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What are the Stages of CLL?
Posted: Jun 11, 2024
What are the Stages of CLL? image

Drs. Ravi Vij and Alvaro Alencar explain the staging process of CLL and its impact on treatment decisions. Watch their interview below: 

What's the Rai Staging System in CLL?

The Rai staging system categorizes CLL into five stages: 

  • Rai stage 0: Many lymphocytes (white blood cells) with no other issues
  • Rai stage I: Lymph glands are swollen
  • Rai stage II: Spleen and/or liver are enlarged
  • Rai stage III: Low numbers of red blood cells (anemia)
  • Rai stage IV: Low numbers of platelets (thrombocytopenia)

In Europe there is the Binet staging system that considers lymph nodes, anemia severity, and platelet count to stage CLL. 

  • Stage A: Hemoglobin ≥ 10 g/dL, platelets ≥ 100,000/mm3, and less than 3 groups of enlarged lymph nodes
  • Stage B: Hemoglobin ≥ 10 g/dL, platelets ≥ 100,000/mm3, and more than 3 groups of enlarged lymph nodes
  • Stage C: Hemoglobin < 10 g/dL, platelets < 100,000/mm3, and any number of groups of enlarged lymph nodes  

Why Staging Matters for CLL?

Staging helps doctors predict disease progression and decide on potential treatments. Treatment is typically considered if a patient progresses to Rai stage II or beyond. Other reasons to consider treatment include rapid white blood cell count increases or the manifestation of symptoms such as fevers, night sweats, and weight loss.

Once CLL is diagnosed, according to how it presents in each patient, it can be described as: 

  • Asymptomatic: Has few or no symptoms.
  • Symptomatic or progressive: There are significant changes in blood counts or other symptoms affecting quality of life.
  • Recurrent: The leukemia came back after a period of time in which it could not be detected.
  • Refractory: Even with treatment, the leukemia is not responding, and abnormal cells keep showing up in tests; this can be more challenging to treat.

What Other Factors Can Influence CLL Prognosis?

Apart from Rai stages, the genetic features of CLL cells can influence how the disease progresses. These factors may be added to future CLL staging systems. Two of the main genetic mutations doctors look for in CLL cells are: 

  • Immunoglobulin Heavy Gene mutation (IGHV): If the IGHV gene is mutated, the CLL cell is older, and the disease typically progresses slowly. If IGHV is unmutated, the CLL cell is young, and the disease may progress quickly. 
  • Del(17p)/TP53 mutation: The TP53 gene (located on chromosome 17) is in charge of creating protein 53 (p53), which helps suppress cell tumor growth, repair cell DNA, and instructs cells beyond repair to die. If CLL patients have either a deletion or mutation of the TP53 gene, they may require treatment at the time of diagnosis as their disease might progress and grow more quickly. 

CLL patients can talk with their CLL specialist about any concerns related to their disease's stage and treatment options

If you want to stay updated on CLL treatment advancements and news, join our newsletter!

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The author Megan Heaps

about the author
Megan Heaps

Megan joined HealthTree in 2022. As a writer and the daughter of a blood cancer patient, she is dedicated to helping patients and their caregivers understand the various aspects of their disease. This understanding enables them to better advocate for themselves and improve their treatment outcomes. In her spare time, she enjoys spending time with her family. 

Thanks to our HealthTree Community for Chronic Lymphocytic Leukemia Sponsors:

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