About 9 out of 10 children, and 7 out of 10 adults with Lymphoblastic Lymphoma can be cured after their first line of treatments.
Others may need more than one line of treatment to achieve a cure or remission.
What are Lymphoblasts?
Lymphoblastic Lymphoma (LL) is a rare aggressive cancer of white blood cells called lymphoblast cells, so to understand Lymphoblastic Lymphoma, you need to know a bit about lymphoblasts.
How are lymphoblasts made?
Lymphoblasts are made from stem cells in our bone marrow.
Stem cells
Stem cells are the most immature, but cleverest cells we have. They are able to get messages from different chemicals in our body and then develop into any type of cell we need.
Haematopoietic stem cells can become any type of blood cell, including red blood cells, white blood cells or platelets. These stem cells are in our bone marrow, and once they know what blood cell to become, they develop into blast cells before they mature into fully grown cells.
Haematopoietic stem cells can develop into T-cell or B-cell lymphoblast cells in our bone marrow, and then move out into our lymphatic system, thymus and spleen where they continue to develop and become mature B-cell lymphocytes, or T-cell lymphocytes.
Lymphoblasts
Lymphoblasts are a type of white blood cell in its most immature form – before it fully develops into a lymphocyte cell. Lymphocytes are an important part of our immune system that help to fight infection and disease, and also make sure our immune system does not become overactive.
Lymphoblastic Lymphoma starts when cancerous changes happen to lymphoblast cells, before they mature into lymphocytes. Because of this, you may not have enough mature lymphocytes to fight infection and disease.
Lymphocytes
Click on the headings below to learn more about B-cell and T-cell lymphocytes.
About B-Cell lymphocytes
- Are a type of white blood cell.
- Fight infection and diseases to keep us healthy.
- Memory B-cell lymphocytes remember infections we had in the past, so if we get the same infection again, our body’s immune system can fight it more effectively and quickly.
- Are made in our bone marrow (the spongy part in the middle of our bones), but usually live in our spleen and lymph nodes. Some live in our thymus and blood too.
- Can travel through our lymphatic system, to any part of our body to fight infection or disease.
- The most mature type of B-cell lymphocytes are called Plasma cells, and these cells make antibodies that are specific to each type of antigen. Antigen is a word used to describe anything that may make us sick including germs, infections, pollen or other allergens, diseased or damaged cells. Antibodies attach to the antigen to let our immune system it needs to come and fight the antigen.
About T-cell Lymphocytes
- T-cells are made in our bone marrow, mature in our thymus, but can live in any part of our lymphatic system – including our lymph nodes.
- Most T-cells need to be activated to work effectively. They are usually resting in our thymus or other parts of our lymphatic system, and only wake up and fight infection when other immune cells let them know there is an infection or disease to fight. When they wake up, T-cells can travel to any part of our body to fight the infection or disease.
- Some T-cells are responsible for “regulating” the immune response. This means that once an infection has been destroyed, the Regulatory T-cells tell other immune cells to “stand down” so they don’t keep fighting and cause harm to our good cells.
- Natural Killer (NK) cells are often called “NK T-cells”. They are specialised immune cells that recognise and destroy cancer cells. Unlike other T-cells, NK cells do not need to be activated to fight cancer. They are ready to fight at all times, and actively move around our body on the look-out for cells that have cancerous changes.
- After fighting an infection or disease, some T-cells become “Memory T-cells”. They remember everything there is to know about the infection and how to fight it. That way, if we ever get the same infection or disease again, our immune system can fight it a lot more quickly and effectively.
- Some T-cells help other immune cells, such as B-cell lymphocytes to work effectively. These T-cells are called “Helper T-cells”.
Difference between Lymphoblastic Lymphoma and Lymphoblastic leukemia
Lymphoblastic Lymphoma is very closely related to Lymphoblastic Leukemia. The table below provides a summary of the main differences between the two cancers.
Lymphoblastic LYMPHOMA ↓ | Lymphoblastic LEUKEMIA ↓ | |
Most commonly is cancer of | T-cell Lymphoblasts | B-cell Lymphoblasts |
Rarely is cancer of | B-cell Lymphoblasts | T-cell Lymphoblasts |
Cancerous cells mostly IN | Lymphatic system, skin or other organs | Bone marrow and blood stream |
Symptoms of Lymphoblastic Lymphoma
Because your lymphoblasts become cancerous in Lymphoblastic Lymphoma, you will have less functioning lymphocytes.
- When you don’t have enough healthy lymphocytes, you can get lots of infections or have difficulty getting over infections.
- Too many cancerous lymphoblastic cells in your bone marrow can take up too much room, so your bone marrow may not be able to make other blood cells such as red blood cells, other white blood cells or platelets. A blood test may show that you have low levels of these blood cells.
- When the cancerous lymphoblasts are in your lymphatic system such as your lymph nodes or organs, they may swell up and look or feel like a lump under your skin.
- Lymphoblastic Lymphoma in your skin may give you a rash that does not go away, and may or may not be painful or itchy.
Other symptoms of Lymphoblastic Lymphoma may include general symptoms of lymphoma or be specific to the area of your body the lymphoma is growing. You may also have different symptoms depending on whether your lymphoma is T-cell or B-cell lymphoma.
Click on the heading below if you know if your Lymphoblastic Lymphoma is T-cell or B-cell for more specific symptoms you may get.
B-cell Lymphoblastic Leukemia is more common than B-cell Lymphoblastic Lymphoma
B-cell lymphoblasts usually mature into B-cell lymphocytes in your bone marrow before moving into your lymphatic system, including your lymph nodes, spleen, and a small number into your thymus.
For this reason, most people with a B-cell Lymphoblastic cancer will develop B-cell Lymphoblastic Leukemia (where 1 in 4 cells in your bone marrow are cancerous), rather than B-cell Lymphoblastic Lymphoma.
Lymphoblastic Lymphoma
However, rarely the cancerous B-cell Lymphoblastic cells can travel you your lymph nodes, bones, skin, spleen or other areas. When this happens, you have less cancerous B-cells in your blood and bone marrow, and more in your lymphatic system or other parts of your body. When this happens, you will have B-cell Lymphoblastic Lymphoma.
The symptoms you may have with this subtype of lymphoma include:
- Lots of infections or difficulty getting over them.
- Swollen lymph nodes – you may notice these more in your neck, armpits or groin as these lymph nodes are closer to your skin.
- Bone pain, or bones that break easier than they should.
- Swollen (or enlarged) organs such as of your liver or spleen. A swollen liver is called hepatomegaly, and a swollen spleen is called splenomegaly. When they are both swollen it is called hepatosplenomegaly.
- Hepatomegaly can cause pain in your abdomen or chest, yellowing of your skin or eyes, weight loss, pain when you drink alcohol, bruising or bleeding more than usual, or blood clots.
- Splenomegaly can cause pain in your chest or abdomen, make you feel full even when you haven’t eaten, or have eaten very little, anemia (which is low levels of haemoglobin and red blood cells), weight loss, fatigue or easy bleeding and bruising.
- A rash, lumps or sores on your skin that don’t heal or go away.
- Low red blood cells, white blood cells and/or platelets in your blood tests.
- Pale skin (lighter than your usual colour).
- B-symptoms (see image below).
Most T-cell lymphoblasts travel to and live in our thymus before maturing into T-cell lymphocytes. Our thymus is a butterfly shaped organ in the middle of our chest behind our sternum (the bone that runs down the middle of our chest).
For this reason, many T-cell Lymphoblastic Lymphomas start in the thymus or in the lymph nodes of your chest. As a result, your thymus or lymph nodes can grow much bigger than they should as they become full of lymphoma cells.
However, T-cells also often travel to the layers of our skin, and other parts of our body too, so you can have T-cell Lymphoblastic Lymphoma in your skin or other organs to. Common symptoms can include:
- Discomfort, pain or pressure in your chest.
- Shortness of breath or changes to how your breathing sounds.
- A noticeable lump in your chest that you can see, feel or that shows on a CT scan or X-ray.
- Changes to your voice.
- Fluid build-up in the lining around your lungs (pleural effusion, also called a PE), making it painful or difficult to breath.
- Fluid build-up in the lining around your heart (pericardial effusion) which can cause chest pain, or changes to how your heart beats.
- Skin rashes, sores or lumps that don’t improve within 2 weeks.
- Superior vena cava syndrome. This is where the lymphoma starts to put pressure on your blood vessels, obstructing blood flow. You may notice veins sticking out or swelling more than usual in your chest, arms, neck and head. You may also have a cough or trouble breathing.
- B-symptoms (see image below).
General symptoms of lymphoma
As well as the symptoms listed above you may get any of the more general symptoms of lymphoma. Click on the pictures below to link to our Symptoms of Lymphoma webpage.
How is Lymphoblastic Lymphoma diagnosed?
To diagnose any type of lymphoma you will need a biopsy. There are several different types of biopsies used to diagnose lymphoma in different parts of your body. The main types of biopsies you may have if your doctor suspects you have Lymphoblastic Lymphoma include:
- Core biopsy of an affected lymph node.
- Skin biopsy of any affected skin.
- Excisional biopsy to remove an entire lymph node.
- Bone marrow biopsy to check if you have lymphoma cells in your bone marrow.
- Other biopsies of affected organs (such as thymus, liver or spleen) during surgery.
Staging
After a diagnosis is made, you will need more tests to stage your lymphoma. Staging tests check to see how much of your body is affected by the lymphoma. It also looks at where the lymphoma is, and helps your doctor to work out what the best type of treatment will be. Lymphoma is staged using the numbers 1 to 4. Staging tests may include:
- A bone marrow biopsy if you haven’t already had one.
- Blood tests.
- PET scan.
- CT scan.
Early-stage lymphoma
Stage 1 and 2 lymphoma means that your lymphoma is an early stage and only affecting one or two areas of your body. If you have 2 areas affected, they will be both be either above, or both below your diaphragm.
Advanced stage lymphoma
Stage 3 and 4 lymphoma are considered advanced stages and mean that you have lymphoma both above and below your diaphragm. In stage 4 you will also have lymphoma in 1 or more of your organs, such as your bones, skin, lung, liver or spleen.
Many advanced stage lymphomas can still be cured or put into remission or be cured.
What causes Lymphoblastic Lymphoma?
We don’t know what causes most lymphomas. It is not infectious so you cannot pass it on to someone else, and it is not related to lifestyle factors such as your weight and activity levels, alcohol or smoking.
There are some things that may increase your risk of getting Lymphoblastic Lymphoma, but most people with the same risk factors never develop lymphoma, and others with none of the risk factors sometimes do get lymphoma.
Some risk factors may include if you have, or have had a:
- previous infection with Epstein-Barr virus (EBV) – this virus causes glandular fever (also called mono or the kissing disease).
- weakened immune system due to an inherited immune deficiency disease (autoimmune disease).
- infections with human immunodeficiency virus (HIV).
- immunosuppressant medication that is taken to prevent you rejecting an organ or stem cell transplant.
- if you have a brother or sister with Hodgkin Lymphoma (especially if they are your twin). However, this is a very slight increased risk and in most cases, there is no need for any genetic testing. Most people with Lymphoblastic Lymphoma have no family history.
Before treatment for Lymphoblastic Lymphoma
Multidisciplinary care
In many cases, your haematologist will meet with other haematologists and other health professionals to discuss your case. This is a great thing when you have a rare lymphoma because you get many different, very experienced people looking at your individual situation to determine the best way to help you, and make sure you get best the best treatment options and care.
You can also ask your haematologist to present your case at the Australasian Lymphoma Alliance meeting. This meeting brings together experts from around the country who are up to date with the latest research developments in rare lymphomas. They can advise your haematologist of any clinical trials or new treatment options available to you.
Whether you are male or female, many treatments can affect your fertility, making it more difficult or impossible to get pregnant, or get somebody else pregnant. If you are of child-bearing age (or younger), even if you are not thinking about having a family or extending your family just yet, you need to talk to your doctor about how to preserve your fertility, prevent unwanted pregnancies and prevent or manage early menopause or ovarian insufficiency.
When you are first diagnosed, or starting treatment after a relapse, it can be difficult to think straight and know what questions to ask. But it’s important you have all the right information you need to make the decisions about your healthcare and know what to expect. Click on the link below to download Questions to ask your doctor.
Having treatment is going to change your life and the lives of those closest to you for a while. It is important to know what to expect and prepare for. You also need to know what support is available to you. We have developed some tips on practical things you should know when you or a loved one has lymphoma. Click the link below for more information.
Starting treatment
The doctors will take into consideration many factors about the lymphoma and your general health to decide when and what treatment is best for you. They will consider:
- The subtype of Lymphoblastic Lymphoma you have.
- Whether your lymphoma is mostly in your blood and bone marrow, or lymphatic system and other parts of your body.
- Any symptoms you have.
- Your age, past medical history, physical and emotional wellbeing.
- Whether you have ever had treatment for the Lymphoblastic Lymphoma in the past.
- Your eligibility to join a clinical trial.
- Your personal preferences after you’ve been given all the information you need to make a decision.
Treatment protocols
Treatment protocols are another of way of saying what your treatment plan is. Because of the similarity between Lymphoblastic Lymphoma and Lymphoblastic Leukemia, you may have treatments similar to people with Lymphoblastic Leukemia, or other subtypes of lymphoma. This will depend on where your lymphoma is mostly growing and how it is affecting your body.
Your treatment protocol may include one or more of the following:
- Radiation alone or with chemotherapy and other treatments
- Surgery to remove the whole lymphoma if it is limited to a small area.
- Medicine such as chemotherapy with or without other medicines called monoclonal antibodies.
- Stem-cell transplant.
- Clinical trial.
Your treatment protocol may include an induction, consolidation and/or maintenance phase to get you into, and keep you in remission or to increase your chance of cure.
Induction treatments usually last several months however, maintenance treatment can continue for about 2 years.
Common chemotherapy protocols
Most people with Lymphoblastic lymphoma will need chemotherapy.
ALL06 is given in 3 phases and is given to young people with either Lymphoblastic Leukemia or Lymphoblastic Lymphoma. The 3 phases include induction, consolidation and maintenance. Click on the links for more information on each phase.
Hyper CVAD and dasatinib given with alternating cycles of Part A and Part B, then maintenance.
CHOP (cyclophosphamide, doxorubicin, vincristine and a steroid called prednisolone).
Finishing treatment
Finishing treatment can be exciting, or a very anxious time for you. It could even make you feel both excited and anxious. It is normal to feel many different emotions when you finish treatment. And, even if you coped well during treatment, you may find that once everything slows down and you have time to think about what you’ve been through, and how to get back to some kind of life that resembles life before treatment, you become overwhelmed.
You also have to deal with the emotions of others around you and their expectations. Many patients mention how hard it is when people around them expect them to be back to normal long before they are ready.
On the other hand, other patients have mentioned feeling like people around them hold on too tight and don’t let them get back to normal, because they are afraid something else might happen.
We have tips on what to expect, and how to manage emotions, and the expectations of other on our Finishing treatment webpage.
Follow up
You will continue to see your haematologist or oncologist regularly even after treatment ends. They will assess you for any ongoing side-effects from treatment, or any new side-effects that can happen months or even years after treatment ends. You can find more information about late effects by clicking the side effects of treatment link above.
They will also be assessing you signs for any signs of your lymphoma relapsing so that any needed treatment can start as soon as possible.
Your follow-up appointments may include any of the below:
- PET or CT scans and results.
- Blood tests.
- Physical examination of your lymph nodes.
- Questions about how you are feeling, and adapting to life after treatment.
- Referrals for extra care if needed.
Treatment under investigation for lymphoblastic lymphoma (LL)
There are many treatments that are currently being tested in clinical trials in Australia and around the world for patients with both newly diagnosed and relapsed lymphoma. For a list of the current treatments that are being investigated include:
- Chimeric antigen receptor T-cell therapy (CAR-T-cell therapy)
- Blinatumomab
Summary
- Lymphoblastic Lymphoma is the 2nd most common subtype of lymphoma in children.
- Anyone can develop Lymphoblastic Lymphoma including older adults, but it is more common in children and young adults about 30 years of age.
- There are two main subtypes including B-cell Lymphoblastic Lymphoma and T-cell Lymphoblastic Lymphoma.
- Lymphoblastic Lymphoma is an aggressive lymphoma needing treatment soon after diagnosis. However, most people will be cured after their first line of treatment.
- Symptoms may be related to general symptoms of lymphoma, or specific to the area of your body the lymphoma is growing. Report all new and worsening symptoms to your doctor.
- Ask if you are eligible to join a clinical trial if you are newly diagnosed with Lymphoblastic Lymphoma, or it relapses after and time of remission.
- Finishing treatment is exciting, but can come with challenges. Make sure you visit our Finishing treatment page to find out what to expect and how to prepare for life after treatment.
