Nodular sclerosis Hodgkin lymphoma (NSHL)

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NSHL is a type of lymphoma. Lymphomas are cancers that develop in the lymphatic system, an important part of the immune system that helps protect the body against infection and disease. These cancers cause white blood cells, known as lymphocytes, to grow uncontrollably.

Doctors subdivide lymphomas into Hodgkin and non-Hodgkin types depending on the type of lymphocyte they affect. Classic HL is characterized by the presence of Reed-Sternberg cells, which are large lymphocytes that may have more than one nucleus.

The term “nodular sclerosing” refers to nodules and scar tissue (sclerosis) that can form in the lymph nodes due to HL. These nodular lesions are more common in NSHL than in other subtypes of HL.

Yes, NSHL is curable. Data from the Surveillance, Epidemiology, and End Results (SEER) Program from 2012–2018 suggest that there is a high likelihood that people with HL will live for at least 5 years after their diagnosis, in comparison to people without the condition.

According to this data, the chances of surviving HL for at least 5 years were:

• 93% for localized HL
• 95% for regional HL
• 83% for distant HL

However, according to research, around 5–10% of people with HL do not respond to treatment, and 10–30% of people will relapse after going into remission.

Generally, the earlier doctors detect and treat NSHL, the better the person’s outlook. Other factors can make HL more serious and affect a person’s outcome, including:

• being male
• being over 45 years of age
• having additional symptoms
• having a high white blood cell count
• having low red blood cell, lymphocytes, and blood albumin levels

The most frequent symptom of HL is swollen lymph nodes. In NSHL, the nodes can be large and may feel hard or rubbery, but they are usually painless.

Although NSHL typically affects lymph nodes in the neck, a person may also have a cough and shortness of breath if it involves lymph nodes in the chest.

Other common symptoms of HL include:

• fatigue
• fever
• night sweats
• unintentional weight loss

An unusual symptom of NSHL is itchy skin and pain in the lymph nodes after drinking alcohol.

Some symptoms, called “B symptoms,” are significant to the person’s outlook and staging of the disease. They include:

• fever
• extreme night sweats
• loss of more than 10% of body weight over 6 months

Doctors use staging to determine the extent and spread of cancer throughout the body. The Lugano classification divides HL into four stages, which are:

• Stage 1: The cancer affects a single lymph node area, or lymphoid organ, such as the thymus. Alternatively, it may affect one part of one organ outside the lymphatic system.
• Stage 2: The cancer is present in two or more lymph node groups on the same side of the diaphragm or in a lymph node group and a nearby organ.
• Stage 3: The cancer is present in lymph node groups on both sides of the diaphragm or above the diaphragm and in the spleen.
• Stage 4: The cancer has spread widely outside the lymphatic system, such as to the liver, lungs, or bone marrow.

Doctors also use sub-stages A and B to indicate the presence or absence of certain symptoms, such as fever, night sweats, and weight loss.

Doctors do not know why some people get NSHL and others do not, but there are certain risk factors that increase the chances of a person getting it, including:

• being an adolescent or young adult
• having a previous Epstein-Barr virus infection, such as mononucleosis
• having an autoimmune disease
• having a weakened immune system
• having family members with the condition
• having obesity

However, these factors do not guarantee that a person will get NSHL.

Doctors diagnose NSHL by taking a sample of tissue from a lymph node. This is known as a biopsy. They may also perform other tests to rule out infections or help with cancer staging. These include:

• Blood tests: These may include a complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, and tests for viruses such as HIV, hepatitis B, or hepatitis C virus.
• Immunohistochemistry: These tests look for CD15 and CD30, which are proteins on the surface of HL cells.
• Imaging studies: These may include chest X-rays and CT scans to look for organ involvement and help with staging.

NSHL treatment depends on the stage of the disease and how favorable the outlook is. Usually, doctors recommend that those with early stage HL undergo short courses of chemotherapy, followed by radiation therapy.

They may recommend that people with more advanced disease receive longer courses of chemotherapy and no radiation.

In people who experience a relapse, doctors may consider high dose chemotherapy, followed by a stem cell transplant. This is a challenging procedure, though, and not all people will be good candidates.

Scientists are still researching newer treatments, such as monoclonal antibody drugs.

NSHL is a type of cancer affecting the lymphatic system. It is the most common subtype of classical Hodgkin lymphoma and tends to affect adolescents and young adults.

Symptoms of NSHL include painless swelling of the lymph nodes, fever, night sweats, and unexplained weight loss. Treatment may involve chemotherapy with or without radiation or stem cell transplants.

HL has a high survival rate, and this improves if doctors diagnose and treat it early.