Have you ever heard of Anaplastic Large Cell Lymphoma (ALCL)? Maybe this name sounds a little complicated. But don't worry, this is a very rare type of cancer. Today we will talk about what ALCL is, how it develops, what are the symptoms, what are the treatments, and many other things in a very simple way that you can understand. Just like talking to a friend.
What is ALCL (Anaplastic Large Cell Lymphoma)?
Simply put, ALCL is a rare, uncommon type of cancer that belongs to a larger group of cancers called Non-Hodgkin Lymphoma . Did you know that like all Non-Hodgkin Lymphoma, ALCL is also a type of cancer? It occurs when a type of white blood cell called lymphocytes in our body grows out of control and at a faster rate than it should.
Think of these lymphocytes as little soldiers in our bodies. They are part of our immune system, protecting us from germs and diseases. There are two main types of lymphocytes: B cells and T cells . In ALCL, your T lymphocytes, or T cells, grow abnormally.
Now let's see what this name "anaplastic large cell lymphoma" means.
- Anaplastic : This word means that when viewed under a microscope, these cancer cells look very strange and different compared to healthy T lymphocyte cells. It's as if their shape and appearance have been distorted.
- Large Cell : This means that the abnormally behaving T lymphocytes appear larger than normal T cells. This difference is clearly visible under a microscope.
- Lymphoma : Lymphoma is a cancer that occurs due to the uncontrolled growth of white blood cells called lymphocytes. ALCL develops specifically from T lymphocytes.
Got it? Simply put, ALCL is a cancer in which T cells become abnormally large, mutated, and grow uncontrollably.
Are there different types of ALCL?
Yes, there are different types of ALCL. These are classified based on the location of the cancer in your body and the characteristics of the cancer cells.
Systemic ALCL
This is the main type. "Systemic" means it can affect the whole body. It can affect your lymph nodes , or "glands" as we call them, and internal organs like your liver and lungs, and sometimes your skin.
Systemic ALCL is divided into two types. One is based on whether or not there is a mutation in a gene called the ``Anaplastic Lymphoma Kinase (ALK)'' gene . Genes are like little instruction books for our cells, and these genes tell even a T lymphocyte how to function.
- ALK-positive ALCL : This is a type of cancer that has a mutation in the ALK gene. This is often an aggressive cancer. However, it is more common in children and young adults . Fortunately, this type responds well to chemotherapy .
- ALK-negative ALCL : This type does not have the ALK gene mutation. This is also a fast-growing (aggressive) cancer. However, it mostly affects older people , usually those over 60 years of age. This type may go into remission after chemotherapy and then recur. Therefore, ALK-negative ALCL is more difficult to treat, and the prognosis is worse than ALK-positive ALCL.
Other types of ALCL
There are also types of ALCL that grow more slowly than the systemic types.
- Primary cutaneous ALCL : This is a type that mainly affects the skin. Symptoms such as blisters, bumps, and eczema may occur. In most cases (about 90%) it does not spread beyond the skin.
- Breast implant-associated ALCL (BIA-ALCL) : This is a type of ALCL that develops around breast implants. It is usually diagnosed about 10 years after a breast augmentation or breast reconstruction procedure.
Who gets ALCL?
Although ALCL can develop in people of any age, some types affect certain groups more.
- ALK-positive ALCL : This most commonly affects late preschool-aged children, teenagers, and young adults in their 20s and 30s . It is more common in men .
- ALK-negative ALCL : This mainly affects people over the age of 60. It is also slightly more common in men.
- Primary cutaneous ALCL : This most commonly occurs in adults over the age of 40. It is more common in men and white people.
- BIA-ALCL : This is usually diagnosed in people in their 50s . It can develop in people who have had both silicone and saline breast implants. It has been found to be particularly associated with textured breast implants .
How common is ALCL?
ALCL is actually a very rare cancer. Only about 2% of non-Hodgkin lymphomas in adults are diagnosed as ALCL. That's a very small percentage.
What are the symptoms of ALCL?
The symptoms of ALCL vary depending on the type.
Symptoms of systemic ALCL
In both ALK-positive and ALK-negative ALCL, swollen lymph nodes are common where the cancer is growing. They may feel like lumps in places like your neck, armpits, or groin . Other symptoms include:
- Frequent fever.
- Feeling very tired (`Fatigue`).
- Sweating at night (`Night sweats`).
- Unexplained weight loss.
When most people are diagnosed, the cancer may have spread a little, meaning it is in an `advanced stage` . In that case, the cancer may have spread to organs such as your lungs, liver, or bones. Then, symptoms may occur depending on the organ that is affected. For example, if you have chest tightness or a frequent cough, the ALCL may have affected the chest.
Characteristics of primary cutaneous ALCL
Skin changes can be seen in this type.
- Unusual red or reddish-brown bumps appear on one or more areas of the skin, which may grow larger over time.
- Raised lumps , larger than a coin. These can sometimes be itchy.
- The bumps may become sore and then develop into a scab .
Characteristics of BIA-ALCL (Breast Implant-Associated ALCL)
In this, clear changes in the breasts can be seen.
- Breast pain.
- Breast swelling.
- A skin rash or eczema.
- Swelling or fluid accumulation near your breast implant.
- A solid mass near the breast implant.
These changes are usually seen in one breast, but sometimes changes can be seen in both breasts.
What causes ALCL?
ALCL occurs when the lymphocyte cells we talked about earlier multiply uncontrollably and start to grow beyond the healthy tissue around them. Researchers still don't know exactly why these lymphocyte cells become cancerous (malignant cells).
However, they have identified several genetic mutations that are commonly seen with ALCL. It is thought that these genetic mutations can cause healthy cells to transform into cancer cells.
For example, the main characteristic of ALK-positive ALCL is mutations in the ALK gene . Similarly, other gene mutations are also common in other types of ALCL.
How is ALCL diagnosed? (Diagnosis)
Your doctor will first do a physical exam to check for signs of ALCL, such as swollen lymph nodes. If they suspect this, they will do several different tests and procedures.
- Imaging procedures : These can help identify the location of the cancer. The type of imaging tests you need will depend on the type of ALCL you have. Your doctor may do a chest X-ray , CT scan , or MRI to look for tumors. A PET/CT scan or PET scan can help determine if the cancer has spread. If BIA-ALCL is suspected, a breast ultrasound may be done.
- Bloodwork : The doctor will do various blood tests, such as a complete blood count (CBC), to check for signs of ALCL. If the number of blood cells (red blood cells, white blood cells, or platelets) is abnormal, it may be a sign of ALCL. They will also look for certain enzymes and other markers in the blood, as these can also be signs of ALCL.
- Biopsy : The only way to confirm a diagnosis of ALCL is to have a biopsy. In a biopsy, the doctor takes a small sample of suspicious tissue and examines it under a microscope. By studying these cells, the doctor can determine exactly what type of ALCL you have. This also helps to plan the most effective treatment.
How is ALCL treated?
The usual treatment for systemic ALCL is chemotherapy . Surgery is the preferred treatment for primary cutaneous ALCL and BIA-ALCL.
Treatment of systemic ALCL
ALK-positive and ALK-negative anaplastic large cell lymphomas usually respond well to systemic chemotherapy. Systemic chemotherapy involves giving cancer-killing drugs through a vein, which travel throughout the body through the bloodstream to kill cancer cells.
Chemotherapy for systemic ALCL often uses a combination of drugs that work together to kill cancer cells. Some examples include:
- BV-CHP : This includes the drugs `Brentuximab Vedotin`, `Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, and `Prednisone`.
- CHOP : This involves the use of drugs called `Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, `Vincristine (Oncovin®)`, and `Prednisone`.
- CHEOP : This involves the use of drugs called `Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, `Etoposide`, `Vincristine (Oncovin®)`, and `Prednisone`.
These treatments can usually make all signs of cancer go away for a short time (we call this `remission` ). However, the cancer can come back. If your doctor thinks that the cancer is likely to come back, they may recommend a `stem cell transplant` while you are in remission. A stem cell transplant replaces the cells that have been destroyed by chemotherapy with healthy cells. These healthy cells then produce healthy (non-cancerous) blood cells.
If the cancer comes back, your doctor may recommend various additional chemotherapy treatments designed to treat lymphoma.
Treatment of primary cutaneous ALCL
The most common treatment for this is surgery . If the cancer has spread to the lymph nodes, you may also need radiation therapy . Radiation therapy works by directing energy at the cancer cells and destroying them.
Primary cutaneous ALCL can often recur within five years of treatment. If this happens, surgery and radiation therapy will be required again.
If you have localized tumors that cannot be removed with surgery, or if the cancer keeps coming back, you may need other treatments. These may include:
- Oral Methotrexate : A drug used to treat non-Hodgkin lymphoma.
- Bexarotene : A drug used to treat T-cell lymphoma affecting the skin.
- Brentuximab Vedotin : A drug used to treat systemic ALCL and other types of recurrent ALCL.
- Interferon : A drug that helps your immune system recognize and fight cancer cells, including lymphoma.
Treatment of BIA-ALCL
BIA-ALCL can usually be cured with surgery to remove your breast implant and any cancer cells around it. If surgery is not possible, radiation therapy can be given to kill the cancer cells.
If the cancer comes back or spreads beyond the breast, your doctor may recommend chemotherapy treatments used to treat ALK-negative ALCL. These may include CHOP chemotherapy and treatments such as Brentuximab Vedotin.
How serious is ALCL?
Regardless of the type, ALCL is a serious cancer that requires careful monitoring and appropriate treatment. However, your prognosis and treatment plan depend on factors such as the type of ALCL you have, how you respond to treatment, and your International Prognostic Index (IPI) score.
The IPI was developed by oncologists to help predict the outcome of fast-growing non-Hodgkin lymphomas, including systemic ALCL. The score is based on the following factors:
- your age.
- Your ability to perform daily tasks.
- How far your cancer has spread (cancer stage).
- The number of sebaceous glands affected.
If you have systemic ALCL, ask your doctor how your IPI score affects your chances of recovery.
What is the survival rate for ALCL?
Survival rates for systemic ALCL vary widely. ALK-positive ALCL is associated with a longer survival time than ALK-negative ALCL. Depending on factors such as your cancer type and IPI score, the five-year survival rate for ALK-positive ALCL can range from 33% to 90%. Similarly, the five-year survival rate for ALK-negative ALCL can range from 13% to 74%.
Primary cutaneous ALCL and BIA-ALCL have very good survival rates. Although primary cutaneous ALCL usually goes into remission and comes back within five years, follow-up treatments can extend your life. The survival rate is about 80% five years after diagnosis. In BIA-ALCL, if the cancer is completely removed with surgery, the cancer is often completely gone.
What questions should I ask my doctor?
Here are some important questions to ask your doctor:
- What type of ALCL do I have?
- Is my cancer confined to one spot, or has it spread?
- Who are the specialists on my care team?
- What treatment do you recommend for me?
- How do I manage the side effects of treatment?
- What is my IPI score?
- What are the chances of my cancer going into `remission`?
- What are the chances that my cancer will come back?
- How often do I need to come for follow-up visits to monitor my condition?
It's normal to feel scared when you learn you have cancer. However, the most important thing to remember is that ALCL is not a single type of cancer with the same outcome . The type of ALCL you have determines whether it spreads quickly or slowly. That will shape your treatment options and outlook. Some people's ALCL spreads quickly. For others, it grows slowly, giving you enough time to get treatments that can help control the cancer. Talk to your doctor about the possible outcomes based on your diagnosis.
The most important things you need to remember (Take-Home Message)
Okay, so let's summarize some of the most important things you need to remember from what we've talked about.
- ALCL is a rare type of cancer, and there are different types of it.
- Not all types of ALCL are the same. Some spread quickly, some slowly.
- Treatment and chances of recovery vary depending on the type of ALCL you have, your age, and your overall health.
- Although ALK-positive ALCL occurs more frequently in younger people, it responds well to treatment.
- ALK-negative ALCL affects more adults and can be a bit more complicated to treat.
- The prognosis for cutaneous ALCL and breast implant-associated ALCL (BIA-ALCL) is generally good.
- Don't panic. If you have any doubts or symptoms, seek medical advice immediately.
- Talk openly with your doctor about your condition, your treatment, and any questions you may have. They are the ones who can best help you.
Remember, not all cancer diagnoses are equally serious. Even with ALCL, with an accurate diagnosis, prompt treatment, and good monitoring, many people can achieve successful outcomes.
` Anaplastic Large Cell Lymphoma, ALCL, Lymphoma, Cancer, T Cells, ALK Gene, Chemotherapy
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