Nodular lymphocyte-predominant Hodgkin lymphoma

This information is about nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), which is an uncommon type of Hodgkin lymphoma.

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What is NLPHL?

Who gets NLPHL and why?

Symptoms

Diagnosis and staging

Outlook

Treatment

Follow-up

Relapse

Research and targeted treatments

What is NLPHL?

Nodular lymphocyte-predominant Hodgkin lymphoma is an uncommon type of Hodgkin lymphoma – a type of cancer that starts in the lymphatic system

About 1 in 20 Hodgkin lymphomas diagnosed (5%) are NLPHL. That means that there are about 90 cases diagnosed in the UK each year.

It is called ‘nodular lymphocyte-predominant’ because it has particular cells that can be seen under a microscope. These are called LP cells (for ‘lymphocyte-predominant’), but doctors sometimes call them popcorn cells because they look a bit like pieces of popcorn.

NLPHL is different to other types of Hodgkin lymphoma. It tends to grow more slowly and to be diagnosed earlier, when it is only growing in one or two groups of lymph nodes (glands). The lymph nodes it grows in tend to be in places that can be easily felt, rather than deep inside the body. For these reasons, treatment is usually very successful and many people are cured.

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Who gets NLPHL and why?

The two main groups that get NLPHL are children and people in their 30s and 40s. Three out of four cases are diagnosed in males. It is more common in children under 10 than in 10–18 year olds.

We don’t know why some people get NLPHL. You can’t catch it, or pass it on to anyone else. There are no particular risk factors that we know of, so it isn’t down to anything you’ve done or not done.

Lymphomas are not normally inherited. But unlike other types of Hodgkin lymphoma, brothers, sisters and parents of someone with NLPHL have a very slightly higher risk of getting it themselves – but it is still very unlikely.

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Symptoms

The only symptom that most people have with NLPHL is enlarged (swollen) lymph nodes. These tend to grow quite slowly and are not usually painful. In most cases, the enlarged lymph nodes are only in one area of the body.

Some people with lymphoma have other symptoms such as weight loss, sweating at night and a high temperature (fever) that comes and goes. These are called B symptoms. They can happen with NLPHL, but only in a small number of cases – about 1 in 6 at most.

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Diagnosis and staging

Like all lymphomas, the best way to get a diagnosis is for your doctor to take a sample of cells from an enlarged lymph node and examine them under a microscope. This means having a small operation, called a biopsy

Special tests will also be done on the cells to look for particular proteins that are commonly found on the surface of lymphoma cells. It may take a while to have all the necessary tests done on your biopsy. The sample will be sent to a specialised laboratory where doctors who are experienced in diagnosing lymphoma can examine it.

Once you have been diagnosed, you will need other tests to find out which areas of your body the lymphoma is growing in - this is called 'staging'. You will have blood tests and scans. Doctors take blood tests to:

  • check your blood cell counts and make sure you’re not anaemic
  • make sure your kidneys and liver are working well
  • rule out infections that could flare up when you have treatment, such as hepatitis.

You are most likely to have a CT scan. This is to look for any signs of lymphoma in internal lymph nodes and body organs such as your liver and spleen. Or you may have an MRI scan. This uses magnetism instead of X-rays and may be used in children because it does not involve any radiation.

PET scans are sometimes used to diagnose lymphoma. They are useful because they can help tell the difference between active lymphoma and scar tissue. So they can help doctors to say whether enlarged lymph nodes deep in the body contain lymphoma cells without needing to remove them.

You usually have your tests done as an outpatient. It will take a few weeks to get all the results back. Understandably, waiting for test results can be a worrying time. It is important for your doctor to gather all of this information to find out what stage the lymphoma is at and to plan the best treatment for you.

Around 3 out of 4 cases of NLPHL (75%) are stage 1 or 2 when they are diagnosed. This is called early stage disease.

Stage 3 and 4 NLPHL is called advanced disease. About 1 in 4 people (25%) have advanced NLPHL when they are diagnosed. Try not to worry if you are one of these people. The main difference is that you will have treatment to your whole body rather than treatment to one area only. Advanced NLPHL still responds very well to treatment.

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Outlook

The outlook for NLPHL is generally very good. This is partly because it tends to be at an earlier stage when it is diagnosed and also because it grows very slowly. It tends to respond very well to treatment and most people are either cured or have a long time with no sign of the disease (known as a remission). Although NLPHL can come back (relapse), treatment is often successful and patients can be cured or have another long remission.

It is hard to give exact figures for cure rates – there is less data to go on than for other types of cancer. Statistics are also very general. They apply to a group of people and not any one person in particular. 

The best person to talk to about the likely outcome of your treatment is your own lymphoma specialist. While they won’t be able to give you any guarantees ahead of time, they will have access to all your test results and treatment plan so will be able to give you a more informed opinion.

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Treatment

Like other types of lymphoma, the treatment you have depends on:

  • how far the disease has grown – the stage of your disease
  • the size of your lymph nodes
  • whether you have B symptoms – weight loss, night sweats and fevers
  • your age, fitness and general health
  • your feelings about treatment and issues that may be important to you in the future, such as having children.

Your doctor will also think about the long-term effects of any treatment you have. This is important because treatment for NLPHL is very successful so most patients live for many years, even if the lymphoma comes back. Your medical team should give you information about the possible side effects and late effects (side effects that develop long after treatment) of your planned treatment.

Through research, treatment for many people with NLPHL has become less intensive than it used to be. Doctors and researchers continue to try and find treatments that have as few side effects as possible, particularly long-term side effects. These are rare, but for a small number of people, they can cause more health problems than the lymphoma itself, especially when the lymphoma is very slow-growing.

Early stage NLPHL without B symptoms

If you have early stage NLPHL you are most likely to have one of the following:

  • surgery to remove your lymph nodes
  • radiotherapy
  • surgery followed by radiotherapy.

It is most usual to have the all the lymph nodes containing lymphoma removed and then to have radiotherapy to the area. The type of surgery, time you spend in hospital and time it takes to recover from your surgery will depend on where in the body the lymph nodes are and how many you need to have taken out.

There has been some research looking into having only surgery and then watching closely for any signs that the lymphoma has come back. This is called ‘watch and wait’. It means that you avoid the side effects of radiotherapy.

You can only have ‘watch and wait’ if there are no signs of lymphoma after your surgery. There is a chance that the lymphoma will come back in the future, but then you can have more treatment and this is nearly always successful. Some research has been done to show that there is no difference in survival rates in children who had watch and wait compared to those who had radiotherapy after their surgery.

You usually have radiotherapy over three to four weeks. You have a small dose daily, from Monday to Friday each week. Having treatment is similar to having an X-ray. It doesn’t hurt and only takes a few minutes. 

Early stage NLPHL with B symptoms

If you have early stage NLPHL with B symptoms, you may need more intensive treatment. Doctors generally recommend that you have treatment with a combination of chemotherapy drugs. This is the same as the treatment they give for stage 3 and 4 NLPHL, which is outlined below.

Advanced stage NLPHL

If you have advanced-stage (stage 3 or 4) NLPHL but are well, you may have ‘watch and wait’.

If you have symptoms, you are most likely to have treatment with a combination of chemotherapy drugs. You may have:

  • R-CHOP
  • ABVD or R-ABVD
  • CVP or R-CVP

The R in each of these combinations stands for rituximab. This is an antibody treatment. These target particular proteins on the surface of cancer cells. Rituximab targets CD20, which is found on the surface of NLPHL cells. The antibody sticks to all the CD20 protein it finds, which marks them out for the immune system to then find and kill them.

R-CHOP stands for:

  • R – rituximab
  • C – cyclophosphamide
  • H – doxorubicin (also called hydroxydaunorubicin or doxorubicin hydrochloride)
  • O – vincristine (also called Oncovin®)
  • P – prednisolone

Cyclophosphamide, doxorubicin and vincristine are all chemotherapy drugs. Prednisolone is a steroid tablet.

You have prednisolone as a tablet. All the other drugs you have through a drip into a vein (intravenously). You usually have this treatment in several cycles, each lasting three weeks. You have the rituximab and chemotherapy on the first day of each cycle. You take prednisolone tablets for the next five days and then you have a break with no treatment for just over two weeks.

Three weeks after your treatment started, the second cycle begins and you have chemotherapy and rituximab injections again. For NLPHL, you usually have six cycles, so the treatment lasts for about 18 weeks in total.

CVP is a chemotherapy combination often used for children with NLPHL. It stands for:

  • C –  cyclophosphamide
  • V –  vincristine
  • P –  prednisolone

You have this treatment in three-weekly cycles. You have cyclophosphamide and vincristine intravenously on the first day of each cycle. You then take prednisolone tablets for the next five days. After a break for just over two weeks, the cycle begins again. 

You may have CVP along with rituximab. This is called R-CVP.

ABVD is another chemotherapy combination you may have for NLPHL. It stands for:

  • A – doxorubicin (Adriamycin)
  • B – bleomycin
  • V – vinblastine
  • D – dacarbazine

All these drugs are chemotherapy drugs that you have intravenously. You usually have several cycles of treatment, each lasting four weeks. You have all the drugs together twice in each cycle, on the first day and the 15th day. 

ABVD is used less often on its own these days. But you may have it along with rituximab, which is called R-ABVD.

People who are not well enough to have chemotherapy might have rituximab on its own.

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Follow-up

You will have regular follow-up appointments with your specialist after you finish your treatment. At first, these will be every three months or so. But if all is well, they will gradually become less frequent.

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Relapse 

Unfortunately, NLPHL can sometimes come back (relapse). This may be many years after you were first treated and is one reason why it is so important to keep your follow-up appointments. Fortunately NLPHL usually responds just as well to treatment as it did the first time round and many people will be cured.

NLPHL usually comes back in the same place and rarely becomes more widespread. Your doctor will want to take another biopsy before deciding on treatment.

It is important to check the lymphoma type again because, in very rare cases, NLPHL can come back as a type of fast growing non-Hodgkin lymphoma. Doctors call this ‘transformation’. In one study of 95 patients, NLPHL transformed in around 1 in 6 people. In another study, it transformed in around 1 in 12 people.

If your NLPHL comes back, you may have:

  • radiotherapy
  • chemotherapy – usually with different drugs if you had chemotherapy before
  • rituximab, either on its own or with chemotherapy
  • high dose chemotherapy with a stem cell transplant – you will only usually have this if your NLPHL has transformed into a fast-growing type of lymphoma.

Your doctor will decide on your treatment by taking into account:

  • the treatment you had before and how well you coped with it
  • how long it was before your NLPHL came back
  • the result of your biopsy and tests (the type and stage of the lymphoma that has come back)
  • your age, fitness and general health.

You are bound to be upset that your lymphoma has come back. But do remember that it can often be treated successfully. You are likely to get back into remission and may well be cured.

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Research and targeted treatments

NLPHL treatment is often very successful. Doctors continue to try and find treatments that can still cure the lymphoma but cause fewer side effects, particularly long-term side effects.

Other clinical trials are looking at treatment for NLPHL that has come back (relapsed) or hasn’t responded very well to treatment. Drugs being investigated include:

It is too early to say whether any of these treatments will work for NLPHL or whether they will work better than the treatments doctors already use. Your doctors may offer you the chance to take part in a clinical trial, if there is one suitable for you. You can find out more about clinical trials and search for a trial that might be suitable for you at Lymphoma TrialsLink.

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