Table of ContentsView AllTable of ContentsTypes of LymphomasCharacteristics of NSHLSymptomsCausesDiagnosisTreatmentsPrognosisSurvivorship
Table of ContentsView All
View All
Table of Contents
Types of Lymphomas
Characteristics of NSHL
Symptoms
Causes
Diagnosis
Treatments
Prognosis
Survivorship
NSHL occurs most commonly between the ages of 15 and 35 and affects equal numbers of males and females. Treatment most often includes chemotherapy with or without radiation, and monoclonal antibody therapy or a stem cell transplant may be needed in the advanced stages or with a recurrence.
The prognosis is very good, in general, with roughly 90 percent 5-year survival. That said, the treatment can be challenging. Support, as well as long-term management, are important.
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There are two main categories of lymphoma, includingHodgkin and Non-Hodgkinlymphoma. Hodgkin lymphoma (also referred to as Hodgkin disease) is in turn broken down into two groups—classic Hodgkin lymphoma (roughly 95%) and nodular lymphocyte predominant Hodgkin lymphoma (around 5%).
Classic Hodgkin lymphoma is broken down into four subtypes based on pathology (how the tumor looks under the microscope) and includes:
It’s important to note that NSHL is different and treated differently than nodular lymphocyte-predominant Hodgkin lymphoma even though the names appear similar.
Nodular sclerosing Hodgkin lymphoma differs from other subtypes of classic Hodgkin lymphoma based on the appearance of the cells, and the most likely areas of the body where they occur.
Origin
Hodgkin lymphomas arise in a type of lymphocytes called B lymphocytes orB cellsand begin in lymph nodes. Lymph nodes are like outposts that occur at various points along thelymphatic channelsthat run throughout the body.
While Hodgkin lymphomas may arise in any lymph nodes, NSHL is found most often in lymph nodes in themediastinum(chest), neck, and axilla (armpits). Of these sites, over 50% occur in the chest.
Pathology
The abnormal B lymphocytes found in NSHL are referred to asReed Sternberg cells. These cells are larger than normal B cells and have two nuclei; giving the cell the appearance of an owl’s face when they are examined with a microscope.
The name “nodular sclerosing” is derived from the appearance of lymph node tissue, which contains a large amount of scar or fibrous tissue (sclerosis).
Concerns over “swollen glands” are what usually alert a person with NSHL to see a doctor, but many people have nonspecific symptoms such as fatigue and loss of appetite as well.
Enlarged Lymph Nodes
With NSHL, it’s thought that the swelling in lymph nodes occurs due to activation of other immune cells in the nodes rather than from a large number of cancerous B cells.
B Symptoms
Roughly 40% of people with NSHL will also have what has been coined theB symptomsof lymphoma.
These include:
Itching, along with a burning sensation is common in Hodgkin lymphoma and has been coined the “Hodgkin itch.” It’s most common in the lower legs and may begin even before the diagnosis is made. However, itching is not one of the B symptoms.
Causes and Risk Factors
The exact causes of NSHL are unknown, but several risk factors have been identified.
Rates of NSHL do not appear to be different based on sex or ethnic background.
The diagnosis of lymphoma is made with alymph node biopsy. Other tests that may be done include:
How Lymphoma Is Diagnosed
Staging
Staging lymphomas is very important to determine the best treatment options (NSHL is most often diagnosed at stage II).
A positron emission tomography combined with computed tomography (PET/CT) scan is most sensitive in determining the extent of these cancers, as cancer may be found even in normal-sized lymph nodes.
NSHL is assigned a stage and category based on symptoms, findings on physical exam, results of the lymph node biopsy, results of imaging tests such as PET/CT, and bone marrow test results (when needed).
Stages include:
Categories include:
The size of the mass is also part of the description of the tumor.
Differential Diagnosis
One type of non-Hodgkin’s lymphoma, primary mediastinal large B-cell lymphoma (PMBL), may also be found in the chest and can look similar under the microscope. Immunohistochemistry tests may be done to tell the difference as the two diseases are treated in different ways.
The treatment of NSHL is based more on the stage of the disease than the type of Hodgkin lymphoma. Options will depend on the number of lymph nodes affected and their locations, as well as the presence of lymphoma in other tissues.
With early-stage lymphomas (stage I or stage II) chemotherapy with or without radiation is often curative, yet (unlike many solid tumors), a cure may be possible even with advanced lymphomas.
Fertility Preservation and Pregnancy
For those who would like to have children after treatment, it’s important to be aware of the effects of Hodgkin lymphoma on fertility. There are different options for preserving fertility for those who are interested.
For those who are pregnant when diagnosed, the management of Hodgkins during pregnancy also requires special consideration.
Chemotherapy
Chemotherapy is the mainstay of treatment for NSHL. With early-stage disease, common regimens includeABVDor dose-escalatedBEACOPP(the letters represent the different chemotherapy drugs) with or without radiation.
Radiation Therapy
Radiation may be given to areas of lymph node involvement after chemotherapy.
Monoclonal Antibodies
Monoclonal antibodytreatment with the drug Adcentris (brentuximab) is now available for those who have resistant or recurrent tumors. Adcentris may also be used along with chemotherapy (ABVD) with advanced stage lymphomas.
Immunotherapy
A type ofimmunotherapyreferred to as immune checkpoint inhibitors may be used. Drugs in this category include Opdivo (nivolumab) and Keytruda (pembrolizumab) and are expected to improve survival for those with difficult-to-treat lymphomas.
Stem Cell Transplants
Nonmyeloablative stem cell transplantsare another option for some people who might not tolerate the high dose chemotherapy used with a conventional stem cell transplant.
Clinical Trials
For people who relapse or have lymphomas that aren’t responding to the above treatments (refractory tumors), other options that aren’t well enough established to be FDA-approved are sometimes available through clinical trials.
Treatment Side Effects
Fortunately, people with Hodgkin lymphomas often receive less toxic chemotherapy and have radiation delivered to smaller fields than in the past.
Short-Term Side Effects
The immediate side effects of chemotherapy include hair loss,bone marrow suppression(reduced levels of white blood cells, red blood cells, and platelets), and nausea and vomiting. Fortunately, advances have made these side effects much more tolerable than in the past.
Side effects of radiation therapyinclude skin redness and fatigue. When radiation is delivered to the chest, inflammation of the lungs and esophagus may occur.
Long-Term Side Effects
Since many people with nodular sclerosing Hodgkin lymphoma are young, and the survival rate is high, thelong-term effects of cancer treatmentbecome very important. One of the greatest concerns is the risk ofsecondary cancers in Hodgkin lymphoma survivors.
People who have been treated for Hodgkin lymphoma are roughly 4.6 times more likely to develop secondary cancer (cancer due to chemotherapy drugs or radiation), with the most common tumors being breast cancer, lung cancer, and thyroid cancer.
Coping and Support
Even though NSHL has a good survival rate, the treatment to get to that point can be challenging and sometimes grueling. Support is critical, and in addition to reaching out to friends and family, many people find it helpful to become involved in a support group. The internet provides an opportunity for people to connect with others with their subtype of Hodgkin lymphoma.
Living with Hodgkin Lymphoma: Coping and Support
Nodular sclerosing Hodgkin lymphoma has a higher survival rate than other types of classic Hodgkin lymphoma, with the five-year survival rate being greater than 90%.
Recurrence
NSHL may recur. Roughly half of all recurrences will occur within two years, with 90% of recurrences occurring within five years.
Rising Survival Rates with Hodgkin Lymphoma
The concept of survivorship and survivorship care is relatively new but critical with cancers such as NSHL that often occur in young people and have high survival rates.
For those who are diagnosed as children, issues such as fatigue, growth delays, thyroid problems, and hearing loss may arise. For everyone who has been treated for NSHL, the risk of secondary cancers is present.
It’s important to be aware ofsurvival guidelinesfor Hodgkin lymphoma and what this might mean for you, as well as to stay abreast of these as more is learned.
For example, at the current time, it’s recommended that women who have received radiation therapy to the chest for Hodgkin lymphoma between the ages of 10 and 30 havebreast magnetic resonance imaging (MRI)screening in addition to mammograms.
When you have completed treatment, your oncologist should fill out a survivorship care plan for you outlining some of these recommendations.
A Word From Verywell
Nodular sclerosing Hodgkin lymphoma is often curable, but the treatment is challenging. In addition, late side effects may occur, and lifelong monitoring is needed. What this means is that support is crucial throughout the journey. Learning to ask for and accept help, and connecting with the Hodgkin community to find your “tribe” are invaluable as you face this disease.
SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Bowen, E., Pfeiffer, R., Linet, M. et al.Relationship Between Ambient Ultraviolet Radiation and Hodgkin Lymphoma Subtypes in the United States.British Journal of Cancer.2016;114(7):826-831.NCCN Clinical Practice Guidelines in Oncology. Hodgkin Lymphoma. Version 3.2018. April 14, 2018.Radford, J., and D. Longo.Second Cancers after Treatment for Hodgkin Lymphoma – Continuing Cause for Concern.The New England Journal of Medicine. 2015. 373:2572-2573.Schaapveld, M., Aleman, B., van Eggermond, A. et al.Second Cancer Risk for Up to 40 Years after Treatment for Hodgkin’s Lymphoma.The New England Journal of Medicine. 2015. 373:2499-2511.
Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Bowen, E., Pfeiffer, R., Linet, M. et al.Relationship Between Ambient Ultraviolet Radiation and Hodgkin Lymphoma Subtypes in the United States.British Journal of Cancer.2016;114(7):826-831.NCCN Clinical Practice Guidelines in Oncology. Hodgkin Lymphoma. Version 3.2018. April 14, 2018.Radford, J., and D. Longo.Second Cancers after Treatment for Hodgkin Lymphoma – Continuing Cause for Concern.The New England Journal of Medicine. 2015. 373:2572-2573.Schaapveld, M., Aleman, B., van Eggermond, A. et al.Second Cancer Risk for Up to 40 Years after Treatment for Hodgkin’s Lymphoma.The New England Journal of Medicine. 2015. 373:2499-2511.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Bowen, E., Pfeiffer, R., Linet, M. et al.Relationship Between Ambient Ultraviolet Radiation and Hodgkin Lymphoma Subtypes in the United States.British Journal of Cancer.2016;114(7):826-831.NCCN Clinical Practice Guidelines in Oncology. Hodgkin Lymphoma. Version 3.2018. April 14, 2018.Radford, J., and D. Longo.Second Cancers after Treatment for Hodgkin Lymphoma – Continuing Cause for Concern.The New England Journal of Medicine. 2015. 373:2572-2573.Schaapveld, M., Aleman, B., van Eggermond, A. et al.Second Cancer Risk for Up to 40 Years after Treatment for Hodgkin’s Lymphoma.The New England Journal of Medicine. 2015. 373:2499-2511.
Bowen, E., Pfeiffer, R., Linet, M. et al.Relationship Between Ambient Ultraviolet Radiation and Hodgkin Lymphoma Subtypes in the United States.British Journal of Cancer.2016;114(7):826-831.
NCCN Clinical Practice Guidelines in Oncology. Hodgkin Lymphoma. Version 3.2018. April 14, 2018.
Radford, J., and D. Longo.Second Cancers after Treatment for Hodgkin Lymphoma – Continuing Cause for Concern.The New England Journal of Medicine. 2015. 373:2572-2573.
Schaapveld, M., Aleman, B., van Eggermond, A. et al.Second Cancer Risk for Up to 40 Years after Treatment for Hodgkin’s Lymphoma.The New England Journal of Medicine. 2015. 373:2499-2511.
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