Chronic Lymphocytic Leukaemia

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Overview of CLL Chronic lymphocytic leukaemia (CLL) is a cancer that affects a specific type of white blood cells called lymphocytes. It develops slowly and is most commonly diagnosed in people over the age of 60, rarely affecting individuals younger than 40, and almost never children.

In CLL, the bone marrow produces an excessive number of lymphocytes that are not fully developed and don’t function properly, leading to various health issues. Over time, this can cause a higher susceptibility to infections, fatigue, swollen lymph nodes, and abnormal bleeding or bruising.

CLL is distinct from other types of leukaemia, such as chronic myeloid leukaemia (CML), acute lymphoblastic leukaemia (ALL), and acute myeloid leukaemia (AML).

Symptoms of CLL CLL often does not cause symptoms in its early stages and may only be detected during routine blood tests. When symptoms do occur, they can include:

  • Frequent infections
  • Anaemia, characterized by tiredness, shortness of breath, and pale skin
  • Easy bleeding or bruising
  • High temperature (fever)
  • Night sweats
  • Swollen lymph nodes in the neck, armpits, or groin
  • Abdominal swelling and discomfort
  • Unintentional weight loss

If you experience persistent or concerning symptoms, it’s important to see your GP. These symptoms could be caused by conditions other than cancer, but it is always best to have them evaluated.

Treatments for CLL Since CLL progresses slowly and may not cause immediate symptoms, treatment might not be necessary right away if the condition is detected early. Instead, regular monitoring through check-ups over months or years may be recommended to track any changes.

When symptoms develop or the disease advances, treatment options may include:

  • Chemotherapy: Medications taken orally or administered through an IV to destroy cancer cells.
  • Stem Cell or Bone Marrow Transplant: Healthy donor stem cells are transplanted to help your body produce functional white blood cells.

While CLL is not usually curable, treatment can slow its progression and induce symptom-free periods. Treatment may be repeated if CLL returns.

Outlook for CLL The prognosis for CLL depends on factors such as the stage at diagnosis, age, and overall health. Generally, younger and healthier individuals diagnosed at an early stage have a more favorable outlook. While complete cure is uncommon, treatment can help manage and control the disease over many years.

Causes of CLL The exact cause of CLL is not well understood, and it has not been linked to radiation, chemicals, diet, or infections. It is not contagious and cannot be passed from person to person.

Certain genetic factors may increase the risk of developing CLL. Having a close family member with the condition may slightly increase your risk, although it remains relatively low overall.

Diagnosing Chronic Lymphocytic Leukaemia (CLL)

Initial Steps in Diagnosis Chronic lymphocytic leukaemia (CLL) is often detected through routine blood tests conducted for other medical reasons. However, if you experience symptoms such as persistent tiredness, unusual bleeding or bruising, unexplained weight loss, or night sweats, you should consult your GP. Your GP may:

  • Ask about your symptoms, medical history, and any family history of related conditions.
  • Conduct a physical exam to check for swollen lymph nodes, spleen, or liver.
  • Arrange for a blood test to check your overall health and assess white blood cell counts.

Referral to a Specialist If your GP suspects CLL, you will be referred to a haematologist—a doctor specializing in blood disorders—who will conduct further tests to confirm the diagnosis.

Blood Tests

The primary diagnostic test for CLL is a full blood count. This involves checking the number and appearance of different blood cells in a laboratory. An abnormally high count of atypical white blood cells (lymphocytes) often indicates CLL. Analyzing these cells under a microscope can help confirm the diagnosis and provide important information about the progression of the disease.

Imaging Tests

To better understand how CLL is affecting your body, the following imaging tests may be recommended:

  • Chest X-ray to detect any swelling of lymph nodes in the chest area.
  • Ultrasound of the abdomen to check for an enlarged spleen or other organ changes.
  • Computerised tomography (CT) scan to provide detailed images of internal organs, allowing the doctor to detect swollen lymph nodes, spleen, or other abnormalities.

Bone Marrow Biopsy

In some cases, a bone marrow biopsy may be performed. This procedure involves:

  • Extracting a small sample of bone marrow tissue from your hip bone using a needle.
  • Numbing the area with local anaesthetic before the needle is inserted.
  • The procedure typically takes around 15 minutes, and you won’t usually need to stay in the hospital overnight. Some bruising and discomfort may occur for a few days after the procedure. The sample is examined under a microscope for cancerous cells to assess disease progression and determine the most appropriate treatment.

Lymph Node Biopsy

In certain instances, a lymph node biopsy may be required. This involves:

  • Surgically removing a swollen lymph node.
  • The procedure can be performed under local or general anaesthetic, depending on the circumstances, and typically does not require an overnight hospital stay.
  • The excised lymph node is examined for cancer cells, providing further clarity in the diagnosis of CLL.

Genetic Testing

Your blood and bone marrow samples may also be tested for genetic abnormalities in cancerous cells. Identifying unusual genes can help doctors determine:

  • How aggressive your CLL is likely to be.
  • When you should start treatment.
  • Which treatments are likely to be most effective for your condition. Certain genetic mutations may make specific treatments less effective, and these tests provide important information for personalized care planning.

Treating Chronic Lymphocytic Leukaemia (CLL)

Treatment for chronic lymphocytic leukaemia (CLL) depends on the stage of the disease at diagnosis. Many people may not require immediate treatment if the condition is detected early. However, for more advanced stages, treatments such as chemotherapy are often necessary. While treatment can help control CLL for extended periods, it may not cure it completely. Remission may occur after initial treatment, but the disease often returns and may require additional treatment.

Stages of CLL

Doctors classify CLL into three main stages, which helps determine the need for treatment:

  • Stage A: Enlarged lymph nodes in fewer than three areas and a high white blood cell count.
  • Stage B: Enlarged lymph nodes in three or more areas and a high white blood cell count.
  • Stage C: Enlarged lymph nodes or spleen, a high white blood cell count, and a low red blood cell or platelet count.

Stage B and C CLL generally require immediate treatment, while Stage A usually needs treatment only if the condition worsens or starts causing symptoms.

Monitoring Early-Stage CLL

If there are no symptoms at diagnosis:

  • Regular check-ups and blood tests are used to monitor the condition.
  • Immediate treatment is avoided due to possible side effects, and treatment begins only if symptoms develop or the disease progresses.

Chemotherapy for Advanced CLL

Most patients eventually require chemotherapy to control the cancer. Treatment typically involves:

  • Fludarabine (tablet form): Taken for 3-5 days at the start of each treatment cycle.
  • Cyclophosphamide (tablet form): Taken for 3-5 days at the start of each treatment cycle.
  • Rituximab (intravenous infusion): Administered in a hospital over several hours at the start of each cycle.

Alternative Medicines: If these medications are unsuitable or ineffective, options include bendamustine, chlorambucil, ibrutinib, idelalisib, obinutuzumab, ofatumumab, and prednisolone.

Side Effects of Treatment

Common side effects include:

  • Fatigue
  • Nausea
  • Increased risk of infection
  • Easy bruising or bleeding
  • Anaemia (shortness of breath, pale skin, weakness)
  • Hair loss or thinning
  • Allergic reactions
  • Irregular heartbeat

Notify your care team if you experience side effects, as they may offer treatment or adjustments to help.

Stem Cell or Bone Marrow Transplants

Stem cell transplants may help achieve prolonged control or remission:

  • High-dose chemotherapy and radiotherapy are used to destroy cancer cells.
  • Stem cells from a donor (preferably a close relative) are transplanted into the patient’s bloodstream.
  • This is a potential cure but is rarely used due to its intensity, risks, and unsuitability for many older CLL patients.

Other Treatments

Additional options include:

  • Radiotherapy: To reduce swollen lymph nodes or spleen.
  • Surgery: Removal of a swollen spleen.
  • Infection Prevention: Antibiotics, antifungals, and antivirals.
  • Blood Transfusions: For severe anaemia or bleeding.
  • Immunoglobulin Replacement Therapy: Prevents infections.
  • G-CSF Injections: Boosts white blood cell counts.

Deciding Against Treatment

Choosing not to undergo certain treatments due to potential side effects affecting quality of life is a personal decision. If you opt out of a specific treatment, pain relief and supportive care will still be available.

Before making any treatment decisions, speak with your doctor and consider involving your family or friends. You’ll be given time to make informed choices about managing CLL.

Complications of Chronic Lymphocytic Leukaemia (CLL)

Chronic lymphocytic leukaemia (CLL) can sometimes lead to various complications, affecting overall health and quality of life. Here are the key complications associated with CLL:

1. Infections

People with CLL often have a weakened immune system, making them more vulnerable to infections due to a reduced number of healthy, infection-fighting white blood cells. Chemotherapy treatment can further suppress immune function.

Managing Infection Risk:

  • Report Symptoms Quickly: Notify your GP or care team if you experience signs of an infection, such as fever, muscle aches, diarrhea, or headaches.
  • Vaccination Guidance: Stay up-to-date on vaccinations, but consult your GP or care team regarding which vaccines are safe, as some may not be recommended due to a weakened immune system.
  • Avoid Infected Individuals: Keep a distance from people with infections, even if you’ve had immunity to conditions like chickenpox in the past.
  • Preventive Medications: Your doctor may prescribe regular doses of antibiotics or other medications to lower infection risks.

2. Richter’s Syndrome

In about 1 in 20 people with CLL, the disease can transform into an aggressive type of non-Hodgkin lymphoma, known as Richter’s transformation or Richter’s syndrome.

Symptoms of Richter’s Syndrome Include:

  • Rapid swelling of lymph glands
  • Fever
  • Night sweats
  • Unexplained weight loss
  • Abdominal pain

Treatment: Usually involves intensive chemotherapy combined with other medications. More detailed information is available through resources like Cancer Research UK.

3. Autoimmune Haemolytic Anaemia

Approximately 1 in 10 people with CLL may develop autoimmune haemolytic anaemia, a condition where the immune system attacks and destroys red blood cells, leading to severe anaemia.

Symptoms of Anaemia:

  • Fatigue
  • Breathlessness
  • Weakness

Treatment: Typically managed with steroid medications to suppress immune system activity against red blood cells.

4. Psychological Effects

Being diagnosed with CLL can be emotionally challenging, especially since it often cannot be cured, and treatment may involve long waiting periods before active management. These circumstances can lead to stress, anxiety, and depression.

Coping with Psychological Effects:

  • Seek Support: Reach out to your GP or care team if you’re struggling emotionally.
  • Connect with Others: Consider joining support groups to speak with others who have leukaemia. Your care team or GP can provide information about local support options.
  • Helpline Support: Macmillan Cancer Support offers extensive help and advice. You can contact their helpline at 0808 808 00 00 (Monday to Friday, 8 am to 8 pm).

Living with CLL involves ongoing management and support. By staying informed about potential complications and being proactive in seeking care and emotional support, you can better manage the challenges of the condition.

Causes of Chronic Lymphocytic Leukaemia (CLL)

The exact cause of chronic lymphocytic leukaemia (CLL) is not fully understood. However, researchers have identified several factors that may increase the likelihood of developing the condition. Below is a summary of these risk factors:

1. Family History

In some instances, CLL appears to run in families. This suggests a potential inherited genetic mutation that may increase susceptibility to developing the disease. More research is necessary, but having a parent or sibling with CLL slightly raises your risk of also developing the condition.

2. Ethnicity

CLL is more prevalent among people of European, American, and Australian origin and less common in individuals from China, Japan, and Southeast Asia. The reason for these ethnic differences is not well understood, but studies show that CLL is more frequent in white populations than in black or Asian populations.

3. Other Medical Conditions

Certain medical conditions may slightly elevate your risk of developing CLL. These conditions include:

  • Pneumonia (chest infections)
  • Sinusitis
  • Shingles
  • Autoimmune haemolytic anaemia
  • Chronic osteoarthritis
  • Prostatitis (inflamed prostate)

Some of these conditions may occur due to lowered immunity, which can happen during the early stages of CLL, rather than directly causing it. Additionally, having a weakened immune system, such as due to HIV/AIDS or taking immunosuppressive medications after an organ transplant, may also increase your risk of developing CLL.

4. Radiation Exposure

While exposure to radiation has been linked to an increased risk of other types of leukaemia, there is no conclusive evidence connecting radiation exposure specifically to CLL.

5. Sex and Age

Men are approximately twice as likely to develop CLL compared to women, for reasons that are not entirely understood. The risk of developing CLL also increases with age; it is most commonly diagnosed in individuals over the age of 60.

The Cancer Research UK website provides additional information about risk factors associated with CLL. If you are concerned about your risk, speaking with a healthcare professional can help you better understand your susceptibility and options for monitoring or reducing potential risks.


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