Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare form of Hodgkin lymphoma that is characterised by variants of Reed-Sternberg cells known as ‘popcorn’ cells because of their appearance. It develops slowly and is often diagnosed earlier than the classical subtypes of Hodgkin lymphoma.
The lymphatic system is a network of tissues and organs that help our bodies fight infection and disease. It is composed of lymph vessels (carries lymph fluid around the body), lymph fluid (carries nutrients around the body and removes unwanted bacteria/viruses) and lymph nodes/glands (filters lymph fluid and empties it into the bloodstream). Some of the most well-known lymph tissues include the bone marrow, the spleen, and the tonsils.
NLPHL is more common in males, and tends to be diagnosed between the ages of 30-50. However, anyone can develop this disease.
Treatment
If NLPHL is detected, it will be staged and graded based on size, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including age, stage of disease and overall health. The types of treatments generally don’t vary between adults and children.
Treatment options for patients with NLPHL may include:
- Chemotherapy.
- Radiation therapy.
- Surgery (usually a lymphadenectomy, which is a procedure that removes affected lymph nodes and surrounding tissue).
- Watch and wait.
- Clinical trials.
- Palliative care.
For more information on the treatment options, please refer to the Rare Cancers Australia Treatment Options page.
Risk factors
While the exact cause of NLPHL remains unknown, the following factors might increase the risk of a person developing this type of cancer.
- Having been infected with HIV or EBV.
- Being immunocompromised.
- Family history of Hodgkin lymphoma.
- Exposure to chemical solvents, such as certain pesticides and fertilisers.
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Early symptoms
The main symptom of NLPHL is a firm, painless lump I the neck, armpits and/or groin. People may also develop what is known as ‘B symptoms’, which are:
- Extreme night sweats.
- Persistent fevers.
- Unexplained weight loss.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis/diagnosing
If your doctor suspects you have Hodgkin lymphoma, they will order a range of diagnostic tests to confirm the diagnosis, and refer you to a specialist for treatment.
Physical examination
Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities.
Blood tests
Your doctor will recommend a blood test for check for signs of Hodgkin lymphoma and determine overall health. The most common test is a full blood count, which will analyse the levels of red blood cells, white blood cells and platelets in the blood. Low counts in any of these categories could be indicative of disease.
Imaging
The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), ultrasound, and/or positron emission tomography (PET scan), to check for signs of tumours and/or metastasis.
Biopsy
Once the location of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. In most cases, this may be done by either an excision biopsy (where the lymph node is completely or partially removed), or a core needle biopsy (where a needle is inserted into the lymph node to remove a sample). Both of these procedures are often performed as a day surgery. In rare cases, a bone marrow biopsy may be performed to see if it contains any cancerous cells.
This process involves inserting the needle into the hipbone (or the breastbone in some cases) to remove samples of solid and liquid bone marrow. These samples will then be analysed for cancer cells.
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on the rate and depth of tumour growth, susceptibility to treatment, age, overall fitness, and medical history. Generally, early-stage NLPHLs have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
References
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.