Acute Lymphocytic Leukaemia: Symptoms, Causes, Treatment

Acute lymphocytic or lymphoblastic leukaemia(ALL) is a rare blood and bone marrow cancer. The term “acute” refers to the rapid production of immature white blood cells, called lymphocytes, in the bone marrow. 

While ALL is a serious condition and the most common type of cancer in children, advancements in treatment options can cure the disease. ALL can also occur in adults, but the possibility of a cure is significantly reduced. 

Acute lymphocytic leukaemia can be classified into subtypes based on specific characteristics of the leukaemia cells. The classification is important as it helps determine the appropriate treatment approach and prognosis for individuals with ALL. The common acute lymphocytic leukaemia types include:

1. B-cell ALL: This is the most common subtype of ALL, accounting for about 75-80% of cases. It originates from abnormal B-lymphocytes. They are a type of white blood cell responsible for producing antibodies and fighting infections.

2. T-cell ALL: It arises from abnormal T-lymphocytes. They are another type of white blood cell responsible for destroying germs and supporting other immune system cells.

3. Natural Killer ALL: The third type affects natural killer cells. These are white blood cells that destroy infected and cancerous cells. Natural killer ALL is a very rare type of ALL.

Acute Lymphocytic Leukaemia Stages

Acute lymphocytic leukaemia is not typically staged in the same way as solid tumours. Instead, it is classified based on the treatment. The stages of ALL include:

1. Untreated: It refers to a new diagnosis of ALL. The patient may have received treatment for symptoms but not for the cancer. 

2. Remission: In this stage, the patient has received treatment to kill as many leukaemia cells as possible. They have a normal complete blood count and no more than 5% leukaemia cells in their bone marrow. 

3. Recurrent or Relapsed: Leukaemia returns after treatment and remission. 

Acute lymphocytic leukaemia can cause various symptoms, varying from person to person. The symptoms may occur suddenly and affect children and adults similarly. Acute lymphocytic leukaemia symptoms can be similar to those of other conditions. The common ALL symptoms include:

1. Fatigue, Weakness or Dizziness: Feeling constantly tired and lacking energy is a common symptom of ALL that results from low red blood cell count. This fatigue may be unrelated to activity level and interfere with daily routines. Anaemia can also cause dizziness. 

2. Shortness of Breath: People affected by ALL may experience shortness of breath during normal physical activities due to decreased healthy red blood cells. 

3. Fever: Unexplained or persistent fever is another common symptom in individuals with ALL. Fever may be accompanied by night sweats. 

4. Frequent Infections: A low count of neutrophils (white blood cell type) can weaken the immune system in ALL patients. This makes them more susceptible to infections. 

5. Easy Bruising and Bleeding: Individuals with ALL may experience easy bruising. They may also frequently experience the following symptoms due to a low platelet count:

1. Prolonged bleeding from minor cuts

2. Nosebleeds

3. Bleeding gums

4. Heavier menstrual periods

6. Bone and Joint Pain: Leukaemia cells may accumulate in the bones and cause painful joints and bones. 

7. Swollen Lymph Nodes and Spleen: Individuals may have lumps caused by swollen lymph nodes, particularly in the armpits, neck, abdomen, or groin. An enlarged spleen may cause pain or a feeling of fullness below the ribs. 

8. Pale Skin or Pallor: A shortness of red blood cells can result in pale skin. 

9. Petechiae: These are red or purple spots on the skin caused by a low platelet count. 

10. Reduced Appetite and Weight Loss: ALL patients may experience the following:

1. Weight loss without apparent reason

2. Loss of appetite

3. A feeling of fullness after eating small amounts of food

The aetiology of acute lymphocytic leukaemia is unknown. However, certain environmental factors can increase its risk. The pathophysiology of acute lymphocytic leukaemia involves a change in the DNA of bone marrow cells. 

1. Normally, the cells grow and multiply at a set rate. As the cells age, they die to make room for new cells. 

2. However, cells continue to grow in acute lymphocytic leukaemia due to DNA mutations. 

3. When this happens, the bone marrow produces immature cells that develop into lymphocytes. 

4. The buildup of these abnormal cells crowd out healthy cells from the bone marrow. 

Acute Lymphocytic Leukaemia Risk Factors

Several factors can increase the risk of acute lymphocytic leukaemia. However, individuals must note that having one or more risk factors does not necessarily mean they will develop ALL. Risk factors for ALL include:

1. Age: ALL is more common in children and people above 50. 

2. Gender: Girls younger than one year have a higher risk of ALL than boys. After age 1, males are at a higher risk than females. However, the reason for this gender difference is not fully understood.

3. Ethnicity: ALL is more common in white individuals. But the reason for this is unknown. 

4. Radiation Exposure: High levels of ionising radiation have been linked to an increased risk of developing ALL. This may include radiation therapy for previous cancers or exposure to nuclear reactor radiation.

5. Chemical Exposure: Exposure to chemotherapy drugs and certain chemicals, such as benzene, has been associated with an increased ALL risk.

6. Viral Infections: Infections caused by certain viruses, such as the Epstein-Barr virus (EBV) and human T-cell leukaemia virus, can increase the risk of developing ALL.

7. Genetic Disorders: Certain genetic abnormalities and inherited conditions may cause an increased risk of ALL. Some of these disorders may include:

1. Down syndrome (individuals have an extra chromosome)

2. Fanconi anaemia (affects the bone marrow and many other body parts)

3. Bloom syndrome (causes changes in growth and facial features)

4. Klinefelter syndrome (males are born with an extra X chromosome)

5. Ataxia telangiectasia (affects the nervous system, immune system, and other body systems)

6. Li-Fraumeni syndrome (increases the risk of developing several types of cancer)

7. Neurofibromatosis (tumours form on nerve tissue)

Preventing acute lymphocytic leukaemia may not be possible since the exact cause of the disease is often unknown. However, some healthy lifestyle practices can help reduce the risk of developing ALL. These lifestyle practices may include:

1. Avoiding Carcinogens: Individuals should limit exposure to known carcinogens, such as tobacco smoke, benzene, certain pesticides, and other toxic chemicals. These carcinogens have been linked to an increased risk of leukaemia and other cancers.

2. Protecting Against Viruses: Some viral infections can increase the risk of developing ALL. People must take preventive measures to reduce the risk of contracting these viruses. These measures include practising good hygiene and avoiding close contact with individuals affected by infectious illnesses.

3. Minimising Radiation Exposure: Avoiding radiation may not be possible in all cases. However, individuals must follow safety guidelines when undergoing radiation-related medical imaging procedures.

Several signs and symptoms can indicate acute lymphocytic leukaemia. However, certain tests are needed to confirm the diagnosis. The doctor will begin by evaluating the patient’s symptoms, reviewing their medical history, and conducting a physical exam. They may suggest further tests, such as:

1. Blood Tests: A complete blood count (CBC) is an initial test that evaluates the number and types of blood cells. In ALL, the CBC may reveal:

1. Low levels of red blood cells (anaemia)

2. Low platelet counts (thrombocytopenia)

3. Odd numbers of white blood cells

4. Blast cells (immature cells in the bone marrow)

2. Bone Marrow Tests: Bone marrow biopsy and aspiration involve collecting bone marrow samples from the hipbone or breastbone. The samples are examined under a microscope to assess the presence of leukaemia cells and determine their characteristics. Bone marrow examination is used for confirming the diagnosis of ALL.

3. Lumbar Puncture (Spinal Tap): It collects a sample of spinal fluid (fluid surrounding the brain and spinal cord). The procedure helps determine if leukaemia has spread to the spinal fluid. 

4. Imaging Tests: These include tests like an X-ray, MRI scan, CT scan, and ultrasound. Imaging tests are performed to determine whether the leukaemia has spread to the brain, spinal cord, or other body parts. 

5. Flow Cytometry: The test identifies the proteins on the surface of the cancer cells. The test helps determine the exact subtype of ALL. 

6. Chromosome Tests: Cytogenetic analysis examines the chromosomes in leukemic cells to identify specific genetic abnormalities, such as translocations (when a chromosome breaks and attaches to a different chromosome). Other tests, like polymerase chain reaction (PCR) and fluorescence in situ hybridisation (FISH), can detect specific genetic changes and markers associated with ALL.

When caring for patients with acute lymphocytic leukaemia, nurses utilise nursing diagnoses to identify and address the patient’s specific needs and concerns. Acute lymphocytic leukaemia nursing diagnosis includes symptoms like:

1. Weight loss

2. Pain

3. Bruising

4. Fatigue and weakness

5. Fever 

6. Pale skin

7. No appetite

8. Anxiety

How to Prepare for a Doctor’s Consultation? 

If an individual or their child has acute lymphocytic leukaemia symptoms, they should make an appointment with their doctor. If the doctor suspects ALL, they will refer the patient to a haemotologist (a doctor specialising in treating conditions of the bone and bone marrow). The following are some steps to help individuals prepare for their appointment:

1. Know any Pre-appointment Restrictions: People must make a prior appointment and ask if they need to prepare in advance, such as restricting their diet. 

2. Note Down the Symptoms: Patients should write the symptoms they are experiencing, along with their duration. 

3. Collect Medical Information: It would help to gather all relevant medical records, including previous test results, imaging reports, and information about previous treatments.

4. Make a List of Medications: Patients are advised to list their medications, vitamins, and supplements. This helps the doctor understand if there are any drug interactions. 

5. Write Down Questions: Preparing a list of questions and concerns the patient may want to discuss with the healthcare provider is best. Questions may cover the diagnosis, treatment options, potential side effects, prognosis, and any lifestyle modifications to be made.

6. Bring a Friend or Family Member: Consider bringing a family member or friend to the appointment for support. They can also help remember important information discussed during the appointment.

What to Expect from the Doctor?

During an ALL consultation appointment, the healthcare provider will ask various questions to gather information about the patient’s medical history, symptoms, and overall health. Here are some common questions patients can expect:

1. What symptoms have you been experiencing, and when did they start?

2. How severe are your symptoms?

3. Are these symptoms continuous or occasional?

4. Does anything seem to improve or worsen these symptoms?

5. Have you undergone any previous cancer treatments, such as radiation therapy or chemotherapy?

6. Are you exposed to any environmental toxins or chemicals in your work or living environment?

7. Do you smoke?

Questions to Ask the Doctor

Individuals must ask questions during the appointment to gather information and clarify doubts. Here are some questions patients may consider asking their healthcare provider:

1. What is the cause of my symptoms?

2. What tests do I need?

3. What type and subtype of ALL do I have?

4. Is this condition temporary or chronic?

5. What are the recommended treatment options for my ALL?

6. Are there any alternatives to the methods that you are suggesting?

7. What is the drug of choice for acute lymphocytic leukaemia in my case?

8. How long will the treatment last, and what can I expect regarding side effects or complications?

9. Are there any clinical trials or targeted therapies available that may be suitable for my condition?

10. How can I manage other health conditions with ALL?

11. Do I need to follow any restrictions?

12. Can any lifestyle modifications, such as dietary recommendations, support my overall well-being during and after treatment?

13. Will I need to see a specialist? How much will it cost?

14. How often will I need to come in for follow-up visits?

For those wondering how is acute lymphocytic leukaemia treated, there are several methods for the same. Treatment aims to induce remission, eliminate leukaemia cells, prevent relapse, and improve long-term survival. Generally, the treatment for ALL is divided into four phases, which are as follows:

1. Induction Therapy: The primary goal of induction therapy is to induce remission by rapidly reducing the number of leukaemia cells in the blood and bone marrow. It also involves restoring normal blood cell production. The treatment takes place for around four to six weeks. 

2. Consolidation Therapy: Once remission is achieved, consolidation therapy is initiated to eliminate any remaining leukaemia cells in the body. It involves staying in the hospital for several months while the patients receive weekly chemotherapy. 

3. Maintenance Therapy: The third phase of treatment is typically administered to help maintain remission and prevent relapse. It involves lower doses of treatments over a more extended period, usually two to three years. 

4. Preventive Treatments for the Spinal Cord: During each treatment phase, ALL patients may receive additional treatments to kill cancer cells in their central nervous system. This may include injecting chemotherapy drugs directly into the cerebrospinal fluid. 

Treatment options for acute lymphocytic leukaemia may include:

1. Chemotherapy: Doctors use chemotherapy as the primary treatment for ALL. Chemotherapy uses drugs to kill cancer cells. The drug of choice for acute lymphocytic leukaemia typically includes vincristine, prednisone, doxorubicin, etc. 

   Chemotherapy for acute lymphocytic leukaemia is used as induction therapy for children and adults. However, it can also be used in the consolidation and maintenance phases of the treatment. The treatment goal is to put the disease into complete remission. 

2. Targeted Therapy: Targeted drug treatment selectively targets specific genetic abnormalities in leukaemia cells. By blocking these abnormalities, targeted therapy causes cancer cells to die. Targeted therapy can be used alone or with chemotherapy in induction, consolidation, and maintenance phases. 

   Doctors use tyrosine kinase inhibitors (TKI) like imatinib and dasatinib to treat children and adults with Philadelphia chromosomes (a specific mutation that forms when chromosomes 9 and 22 break and exchange parts). 

3. Immunotherapy: These drugs boost an individual’s immune system to kill or slow down the cancerous cells’ growth. Immunotherapy for ALL may include monoclonal antibodies such as blinatumomab and inotuzumab ozogamicin. The FDA has also approved CAR T-cell therapy that uses patients’ own immune cells to treat cancer. 

4. Radiation Therapy: It uses high-energy beams to target and kill cancer cells. Radiation may be employed in specific situations, such as to treat leukaemia cells that have spread to the central nervous system. The therapy is also recommended to treat recurrent ALL or cancer that has not responded to chemotherapy. 

5. Bone Marrow Transplant: Also known as stem cell transplant, a bone marrow transplant may be recommended as a consolidation therapy or to treat ALL relapses. The procedure involves replacing diseased bone marrow with healthy stem cells from a donor (allogeneic transplant). Before a transplant, individuals receive high doses of chemotherapy or radiation to destroy leukaemia cells-producing bone marrow. 

6. Supportive Care: Supportive care plays a crucial role in ALL treatment when treatment to cure leukaemia becomes unlikely. Also known as palliative treatment, it includes managing side effects, preventing complications, and supporting the patient’s well-being. This may include medications to manage pain, nausea and other symptoms and antibiotics to prevent infections.

Please Note: The selection of the treatment approach is based on the patient’s health condition and the treating doctor’s opinion. 

Cost of the Acute Lymphocytic Leukaemia Treatment

The cost of the acute lymphocytic leukaemia in India vary on several factors including the stage of the cancer, the type of the cancer, treatment modality, hospital type and location, patient’s age, patient’s comorbidities, insurance coverage and much more.

To know the exact cost of the acute lymphocytic leukaemia in India, one must consult their healthcare provider. However, the table below also provides the approximate cost of various treatment modalities for acute lymphocytic leukaemia.

Acute Lymphocytic Leukaemia Prognosis

The prognosis is the outcome that an individual may expect after treatment. Generally, the prognosis for ALL has significantly improved over the years, particularly for childhood acute lymphocytic leukaemia. The disease often goes into complete remission after treatment with chemotherapy drugs to kill cancer cells. However, the prognosis for adults with ALL is typically less favourable than children’s. 

Acute lymphocytic leukaemia prognosis can vary depending on factors such as age, subtype of ALL, presence of specific genetic abnormalities, and response to treatment. Criteria for a good prognosis include:

1. Age below 30 years

2. White blood cell count less than 30,000

3. No abnormal cytogenetics (chromosomal abnormalities)

4. Complete remission within four weeks

Acute Lymphocytic Leukaemia Survival Rates

Acute lymphocytic leukaemia survival rates vary based on the patient’s age. It is important to note that survival rates are statistical averages based on past data. It cannot predict the outcome for an individual patient. Acute lymphocytic leukaemia statistics for survival rates are as follows:

1. The five-year survival rate for children aged 14 or below is 90%. 

2. The five-year survival rate for children between 15 to 19 is 70%. 

3. The five-year survival rate for individuals aged 20 and above is 30%.

Acute lymphocytic lymphoblastic leukaemia that spreads to a person’s brain and spinal cord may cause several complications. The complications can vary from person to person and may depend on factors such as the subtype and stage of the disease and the patient’s age. These complications include:

1. Blurred vision 

2. Headaches

3. Balance problems

4. Nausea or vomiting

5. Numbness or weakness of the facial muscle

6. Seizures

7. Hepatomegaly (enlarged liver)

8. Splenomegaly (enlarged spleen)

9. Superior Vena Casa (SVC) syndrome (a group of problems caused when superior vena casa is blocked or compressed)

Risk of Delayed Treatment

The timely initiation of treatment for acute lymphocytic leukaemia is crucial for the best possible outcomes. If ALL treatment is delayed, several potential complications may arise. These complications include:

1. Worsening of Disease: ALL is acute cancer, i.e., it can rapidly progress if left untreated. Delaying treatment allows leukaemia cells to continue multiplying and spreading throughout the body. Therefore, once an individual is diagnosed with cancer, treatment should begin immediately. 

2. Increased Complications Risk: If the treatment is delayed, an individual with CLL becomes increasingly susceptible to infections, fatigue, and excessive bleeding. 

3. Reduced Treatment Response: Early commencement of treatment can result in better treatment outcomes and improve the chances of complete remission. On the other hand, delaying treatment can reduce the effectiveness of ALL treatments.

4. Death: If left untreated, ALL can progress rapidly and be fatal.  

When to Call a Doctor?

Individuals should make an appointment with their doctor if they or their child experience persistent signs and symptoms of ALL. Many symptoms may mimic the signs of the flu. However, if symptoms do not improve, consultation with a doctor becomes important. Some signs that may require medical attention include:

1. Unexplained fever

2. Shortness of breath

3. Persistent or severe pain

4. Significant fatigue

5. Uncontrolled bleeding, etc. 

Although no specific diet can treat ALL, a healthy, well-balanced diet is essential for individuals with acute lymphocytic leukaemia. It helps support overall well-being, maintain strength, and manage potential treatment side effects. The following dietary recommendations can be beneficial:

Foods to Eat

1. Various vegetables like broccoli, cabbage, and cauliflower

2. Fruits like apples and blueberries

3. Whole grains like wheat, oats, and barley

4. Fat-free or low-fat dairy products, such as cottage cheese and yoghurt

5. Healthy oil like canola or olive oil

6. Low-fat protein sources like soy, fish, and chicken

7. Water and tea

Foods to Avoid

1. Foods rich in fibre or sugar, like avocados, cakes, and sweetened drinks

2. Spicy foods

3. Very hot or very cold food items

4. Greasy or fried foods, like french fries and cheese sticks

5. Citrus fruits, like lemon and grapefruit

6. Tomatoes and ketchup

7. Apple juice

8. Alcohol

9. Caffeine

Cancer treatment can weaken an individual’s immune system and increase their risk of foodborne illnesses. The following foods should be avoided to reduce exposure to bacteria:

1. Raw and undercooked meat and seafood

2. Uncooked or unpasteurised eggs

3. Unwashed fruits and vegetables

4. Raw sprouts

5. Soft cheese made of unpasteurised milk

6. Food from buffets and salad bars

Acute lymphocytic leukaemia is a challenging blood cancer that requires timely diagnosis and appropriate treatment. The drug of choice for acute lymphocytic leukaemia depends on the individual’s specific characteristics, subtype, and risk factors.

Advances in medical research and treatment have significantly improved outcomes for individuals with ALL, with higher remission rates and improved long-term survival.

The HexaHealth team understands the complexities of ALL and provides comprehensive care and support. Our experienced healthcare professionals, including haematologists and oncologists, utilise the latest treatment protocols to develop personalised treatment plans.

With a patient-centred approach, we offer guidance, education, and emotional support to empower patients throughout their ALL journey. Contact us TODAY to know more! 

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