Nodular sclerosisc lymphoma is the most common type of classical Hodgkin's lymphoma in developed countries. In this article, we will talk about the symptoms and main features of this type of lymphoma and discuss its treatment methods.
What is lymphoma?
Lymph nodes are part of the human immune system. Lymphoma refers to the developed cancer in body's lymph tissues. In lymphomas, like cancers, unbridled proliferation of cells is observed.
Components of the lymphatic tissues of the body:
Lymph nodes (in axilla, neck, groin, abdomen, etc.), thymus (in children), adenoids and tonsils, spleen and bone marrow, lymph vessels are the main components of the body's lymph tissue. Lymphomas can occur in different parts of the body, such as the brain, skin, etc., and can also spread to other parts of the body after development.
Types of lymphomas:
Lymphomas are usually divided into two main categories:
The shape of the cells, the spreading method, the behavior of the lymphoma and the method of treatment are completely different in these two groups as explained below.
Hodgkin's lymphoma:
Hodgkin's lymphoma can occur in any part of the lymphatic tissue, but the most common location from where Hodgkin's lymphoma usually starts is the lymph nodes above the diaphragm (the muscle that separates the chest from the abdomen). The start location of Hodgkin's lymphoma is usually in the chest, cervical and axilla lymph nodes.
Hodgkin's lymphoma often spreads through the lymph vessels to other lymph nodes and involves the lungs, liver, and bone marrow along its way.
Non-Hodgkin’s lymphoma:
This lymphoma usually develops in the abdomen, groin, neck and axilla lymph nodes, creating painless swelling of the lymph nodes. In this article, we are going to look into the nodular sclerosis lymphoma, which belongs to the category of Hodgkin's lymphoma. Therefore, Non-Hodgkin's lymphoma and its types would be beyond the scope of this article.
Different types of Hodgkin's lymphoma:
classical Hodgkin lymphoma:
This type includes around ninety-five percent of all Hodgkin's lymphoma patients in developed countries. Classical Hodgkin's lymphoma has been divided into four subtypes based on the characterization of cancer cells in Hodgkin's lymphoma. All four subsets of classical Hodgkin's lymphoma have special cells called Reed-Sternberg cells which is an abnormal type of B-type lymphocyte.
In people with the classic type of Hodgkin's lymphoma, the large lymph nodes contain a small number of Reed-Sternberg cells while other immune cells occupy most of the lymph nodes. The four subsets of Hodgkin's lymphoma are:
- Nodular sclerosis Hodgkin's lymphoma:
- It is the most common type of Hodgkin's lymphoma
- Although it can occur at any age, it often affects adolescents and young adults.
- The onset of this type of Hodgkin's lymphoma is usually from the lymph nodes in the neck or chest.
- Hodgkin's lymphoma with mixed cells (mixed cellulite Hodgkin's lymphoma):
- It is also seen in elders.
- Most lymph nodes above the diaphragm are involved
- Lymphocyte rich Hodgkin's lymphoma
- Lymphocyte-depleted Hodgkin's lymphoma
- Hodgkin's rarest lymphoma.
- Older people are more likely to have it.
- This type of lymphoma is more likely to spread if it starts in the abdomen, spleen, liver, and bone marrow.
Non-classical Hodgkin lymphoma:
- Another name for this lymphoma is Nodular lymphocyte-predominant Hodgkin lymphoma.
- The cancer cells in this type of lymphoma are large cells called popcorn cells.
- This type includes about five percent of all Hodgkin's lymphomas.
- It usually starts in the axillary lymph nodes and cervix
- This type of lymphoma is more common in men than women.
Nodular sclerosis Hodgkin's lymphoma:
- It is the most common type of Hodgkin's lymphoma in developed countries.
- The histological characteristics of this lymphoma are:
- Lacunar cells seen under a microscope are characteristically found in nodular sclerosis lymphoma
- In this lymphoma, collagen bands divide the involved lymph node into nodules that are full of inflammatory cells mixed with lacunar cells.
- About sixty to eighty percent of all Hodgkin's lymphomas in developed countries (i.e. US and European countries) are of this type.
- Involvement of lymph nodes inside the chest (mediastinum) includes 80% of cases of this type of lymphoma. In other words, the most common lymph nodes involved in Hodgkin's nodular sclerosis lymphoma are the lymph nodes of the mediastinum.
- The second most common lymph nodes involved in nodular sclerosis are the supraclavicular and neck lymph nodes.
It is interesting to know that most types of Hodgkin's lymphoma are more common in men than women. The only type of Hodgkin's lymphoma that is equally common in men and women is this type of lymphoma.
Clinical manifestations of nodular sclerosis Hodgkin's lymphoma:
About forty percent of sufferers experience the following symptoms:
- Fever
- Night sweats
- Weight loss (weight loss of more than ten percent within six months)
- There is a mass developed somewhere in the body in fifty percent of patients. (which is called bulky disease form of nodular sclerosis)
- Ten percent of patients have lung involvement.
- Spleen involvement occurs in 10% of patients.
- Only 3% of patients get their bone marrow involved.
Histological characteristics of Nodular sclerosis Hodgkin's lymphoma:
- The presence of fibroclagen bands, called sclerosis, that divide the lymph node into high-cellular nodules.
- Lacunar cells are cells with a bicuspid eosinophilic nucleus with a large, compact cytoplasm that gives the cell a hollow appearance.
- Involved lymph node when looked by a macroscope, has a thick capsule where the lymph node is involved partially or completely.
Differential pathological diagnoses of Nodular sclerosis Hodgkin's lymphoma:
Symptoms of the Nodular sclerosis Hodgkin's lymphoma may overlap with the followings:
Symptoms of nodular sclerosis Hodgkin's lymphoma:
- Painless swelling of the lymph nodes in the neck and armpits
- Constant fatigue
- Fever
- Weight loss (more than ten percent weight loss in the last six months) without dietary restrictions
- Severe itching
- Lymph nodes become painful and sensitive after drinking alcohol
- Decreased appetite
Diagnostic tests for nodular sclerosis Hodgkin's lymphoma:
Tissue biopsy:
The presence of lacunar cells in addition to fibroclagen bands that divide tissue into multiple nodules containing lacunar cells and inflammatory cells is the main characteristic of this lymphoma when studying tissue samples.
ICH on tissue sample (immunohistochemistry):
- Positive CDC
- Negative CD45
- Sometimes:
- Positive CD15
- Positive PAX5
- And other markers of B lymphocytes are negative.
Imaging studies:
Blood test:
- Blood cell count
- Liver enzymes
- Creatinine
Causes of nodular sclerosis Hodgkin's lymphoma:
Risk factors for this cancer include:
- Immune system malfunctioning
- Age
- Some infections
- Exposure to some chemicals
- Getting another cancer
- Chemotherapy for other cancers
However, it should be said that the exact cause of this lymphoma is not fully known yet and the aforementioned reasons are considered as risk factors only.
Staging of nodular sclerosis lymphoma:
Stage one:
Only one lymph node or a group of neighboring lymph nodes are involved.
Stage two:
Two or more lymph node groups are located on one side of the diaphragm. Most cases are diagnosed at this stage.
Stage three:
The lymph nodes are located on either side of the diaphragm.
Stage four:
A diffused or advanced type of lymphoma that has spread to at least one organ outside the lymphatic system, such as lungs, liver, or bone marrow.
Diagnosis of nodular sclerosis lymphoma:
We will consider the following in the diagnosis:
- Patient history
- Experiments
- Tissue sampling
- Imaging is required most of the time
CT scan and PET scan provide the most valuable information about the stage of the disease to the doctor.
Lymphoma treatment:
Lymphoma treatment includes the following:
Which treatment is better?
The choice of treatment depends on the following:
- Presence or absence of bulky disease
- Presence or absence of asymptomatic (fever, fatigue, nausea, etc.)
- Patient's age
- Test results
- Patient general health
- patient's adaptation and admission
- stage of the disease
Stage one treatment:
Two or four cycles of chemotherapy (chemotherapy), usually accompanied by regional radiotherapy or just four to six cycles of chemotherapy. In people who cannot do chemotherapy, radiotherapy is the only option.
Stage two treatment:
Four to six cycles of ABVD medication or three cycles of chemotherapy.
Stage three and four treatment:
- At least six cycles of chemotherapy with ABVD diet
- Over eight cycles of chemotherapy with BEACOPP diet
- Immunotherapy with monoclonal antibodies is usually used in more advanced stages or in recurrent cases.
The final word:
Nodular sclerosis Hodgkin's lymphoma has the highest survival rate among classical Hodgkin's lymphomas. The five-year survival of patients (especially in the early stages) is more than ninety percent.