Anaplastic Large Cell Lymphoma: Causes, Symptoms, Treatment

Anaplastic large cell lymphoma (ALCL) is a rare type of non-Hodgkin lymphoma (NHL) that primarily affects the lymphatic system. It commonly has the potential to proliferate quickly and also recur.

Large, aberrant cells known as anaplastic lymphoma kinase-positive (ALK-positive) or anaplastic lymphoma kinase-negative (ALK-negative) cells proliferate abnormally and are characteristic of the disease. Despite being more frequently diagnosed in young people, ALCL can affect both children and adults.

It has a markedly higher prevalence in the first three decades of life and a male predominance overall (male-to-female ratio of 1.5:1).

There are different types of anaplastic large-cell lymphomas, depending on the location of the cancer and the characteristics of the cancer cells. The type of ALCL also influences the treatment option available.

1. Systemic ALCL: This is the most common type of ALCL and primarily affects lymph nodes, but it can also involve other organs and tissues in the body. It is characterized by the presence of large abnormal cells called "anaplastic" cells, which can be identified under a microscope.

2. Cutaneous ALCL: This type of ALCL mainly involves the skin. It usually presents as red, raised skin lesions or tumors. Cutaneous ALCL is often less aggressive than systemic ALCL, and it has an excellent prognosis with a high survival rate.

3. Breast Implant-Associated ALCL: This is a rare form of ALCL that develops in the scar tissue surrounding breast implants. It is typically found in individuals who have undergone breast augmentation or reconstruction surgery. Early detection and removal of the implant are crucial for successful treatment and recovery.

Anaplastic Large Cell Lymphoma Stages

Anaplastic Large Cell Lymphoma (ALCL) can be staged to determine the extent of the disease and guide the appropriate treatment approach. The staging system commonly used for ALCL is the Ann Arbor staging system, which is also used for other types of non-Hodgkin lymphomas. Here are the stages of ALCL:

1. Stage I: The cancer is limited to a single lymph node region or a single extra lymphatic site.

2. Stage II: Cancer involves two or more lymph node regions on the same side of the diaphragm, or it involves a lymph node region and a nearby extra lymphatic organ or tissue.

3. Stage III: The cancer is present in lymph node regions on both sides of the diaphragm, with or without the involvement of nearby extra lymphatic organs or tissues.

4. Stage IV: The cancer has spread beyond the lymph nodes to distant organs or tissues, such as the liver, bone marrow, or lungs.

The symptoms of ALCL can vary depending on the subtype of the disease and the organs involved. Common symptoms of ALCL may include:

Systemic ALCL 

Both ALK-positive and ALK-negative kinds of anaplastic large-cell lymphoma can cause the lymph nodes to swell up. The most common areas are the underarm, neck, and groin.

Symptoms of this include 

1. Night sweats

2. Unexplained weight loss 

3. Fever

4. Tiredness 

When ALCL has spread to other organs like the liver, lungs or bones, symptoms that are related to that organ might persist. For example, if ALCL is present in the chest, people might experience pain and a persistent cough. 

Primary Cutaneous ALCL

As primary cutaneous ALCL usually affects the skin, it appears like rashes or bumps. The most common sites are the arms, face, legs, and abdomen. The symptoms to watch out for are:

1. Large raised bumps that are itchy

2. Reddish or brownish growths that grow over time

3. Growths that cause ulcers and develop scabs. 

Breast Implant-Associated ALCL

BIA ALCL symptoms are easy to observe, as they cause changes in the breast. Usually, one breast changes, but sometimes people can observe symptoms in both. 

1. Breast pain

2. Swelling of breast 

3. Rashes on the breast 

4. Swelling near the implant

5. Lump near the implant 

Currently, there is no exact cause of this condition. As per scientific evidence, it is known that anaplastic large cell lymphoma occurs when T cell lymphocytes (white blood cells) grow out of control, and start to affect the healthy tissues and cells nearby.

That being said, there are some genetic mutations that frequently occur with ALCL. These mutations often cause the healthy cells to become malignant. One such mutation is the ALK gene. This causes systemic ALK-positive ALCL.

Anaplastic Large Cell Lymphoma Risk Factors

The likelihood of getting anaplastic large cell lymphoma can increase in spite of the fact that the condition's origin is still difficult to determine. Among these risk factors are: 

1. Age: While ALCL can affect anyone at any age, it is more common in children and young people.

2. Gender: Males are slightly more likely than females to have ALCL.

3. Race: ALCL is more common in people of African American and Asian descent.

4. Family History: Those who have ALCL in their family are more likely to get the illness.

5. Exposure to Certain Chemicals: It has been established that exposure to specific chemicals, such as pesticides and herbicides, increases the risk of ALCL.

6. Certain Genetic Mutations: Some genetic variations have been connected to a higher risk of ALCL.

There is no way to prevent the occurrence of anaplastic large-cell lymphoma. However, one can undertake the following preventive measures to minimise the risk of developing ALCL

These preventive measures include:

1. Avoid Exposure to Certain Chemicals: If one works with chemicals that have been linked to ALCL, he/she must talk to their employer to know the ways to reduce the chemical exposure.

2. Live a Healthy Lifestyle: A balanced diet, frequent exercise, and giving up smoking can all help lower the chance of developing various cancers, including ALCL.

3. Regular Checkups: One must consult their doctor if they have any worries about their health. The chances of a positive outcome can be improved by early detection and treatment.

4. Get Vaccinated against Hepatitis B: The risk of ALCL may increase if one has hepatitis B, a viral illness. They can lower their risk of developing this virus by being vaccinated against hepatitis B.

5. Quit Smoking: Smoking increases the risk of many types of cancer, including ALCL. If one smokes, quitting can help reduce the risk.

6. Stress Management: Stress can weaken the immune system, making the individual more vulnerable to cancer. Find healthy ways to manage stress, such as exercise, relaxation techniques, or spending time with loved ones.

To diagnose anaplastic large cell lymphoma, doctors will usually perform a physical examination to check for signs, like swelling of the lymph nodes and also assess the patient’s medical history. The doctor will also prescribe various tests and procedures like:

1. Imaging Tests: The doctor prescribes imaging tests like MRIs, CT scans, and X-rays to identify the location of the cancer. A PET scan might also be prescribed to check if the cancer has spread to various parts of the body as well.

    In the case of BIA ALCL, a breast ultrasound is done. This ultrasound is done to check for fluid accumulation or lumps in the breasts. In case the ultrasound is not very clear, an MRI is suggested. 

2. Bloodwork: Anaplastic large cell lymphoma blood tests are useful as they help to check for the signs of blood cancer. Tests like a complete blood count give details of blood cells like red blood cells, white blood cells, and platelets. Any abnormality in their quantity could signify ALCL. Additionally, some tests even check the blood for enzymes and other markers that might indicate ALCL.

3. Biopsy: A biopsy is a procedure in which some tissues are removed from the body, and examined under a microscope. The study of the cells enables doctors to identify the kind of ALCL, and thereby arrive at the best treatment option. For anaplastic large cell lymphoma, a biopsy is the only way to confirm the diagnosis. 

How to Prepare for the Doctor’s Consultation?

Before visiting the doctor to discuss the details of anaplastic large cell lymphoma, it is better to be prepared beforehand. There are also some questions that should be asked in order to get clarity about the cancer. So, be mindful of the following before going for the doctor’s consultation:

1. Get a prior appointment with the doctor.

2. Be prepared for a physical examination and any anaplastic large cell lymphoma test that the doctor might prescribe.

3. Make a note of all the signs and symptoms that one experiences.

4. Make a list of medications and supplements that the patient takes. This needs to be discussed with the doctor.

5. The patient must also talk about any allergic effects or side effects that the patient has.

6. The patient must also get a friend or a family member during the consultation to make a note of the things that the patient may forget.

7. The patient can also make a list of questions that he/she would want to know. Below are some of the questions that one may ask their doctor:

1. What type of ALCL is it?

2. Where is the ALCL located?

3. Is it ALK-positive ALCL or ALK-negative ALCL?

4. Has the cancer spread to other parts of the body?

5. What treatment options are recommended?

6. What are the chances of the cancer going into remission?

7. What is the probability of the recurrence of the cancer?

8. What side effects to expect?

9. How often are follow-up visits required?

The treatment of anaplastic large cell lymphoma depends on the stage of the disease, the subtype of ALCL, and the individual's overall health. Usually, for systemic ALCL, chemotherapy is the standard treatment. For primary cutaneous and BIA ALCL, surgery is preferred. 

Common treatment options based on the type of ALCL may include:

1. Systemic ALCL: Both anaplastic large cell lymphoma ALK-positive and ALK-negative react to traditional systemic chemotherapy. Notably, systemic chemotherapy is a process in which drugs that destroy cancer cells are injected and distributed through the bloodstream via a vein, killing cancer cells. 

Chemotherapy usually includes a combination of drugs that collectively work together to kill cancer cells. Some common drug combinations are:

1. BV-CHP: Brentuximadvedotin, cyclophosphamide, doxorubicin or hydroxydaunorubicin, and prednisone 

2. CHOP: Cyclophosphamide, doxorubicin or hydroxydaunorubicin, vincristine, and prednisone

3. CHEOP: Cyclophosphamide, doxorubicin or hydroxydaunorubicin, etoposide, vincristine, and prednisone.

Sometimes, cancer may recur after some time. If the doctor suspects that the chance of recurrence is high, then he/she might suggest a stem cell transplant during remission. This is a procedure in which cells that were destroyed during chemotherapy, are replaced with healthy cells. If cancer still returns, then other kinds of chemotherapy treatments are suggested. 

2. Primary Cutaneous ALCL: Anaplastic large cell lymphoma treatment guidelines from medical authorities suggest that surgery is the most common treatment for primary cutaneous ALCL. If cancer has infected lymph nodes, radiation therapy is suggested as well, in which energy is directed toward cancer cells, thereby killing them.

Typically, primary cutaneous ALCL returns within five years of treatment, and additional surgery or radiation therapy might be needed to treat it.

If the localized tumour can’t be removed with surgery, the other treatment options available are:

1. Oral methotrexate: This is a drug that is used to treat non-Hodgkin's lymphoma.

2. Bexarotene: This drug is used to treat T cell lymphomas.

3. Brentuximab Vedotin: This drug is used to not only treat systemic ALCL, but also recurring ALCL.

4. Interferon: This drug is used to assist the immune system to identify and fight cancer cells. 

3. BIA ALCL: The treatment for BIA ALCL is surgery that is done to remove the implant and the surrounding cancer cells. If surgery is not an option, then the doctor will prescribe radiation therapy. 

If cancer has returned or spread to various parts of the body, chemotherapy that is used to treat ALK negative ALCL might be suggested. This includes:

1. CHOP: Cyclophosphamide, doxorubicin or hydroxydaunorubicin, vincristine, and prednisone

2. Brentuximab vedotin

1. Lifestyle changes that help include:

2. Consumption of adequate calories and protein

3. Eating frequent small meals in comparison to few large ones

4. Stay active

5. Wash hands frequently

6. Keep distance from sick people

7. Get adequate rest

8. Psychotherapy support

9. Get support from friends and family

10. Join a local ALCL support group

11. Ask doctors for ways to ease symptoms like nausea 

The treatment option and the procedure are also influenced by the patient’s health condition and the opinion of the treating doctor.

Cost of the Anaplastic Large Cell Lymphoma Treatment

The cost of anaplastic large cell lymphoma in India depends on several factors including the stage and the type of cancer, the patient’s age, patient comorbidities, treatment modalities, hospital type and location, doctor’s experience, insurance coverage, and much more.

To know the exact cost of anaplastic large cell lymphoma in India, one must consult their doctor. However, the table below also provides the approximate cost of various treatment modalities.

Anaplastic Large Cell Lymphoma Survival Rate

Anaplastic large cell lymphoma survival rate depends on the type of ALCL and the international prognostic index or IPI score. ALK-positive ALCL has a greater survival rate in comparison to ALK-negative ALCL. The five-year survival rate for ALK-positive ALCL ranges from 33 to 90%, while that of ALK-negative ALCL ranges from 13 to 74%.

For primary cutaneous ALCL, the survival rate is good. Studies show that primary cutaneous ALCL usually returns within five years, but follow-up treatment options are effective in increasing the lifespan. After diagnosis of primary cutaneous ALCL, the survival rate is about 80%. 

Breast implant-associated anaplastic large cell lymphoma survival rate is excellent, and surgically removing the cancer eliminates it permanently.

Anaplastic large cell lymphoma (ALCL) is a serious condition that can have potential risks and complications. While each individual's experience may vary, here are some potential risks and complications associated with ALCL:

1. Disease Progression or Relapse: Despite treatment, there is a possibility of disease progression or relapse in some cases of ALCL. This may occur if cancer cells become resistant to treatment or if the disease is not completely eliminated during initial therapy. Regular follow-up visits and monitoring are important to detect any signs of disease recurrence.

2. Long-Term Effects: Some individuals may experience long-term effects from the treatment of ALCL. These can include infertility, heart problems, lung problems, secondary cancers, or other complications related to the specific treatments received. 

3. Emotional and Psychological Impact: Coping with a diagnosis of ALCL and undergoing treatment can have a significant emotional and psychological impact. Anxiety, depression, fear, and other psychological challenges may arise. 

When to See a Doctor?

It is important to seek medical attention if you experience any concerning symptoms or have risk factors associated with ALCL. Here are some situations when you should consider seeing a doctor:

1. Persistent swelling

2. Unexplained fatigue

3. Weight loss

4. Recurrent fever

5. Persistent itching

To stay healthy, it is important to have a balanced diet. This not only helps the body respond well to treatment but also improves recovery. Notably, a good diet helps to:

1. Prepare the body to accept treatment

2. Helps the body tolerate high doses of chemotherapy

3. Protect the body from infections

4. Improve strength

5. Gives more energy

6. Reduces the risk of developing other kinds of cancer 

The diet should include the following:

1. Fruits and vegetables like apples, bananas, berries, cooked vegetables, and cherry tomatoes. 

2. Carbohydrates give the body energy and also provide essential vitamins and minerals. Eat grains like quinoa, couscous, and whole grains. 

3. Protein is needed by the body to grow and repair damaged cells and tissues. To recover from ALCL, the body requires more protein. Consume food items like meat, fish, eggs, and dairy products. Plant-based sources of protein include tofu, nuts, beans, lentils, and seeds. 

4. Fibre is needed to aid digestion. Fruits, cereals, and vegetables are high in fibre. 

5. Fats like omega-3 are essential sources of energy and vitamins. 

6. Fluid intake should be high, approximately 1.5-2 litres a day.

Any form of cancer can be worrisome and scary. However, anaplastic large-cell lymphoma is a type of cancer that has various prognoses. The type of ALCL greatly impacts the speed at which cancer spreads and impacts the treatment option and the survival rate.

For some people, ALCL spreads rapidly, while for others it develops slowly, allowing more time for treatment. It is best to discuss the same with the doctor. If you notice any symptoms of ALCL, then book an appointment with a doctor from HexaHealth, and get the best treatment possible.

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