Chronic lymphocytic leukemia (CLL) is a type of cancer that affects white blood cells called lymphocytes and causes them to grow abnormally. CLL progresses slowly, and many people live for years without having significant symptoms. However, over time CLL can worsen and treatment may be needed. Knowing what stage of CLL you have is important for determining the best treatment approach.
Doctors determine the stage of CLL based on physical exams, blood tests, and imaging tests. These provide information about the locations and extent of cancer cells in the body. There are two main staging systems used for CLL:
Rai Staging System
The Rai staging system is one of the most commonly used systems for CLL. It divides CLL into five different stages based on disease progression:
| Stage | Definition |
|---|---|
| Stage 0 | Lymphocytosis with no enlargement of lymph nodes, spleen, or liver, and with normal red blood cell and platelet counts |
| Stage I | Lymphocytosis plus enlarged lymph nodes |
| Stage II | Lymphocytosis plus splenomegaly (enlarged spleen) with or without enlarged lymph nodes |
| Stage III | Lymphocytosis plus anemia (low red blood cell count) with or without enlarged lymph nodes, spleen, or liver |
| Stage IV | Lymphocytosis plus thrombocytopenia (low platelet count) with or without anemia, enlarged lymph nodes, spleen, or liver |
Higher stages indicate more advanced disease. Stage 0 means there are abnormal lymphocytes but no other symptoms. Stages I-IV mean lymphocytes are accumulating and causing enlargement of organs and decreased blood cell counts.
Binet Staging System
The Binet staging system categorizes CLL into three stages – A, B, and C. It is based on the number of affected lymph node areas and whether the patient has anemia or thrombocytopenia:
| Stage | Definition |
|---|---|
| Stage A | Fewer than 3 areas of lymph node enlargement, and no anemia or thrombocytopenia |
| Stage B | 3 or more areas of lymph node enlargement, and no anemia or thrombocytopenia |
| Stage C | Anemia and/or thrombocytopenia present regardless of the number of lymph node areas affected |
Stage A indicates fewer lymph node groups affected. Stages B and C indicate more widespread lymph node enlargement, with stage C being associated with decreased blood cell counts.
Diagnostic Tests Used for Staging
Doctors use information from diagnostic tests to determine the Rai or Binet stage for a patient with CLL. Common tests used include:
Complete Blood Count (CBC)
This blood test measures levels of white blood cells, red blood cells, and platelets. It helps identify lymphocytosis and whether a patient has anemia or thrombocytopenia.
Peripheral Blood Smear
A sample of blood is examined under a microscope. Abnormal CLL cells can be identified and provide information about disease progression.
Flow Cytometry
This test identifies particular proteins on the surface or inside of lymphocytes. It can confirm CLL and may provide information about cell characteristics that can influence staging.
Bone Marrow Biopsy
A sample of bone marrow is removed, usually from the hip bone. It is examined to see if the marrow contains CLL cells. This helps determine if the disease has progressed to more advanced stages.
Imaging Tests
Imaging tests like CT scans and ultrasounds allow doctors to visualize the size of lymph nodes, spleen, and liver. Enlargement of these organs indicates more advanced CLL.
Genetic Testing
CLL cells may be tested for genetic abnormalities like deletions or mutations. Certain genetic features can provide information about how aggressive the CLL is likely to be. More mutations generally mean more advanced disease.
Key Factors in Staging
Some key factors help determine CLL staging:
Lymphocyte Count
A higher lymphocyte count indicates more advanced CLL. Counts below 5000/microliter are earlier stage, while counts over 50,000/microliter indicate later stage disease.
Presence of Symptoms
Patients without symptoms like fatigue, night sweats, or weight loss usually have earlier stage CLL. Development of these symptoms often signals progression to later stages.
Number of Affected Areas
For the Binet stages, more areas of enlarged lymph nodes mean more advanced CLL. Widespread lymph node involvement extending to the spleen and liver are later stage features.
Blood Cell Counts
Normal blood cell counts indicate earlier stage CLL. Anemia and low platelets, as seen in Rai stages III-IV and Binet stage C, are markers of advanced CLL.
High Risk Genetic Features
CLL cells that have certain chromosome deletions like 17p or 11q, or mutations like TP53, often behave more aggressively. These genetic abnormalities typically mean later stage disease.
Prognosis Based on Stage
In general, prognosis worsens with increasing stage of CLL:
Stages 0 and I (Rai) and Stage A (Binet)
Early stage disease has a good prognosis overall. Many patients have no symptoms and may not need treatment right away. The 5-year survival is over 90%.
Stages II and III
Intermediate stage CLL has more extensive involvement and higher risk features. Treatment is often started at these stages. The 5-year survival is 70-80%.
Stage IV
Advanced stage CLL has poor prognosis factors like very high lymphocyte counts, extensive enlargement of lymph nodes and organs, and decreased blood counts. Intensive treatment is required. The 5-year survival is under 50%.
However, prognosis depends on more than just the stage. Other factors like the patient’s age, overall health, genetic variants in the CLL cells, and response to treatment also impact outlook. Your doctor considers all of these when discussing prognosis.
Staging Helps Guide Treatment
Staging CLL is important because it helps determine the appropriate treatment approach:
Watch and Wait for Early Stage
In Rai or Binet stages 0-I, treatment might not be needed right away. Doctors follow with regular exams and lab testing. Treatment can be delayed until the disease progresses.
Chemotherapy for Intermediate Stage
For stages II-III, chemotherapy drugs like fludarabine, bendamustine, or chlorambucil are often used as initial treatment. This helps control the disease and prevent progression.
Targeted Drugs for Advanced Stage
Stage IV CLL may be treated with targeted therapies like ibrutinib, venetoclax, or obinutuzumab. These can be more effective than chemotherapy for high risk disease.
Stem Cell Transplant for High Risk Disease
A stem cell transplant may be an option for younger, healthy patients with very advanced or high risk CLL. This can potentially cure the disease when other treatments fail.
Staging guides which treatments would most benefit individual patients. Those with extensive, symptomatic disease require more intensive therapies. The goal is to tailor the treatment plan based on where you are in the course of CLL.
Monitoring and Reevaluation of Stage Over Time
CLL staging provides a snapshot of where a patient is at a particular point in time. However, the disease can evolve and change:
- Early stage CLL may remain stable and not require treatment for years.
- Intermediate stage disease can respond well to chemotherapy and revert to lower stage.
- Advanced CLL can progress rapidly to more concerning stages.
Doctors monitor CLL closely through follow up visits, blood work, and repeat imaging. Restaging is done periodically to see if patients respond to treatment or if the disease advances.
Reevaluation of CLL stage over time ensures patients receive the most appropriate interventions tailored to where they’re at in the course of their disease. Ongoing monitoring also allows doctors to identify those with aggressive, rapidly advancing CLL who may need to move rapidly to stem cell transplant.
What Should You Do If Your Stage Changes?
If restaging shows your CLL has progressed to a more advanced stage, don’t panic. Here are some steps to take:
– Discuss the results thoroughly with your hematologist/oncologist. Make sure you understand what the change in stage means.
– Ask your doctor if your treatment plan needs to be modified based on the new stage. More intensive therapy like targeted drugs or transplant may now be appropriate.
– Seek psychological support if you are feeling anxious or distressed about your CLL worsening. Counselors can help with coping strategies. Support groups connect you with others facing the same concerns.
– Strengthen your optimism and look for hope. Advances in newer CLL treatments allow patients to live longer than ever before, even with advanced disease.
– Focus on what you can control. Adhering closely to your doctor’s recommendations, maintaining a healthy lifestyle, and getting needed support can help you continue to live well.
– Reach out to loved ones and engage your support system. Spend time doing enjoyable activities that bring you comfort. Don’t isolate yourself.
With proper guidance from your medical team and a positive attitude, a change in CLL stage does not have to prevent you from living fully. Be proactive but also be confident you have options.
Conclusion
Determining your CLL stage is important to guide your treatment and provide information about prognosis. Staging is based on test results like blood counts, imaging, bone marrow exams and lymph node assessment. Key factors include lymphocyte levels, symptoms, affected areas, genetics, and blood counts. Generally, higher stages mean more advanced CLL. However, the disease course can vary. Doctors monitor and re-evaluate stage periodically. If your stage increases, work with your doctor on adjusting your treatment plan and utilize healthy strategies to cope with the change. Staying informed, connected and hopeful allows you to continue to live your best life through all stages of CLL.
