A GUIDE TO HODGKIN LYMPHOMA

Introduction

Hodgkin lymphoma (HL) is a type of cancer that affects the lymphatic system, which is part of the body’s immune system. HL is characterized by the presence of abnormal cells called Reed-Sternberg cells in the lymph nodes. The cause of HL is not well understood, but it is thought to be related to a combination of genetic and environmental factors.

Symptoms of HL can include swollen lymph nodes, fever, night sweats, fatigue, and weight loss. Diagnosis is typically made through a combination of physical examination, imaging tests, and biopsy of affected tissue.

Treatment for HL depends on the stage of the cancer and may include chemotherapy, radiation therapy, or a combination of both. In some cases, stem cell transplantation may be used. With appropriate treatment, the prognosis for HL is generally good, with many patients achieving long-term remission.

 

Classification of Hodgkin lymphoma

There are several subtypes of HL, and they are classified based on the appearance of the cancer cells under a microscope and other features. The four main subtypes of HL are:

1. Nodular Sclerosis Hodgkin Lymphoma (NSHL): This is the most common type of HL, accounting for about 70% of cases. It is more common in women than in men and typically affects young adults. The cancer cells appear as large, abnormal cells called Reed-Sternberg cells, surrounded by a fibrous tissue. NSHL usually starts in the lymph nodes in the chest area and spreads to other lymph nodes over time.
2. Mixed Cellularity Hodgkin Lymphoma (MCHL): This subtype accounts for about 20% of HL cases. It is more common in older adults and affects men and women equally. MCHL is characterized by an abundance of Reed-Sternberg cells and other types of cells, such as lymphocytes, eosinophils, and plasma cells. It usually starts in the lymph nodes in the upper body, such as the neck, armpits, or groin.
3. Lymphocyte-Rich Hodgkin Lymphoma (LRHL): This subtype accounts for about 5% of HL cases. It is more common in men than in women and usually affects people in their 30s or 40s. LRHL is characterized by a large number of lymphocytes and few Reed-Sternberg cells. It usually starts in the lymph nodes in the upper body and has a good prognosis.
4. Lymphocyte-Depleted Hodgkin Lymphoma (LDHL): This subtype is rare, accounting for less than 1% of HL cases. It is more common in older adults and people with HIV/AIDS. LDHL is characterized by a small number of Reed-Sternberg cells and an abundance of other cells, such as lymphocytes and histiocytes. It usually starts in the lymph nodes in the abdomen or pelvis and has a poor prognosis.

In summary, HL is classified into four subtypes: nodular sclerosis HL, mixed cellularity HL, lymphocyte-rich HL, and lymphocyte-depleted HL. The classification is based on the appearance of the cancer cells under a microscope and other features, such as age and gender. Each subtype has different characteristics and treatment options, and the prognosis varies depending on the subtype and stage of the disease.

 

Reed-Sternberg Cell Morphology

Reed-Sternberg (RS) cells are large, abnormal cells that are characteristic of Hodgkin’s lymphoma (HL). These cells are derived from B-cells, but they have lost their ability to produce immunoglobulins. There are two main variants of RS cells: classic and variant.

1) Classic RS cells are large, multinucleated cells with abundant eosinophilic cytoplasm. The nuclei are typically bilobed or multilobed, giving the cells a “owl’s eye” appearance. The nuclei contain one or more large, prominent nucleoli. The cytoplasm of classic RS cells is often surrounded by a clear halo, known as the “perinuclear halo.”

2) Variant RS cells, also known as “popcorn cells,” are smaller than classic RS cells and have a more round or oval shape. The nuclei of variant RS cells are often more irregular in shape and may contain multiple small nucleoli. The cytoplasm of variant RS cells is often more abundant and may contain vacuoles.

Both classic and variant RS cells are typically surrounded by an inflammatory background consisting of lymphocytes, eosinophils, plasma cells, and histiocytes. The morphology of RS cells and their variants is important in the diagnosis and classification of Hodgkin’s lymphoma.

 

Staging of Hodgkin Lymphoma

The staging system of Hodgkin lymphoma is used to describe the extent or spread of the cancer throughout the body.

The staging system for Hodgkin lymphoma is known as the Ann Arbor staging system, which is based on the location of the cancerous cells and whether they have spread to other parts of the body. There are four stages in the Ann Arbor staging system:

Stage I: The cancer is located in one lymph node or one group of lymph nodes.

Stage II: The cancer is located in two or more groups of lymph nodes on the same side of the diaphragm.

Stage III: The cancer has spread to lymph nodes on both sides of the diaphragm.

Stage IV: The cancer has spread to other organs, such as the bone marrow, liver, or lungs.

In addition to the four stages, the Ann Arbor staging system includes two additional classifications: A and B. The letter A is added to the stage number if the patient does not have any symptoms of the disease, while the letter B is added if the patient has symptoms such as fever, night sweats, or weight loss.

Overall, the staging system for Hodgkin lymphoma is an important tool for determining the appropriate treatment for each patient, as well as for predicting the patient’s prognosis or likely outcome.

 

Hodgkin lymphoma is a type of cancer that affects the lymphatic system, which is a network of vessels and organs that play a critical role in the immune system. The disease is characterized by the presence of abnormal cells called Reed-Sternberg cells in the lymph nodes.

Clinical features: The most common clinical features of Hodgkin lymphoma include:

1. Enlarged lymph nodes, especially in the neck, armpit, and groin.
2. Fever and chills.
3. Night sweats.
4. Unexplained weight loss.
5. Fatigue and weakness.
6. Itchy skin.
7. Chest pain, coughing, and difficulty breathing (if the disease affects the lungs).

 

Management: The management of Hodgkin lymphoma depends on the stage and extent of the disease, as well as the patient’s age and overall health. The main lines of management include:

1. Chemotherapy: Chemotherapy involves the use of powerful drugs to kill cancer cells. It is often the first line of treatment for Hodgkin lymphoma and is effective in most cases.
2. Radiation therapy: Radiation therapy involves the use of high-energy radiation to kill cancer cells. It may be used alone or in combination with chemotherapy.
3. Stem cell transplant: In some cases, a stem cell transplant may be recommended, particularly for patients with advanced-stage Hodgkin lymphoma who have not responded to other treatments.
4. Targeted therapy: Targeted therapy is a newer form of treatment that targets specific molecules that are involved in the growth and survival of cancer cells. It may be used in combination with chemotherapy or radiation therapy.
5. Clinical trials: Clinical trials are research studies that test new treatments for Hodgkin lymphoma. Patients who are eligible for a clinical trial may have access to new treatments that are not yet available to the general public.

Overall, the prognosis for Hodgkin lymphoma is generally good, especially when the disease is diagnosed early and treated promptly. With appropriate treatment, most patients can achieve long-term remission and lead normal, healthy lives.

 

It is divided into several subtypes, based on histological characteristics. The subtypes are:

• Nodular sclerosis Hodgkin lymphoma (NSHL)
• Mixed cellularity Hodgkin lymphoma (MCHL)
• Lymphocyte-rich Hodgkin lymphoma (LRHL)
• Lymphocyte-depleted Hodgkin lymphoma (LDHL)
• Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL)

 

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL)

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subtype of Hodgkin lymphoma, which is a type of cancer that affects the lymphatic system. It accounts for approximately 5% of all cases of Hodgkin lymphoma.

In NLPHL, the cancerous cells are called lymphocyte-predominant cells, which are large B-lymphocytes that are different from the Reed-Sternberg cells seen in classic Hodgkin lymphoma. These cells are found in a characteristic nodular pattern within the lymph nodes, and are usually surrounded by a mixture of non-cancerous immune cells.

NLPHL tends to occur in younger patients, and typically presents with painless swelling of the lymph nodes, most commonly in the neck, groin, or armpits. Other symptoms may include fatigue, fever, night sweats, and weight loss.

The prognosis for NLPHL is generally good, with a 5-year survival rate of over 90%. Treatment options include radiation therapy, chemotherapy, and sometimes surgery. Because NLPHL is a rare subtype of Hodgkin lymphoma, treatment decisions should be made by a team of medical professionals with experience in managing this condition.

Clinical presentations of Hodgkin’s lymphoma can vary depending on the subtype and stage of the disease. The most common symptoms include:

• Painful lymph node swelling (especially in the neck, armpit or groin).
• Fatigue.
• Night sweats.
• Fever.
• Unexplained weight loss.
• Itching.

 

General guidelines for patient evaluation and management include:

1. Diagnostic evaluation: The diagnosis of Hodgkin’s lymphoma is confirmed by a biopsy of a lymph node. Additional imaging tests such as CT or PET scans may be performed to determine the extent of the disease.
2. Staging: Once the diagnosis is confirmed, staging tests are performed to determine the extent of the disease. Staging is based on the number and location of affected lymph nodes and the presence of cancer in other organs.
3. Treatment: The treatment of Hodgkin’s lymphoma depends on the subtype and stage of the disease. Treatment options include chemotherapy, radiation therapy, targeted therapy, and stem cell transplantation. A combination of these therapies may also be used.
4. Follow-up: After completing treatment, patients will be monitored for any signs of recurrence. Regular follow-up appointments with the oncologist and imaging tests may be recommended.
5. Supportive care: Patients may experience side effects from treatment such as nausea, hair loss, and fatigue. Supportive care may include medications to manage these symptoms, as well as psychological support to address the emotional impact of cancer.

Overall, the prognosis for Hodgkin’s lymphoma is generally good, especially when the disease is diagnosed and treated in its early stages. However, the treatment and management of the disease can be complex, and patients should work closely with their healthcare team to develop an individualized treatment plan.