Focusing on the Blood Count is Sometimes More Significant Than the Stage in CLL

Because a blood cancer is a disease that spreads throughout the body, the staging system for patients with chronic lymphocytic leukemia (CLL) is very different from solid tumor cancers. Once the CLL diagnosis is determined, doctors focus on the blood count to gauge how advanced the disease is and when treatment is necessary. Specifically they look at the abnormal increase in the number of lymphocytes (a type of white blood cells)  in the blood and marrow.

“It’s sometimes very confusing to patients,” says Dr. Nicole Lamanna, a leukemia specialist at Columbia University School of Medicine, because for many patients, treatment is not required for many years because CLL is such a slow-growing disease.

“Early stage disease typically is manifested by an elevated lymphocyte count in the blood without any other manifestations, and we actually call this Rai stage zero CLL,” Dr. Matthew Davids, associate director of the center for CLL at Dana-Farber Cancer Institute, told SurvivorNet.

Dr. Matthew Davids explains how doctors use the Rai system to stage CLL.

“When we can feel enlarged lymph nodes on the physical exam, that’s considered to be Rai stage 1 disease, and when we can feel an enlarged spleen or liver, we consider that Rai stage 2 disease.  All three of these stages, zero, 1, and 2, are typically considered to be early stages of CLL.”

Dr. Davids said that with advanced stages — 3 and 4 — the disease has started to infiltrate the bone marrow, which results in a low red blood cell count (anemia) or low platelets (thrombocytopenia). Typically, people at stage 3 and 4 do require treatment.

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

Still, the staging process can be a bit confusing to patients. Dr. Lamanna attributes this to the fact that it’s quite different from the staging process for solid tumor cancers — like breast or colon cancers — because CLL affects the blood.

“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 stressed that because the staging process can be confusing, the focus should be less on stage and more on if the CLL will require immediate treatment or an active surveillance approach.

How is Prognosis Determined?

There are a few tests that patients may be given to determine CLL prognosis and if treatment will be required. These include:

• Flow cytometry of blood or immunohistochemistry of lymph tissue or bone marrow
• FISH
• DNA sequencing
• CpG-stimulate karyotype
• Physical exams
• Blood tests: CBC with differential and a comprehensive metabolic panel

Dr. Davids explained that these tests can help determine which patients may be able to go significant periods of time with no treatment.

“When we have the full profile of these prognostic markers, it can be very helpful in terms of determining when a patient may need treatment … For example, patients with the chromosome 17 deletion or TP53 mutation often need treatment within a few months or a year or so of diagnosis. Whereas patients who have lower risk disease like chromosome 13 abnormalities or mutated IGHV may actually go for several years, if not decades, before requiring therapy,” Dr. Davids said.

If treatment is required, there have been some pretty incredible developments in the CLL treatment field over the past decade. Where chemotherapy was once the standard approach, doctors now have a handful of targeted therapies they may be able to use to control the cancer with fewer side effects.

Questions to Ask Your Doctor:

• How will my blood count be monitored and how often?
• Will my cancer require treatment immediately?
• What does the stage indicate about my prognosis?
• If needed, what are the treatment options?