Overview of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL)
Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) is a very rare subtype of Hodgkin lymphoma which accounts for around 5% of Hodgkin lymphoma cases each year. NLPHL is slower growing in nature to the more common subtypes of HL which are more aggressive (fast growing) in nature.
NLPHL resembles some of the indolent non-Hodgkin lymphomas as it is more chronic in nature and has long remission periods but respond very well to treatment.
Usually the only sign of NLPHL is a painless lump in the neck, armpit or groin area that does not go away over time. These are enlarged lymph nodes that swell due to the lymphoma. In NLPHL they are often only in one area of the body. Some patients also report fatigue (tired and lacking energy).
Who is affected?
This subtype is more common in people aged between 30 to 50 years. It is around three times more common in men than women. It can also affect all ages, including younger adults.
There are four other subtypes of Hodgkin lymphoma that behave more aggressively and are treated differently to NLPHL. For more information on the other subtypes see classical Hodgkin lymphoma.
Treatment and prognosis
NLPHL is diagnosed by having a lymph node biopsy and the treatment for NLPHL will work very well in most cases . If NLPHL is in only one place or the lumps are close together the only treatment needed might be surgery to remove the lumps. Some patients may also have radiotherapy to the affected area.
More widespread (advanced) NLPHL will be monitored and chemotherapy may be needed. If the NLPHL relapses (comes back), treatment is usually successful again.
What are the symptoms of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL)?
Usually the only sign of NLPHL is a painless lump in the neck, armpit or groin area that does not go away over time. Some patients also report fatigue (tired and lacking energy).
B symptoms can include the following symptoms:
- Night sweats (drenching sleepwear & bedding at night)
- Recurrent fevers (greater than 38C)
- Unexplained weight loss
Diagnosis & Staging
A biopsy is always required for a diagnosis of NLPHL. A biopsy is a surgical procedure to remove part of or all of an affected lymph node or other abnormal tissue to look at it under the microscope by a pathologist to see what the cells look like. The biopsy can be done under local or general anaesthetic depending on what part of the body is being biopsied. The biopsy can be one of three ways:
- Fine needle aspirate
- Core needle biopsy
- Excisional node biopsy
An excisional node biopsy is the best investigative option, as it collects the most adequate amount of tissue to be able to do the necessary testing for a diagnosis.
Waiting for test results can be a difficult time. It may help to talk to your family, friends or a specialist cancer nurse.
Once a diagnosis of NLPHL is made, further tests are required to see if the lymphoma is in other parts of the body. This is called staging. The staging of your lymphoma helps your doctor to know what the best treatment for the patient.
There are four stages from stage 1 (lymphoma in one area) through to stage 4 (lymphoma that is widespread).
- Early stage means stage 1 and sometimes stage 2 lymphoma. Can also be referred to as ‘localised’. Stage 1 or 2 means that the lymphoma is found in one area or a few areas close together.
- Advanced stage means the lymphoma is stage 3 and stage 4, and it is widespread. In most cases, the lymphoma has spread to parts of the body that are far from each other.
What is the ‘grade’ of lymphoma?
Lymphomas are also often grouped as either indolent or aggressive. Indolent lymphomas are usually slow growing and aggressive lymphomas are fast growing. The grade is also referred to as the clinical behaviour of the lymphoma. NLPHL is an indolent lymphoma.
Staging scans and tests
The scans and tests needed for staging and before treatment can start may include:
- Positron emission tomography (PET) scan
- Computed tomography (CT) scan
- Bone marrow biopsy
Patients may also undergo a number of baseline tests prior to any treatment commencing to check organ functions. These are often repeated during and after the treatment has completed to assess whether the treatment has affected the functioning of organs. Sometimes the treatment and follow-up care may need to be adjusted to help manage side effects. These may include:
- Physical examination
- Vital observations (blood pressure, temperature, & pulse rate)
- Heart scan
- Kidney scan
- Breathing tests
- Blood tests
It may take some time for all the necessary biopsies and tests to be done (an average of 1-3 weeks), but it is important for the doctors to have a complete picture of the lymphoma and the general health of the patient in order to make the best treatment decisions
Many of the staging and organ function tests are done again after treatment to check whether the lymphoma treatment has worked and the effect this has had on the body.
It is important to note that lymphoma is what is known as a systemic cancer. It can spread throughout the lymphatic system and nearby tissue and organs. Many patients are diagnosed at an advanced stage, unlike advanced stage solid tumour cancers, such as bowel or lung cancer.
Prognosis of NLPHL
NLPHL is a highly treatable lymphoma with standard first-line treatment and up to 90 percent of patients are cured or have a long remission period ( 10 years or longer at a time).
Treatment for nodular lymphocyte predominant Hodgkin lymphoma (NLPHL)
Once all the results from the biopsy and the staging scans have been completed, the doctor will review these to decide the best possible treatment for a patient. At some cancer centres, the doctor will also meet with a team of specialists to discuss the best treatment and this is called a multidisciplinary team (MDT)meeting.
Doctors take into consideration many factors about the lymphoma and the patient’s general health to decide when and what treatment is required.
This is based on:
- The stage of lymphoma
- Symptoms (including the size and location of the lymphoma)
- How the lymphoma is affecting the body
- Past medical history & general health
- Current physical and mental wellbeing
- Patient preferences
If treatment is needed, the patient’s type and severity of the symptoms, age, overall health and degree of the thickness of the blood, will help determine which treatment is selected.
The standard first line treatments may include (for adult hospitals):
Early Stage NLPHL without B symptoms
Surgery followed by radiotherapy
Early Stage NLPHL with B symptoms
Chemotherapy and monoclonal antibody *CVP-R x 2-3 cycles followed by radiotherapy
Chemotherapy and monoclonal antibody CVP-R x 6 cycles
Advanced Stage NLPHL
Chemotherapy and monoclonal antibody *CHOP-R x 6 cycles
Chemotherapy and monoclonal antibody *ABVD-R x 6 cycles
Chemotherapy and monoclonal antibody CVP-R x 6 cycles
- CVP-R (cyclophosphamide, vincristine, prednisolone and rituximab)
- CHOP-R (cyclophosphamide, doxorubicin, vincristine, prednisolone and rituximab)
- ABVD-R (doxorubicin, bleomycin, vinblastine, dacarbazine and rituximab)
Common side effects of treatment
There are many different side effects of the treatment and these are dependent on the treatment that has been given. The treating doctor and/or cancer nurse can explain the specific side effects prior to the treatment. Some of the more common side effects of treatment may include:
- Anaemia (low red blood cells carry oxygen around the body)
- Thrombocytopenia (low platelets that help bleeding and clotting)
- Neutropenia (low white blood cells help with immunity)
- Nausea and vomiting
- Bowel problems such as constipation or diarrhoea
- Fatigue (tiredness or lack of energy
The medical team, doctor, cancer nurse or pharmacist, should provide information about:
- What treatment will be given
- What are the common and possible side effects for the treatment
- What side effects do you need to report to the medical team
- What are the contact numbers, and where to attend in case of emergency 7 days a week and 24 hours per day
Some treatments for lymphoma can reduce fertility and this is more likely with certain chemotherapy regimens (combinations of drugs) and high-dose chemotherapy used before a stem cell transplant. Radiotherapy to the pelvis also increases the likelihood of reduced fertility. Some antibody therapies may also affect fertility, but this is less clear.
The doctor should advise on whether fertility may be affected and if fertility preservation should be considered before treatment starts (if this is an option).
Once treatment has completed, post treatment staging scans are done to review how well the treatment has worked. The scans will show the doctor if there has been a:
- Complete response (CR or no signs of lymphoma remain) or a
- Partial response (PR or there is still lymphoma present, but it has reduced in size)
If all goes well regular follow-up appointments will be made for every 3-6 months to monitor the below:
- Review the effectiveness of the treatment
- Monitor any ongoing side effects from the treatment
- Monitor for any late effects from treatment over time
- Monitor signs of the lymphoma relapsing
These appointments are also important so that the patient can raise any concerns that they may need to discuss with the medical team. A physical examination and blood tests are also standard tests for these appointments. Apart from immediately after treatment to review how the treatment has worked, scans are not usually done unless there is a reason for them. For some patient’s appointments may become less frequent over time.
Relapsed or refractory NLPHL treatment
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) usually responds very well to chemotherapy, but in some patients the lymphoma comes back (relapses after a period of remission) or in rare cases does not respond to initial first-line treatment (refractory or does not respond to treatment) There are other treatments (second-line) that can work well and can include:
- Combination chemotherapy
- Monoclonal antibody treatment
- Repeat the previous treatment if a long remission was achieved
- Clinical trial participation
If a relapse is suspected another biopsy need to be done often with the same staging tests that were explained above in the staging section.
Treatment under investigation
There are many treatments that are currently being tested in clinical trials around the world and in Australia for patients with both newly diagnosed and relapsed lymphoma.
What happens after treatment?
Sometimes a side effect from treatment may continue or develop months or years after treatment has completed. This is called a late effect.
This can be a challenging time for many people and some of the common concerns can be related to:
- Mental wellbeing
- Emotional health
- Work, study, and social activities
Health and wellbeing
A healthy lifestyle, or some positive lifestyle changes after treatment can be a great help after the treatment has been finished. Making small changes such as eating and increasing fitness can improve health and wellbeing and help the body to recover. There are many self-care strategies that can help during the recovery phase.