Understanding the Staging System for CLL
- In the U.S., CLL is typically staged using something called the Rai system.
- The system is broken down into five levels, stage zero, one, two, three, and four.
- Stage zero is considered low risk for progression, while one and two are considered intermediate, and three and four are considered high.
- In many cases, patients who present with stage zero, one, or two can take a watchful waiting approach to treatment.
Most patients in the early-stage do not require any therapy and may be placed on observation for months, or even years. Advanced-stage means the disease has infiltrated the bone marrow, and caused low red blood cell count (anemia) or low platelets (thrombocytopenia). When the disease reaches more advanced stages, typically treatment is required.
Read MoreWhen is Treatment for CLL Needed?
"When the disease infiltrates the bone marrow and starts to result in low red blood cell count, which we call anemia, or low platelets, which we call thrombocytopenia, these are the advanced stages, 3 and 4, of CLL, and typically those do require treatment," Dr. Davids added.The five stages can also be condensed into three risk groups:
- Stage 0 has a low-risk of getting worse
- Stage 1 and 2 are intermediate risk
- Stage 3 and 4 have a high risk of progressing
Dr. Nicole Lamanna, a leukemia specialist at Columbia University School of Medicine, explained that the staging system for CLL can be a bit confusing to patients because it’s different from standard staging systems used for other common cancers — like colon or breast cancers.
A Different Type of Staging
“We don’t use staging [like] with other solid tumor cancers, where if the mass spread, it has very different implications about whether they can get surgery [or] what types of chemotherapy drugs for breast or colon,” Dr. Lamanna explained. “Because this is a blood disorder, people with CLL either need to be treated for it or they don’t. So, the staging is a little bit different to patients. They want to know, well, what stage am I? What does it mean?”
Dr. Lamanna said that because this system can be confusing to patients, and because CLL affects the blood, which circulates everywhere, the focus should be less on stage and more on if the CLL will require immediate treatment or an active surveillance approach.
It’s also important to note that not everyone with what’s considered low or intermediate risk disease will be able to put off treatment for an extended period of time.
“I’ll note that there are some scenarios, for example, patients with stage 1 or 2 disease who have markedly enlarged lymph nodes or spleens who may require therapy, even if their other blood counts are relatively normal appearing,” Dr. Davids said.
When a patient does not require immediate treatment, how often they’ll need to see their doctor for testing will vary depending on their individual disease and overall health.
Dr. Nicole Lamanna explains how patients are monitored after a CLL diagnosis.
“There are some people who will need treatment within a few years of their diagnosis and there other people who may not need treatment for five, ten years,” Dr. Lamanna explained. “So, following each individual’s blood counts over time is more telling than anything.
“Somebody can rapidly rise,” she contined. “Their white count can go from 50 to 100 to 200 in a short span. That means a lot. Because if they’re doing that over a few months, the likelihood is they’re probably gonna need therapy in the future, because their other good counts are going to decline. For other individuals, they may rise, but ever so slowly over years. So, knowing the tempo and history of what people’s blood counts are doing is the most important thing.”
Questions to Ask Your Doctor:
- What tests should I undergo to get my CLL prognosis?
- What is my Rai stage?
- Is an active surveillance approach a good idea for me?
- How often will I need to be monitored?
Learn more about SurvivorNet's rigorous medical review process.