner and oncologist (a doctor specializing in cancer treatment) are necessary to monitor how well your treatment is working and to keep an eye on any harmful effects from the treatment, such as potential damage to the heart (cardiotoxicity) and lungs (pulmonary toxicity).
