Diagnosing Chronic Lymphocytic Leukemia (CLL) Stages

Diagnosing CLL Stages

Learn about the different CLL stages and how they are determined. In addition, discover how staging impacts treatment.

Diagnosing Chronic Lymphocytic Leukemia (CLL) Stages

When diagnosed with Chronic Lymphocytic Leukemia (CLL), the doctor will also assess how far the disease has progressed. Determining the progression of CLL is called staging.

CLL has two commonly used staging systems: the Binet staging system and the Rai staging system. Keep reading to learn more about these two systems and what they mean for CLL treatment. 

What is CLL?

Chronic Lymphocytic Leukemia is a form of blood cancer that starts in lymphoid cells, which are inside the bone marrow, and eventually spreads into the blood. Although CLL is one of the most common forms of Leukemia, it is slow-growing and typically not diagnosed until later in life.

Read CLL 101 to learn more about the basics of CLL and how to identify its symptoms.

What are Cancer Stages?

Part of a cancer diagnosis typically involves staging the cancer through a series of examinations and diagnostic tests. Cancer stages are a way to describe the state of cancer. It examines how far cancer has progressed by assessing:

  • Your cancer’s location and size
  • How far the cancer may have spread into nearby tissues
  • If the cancer has spread to lymph nodes or distant parts of the body

Cancer stages are important because they allow your care team to compare your cancer to others who have been in similar situations. Staging can help formulate the treatment options and assist in giving a prognosis.

Staging Systems for CLL

Most forms of cancer are staged based on the tumor size and how far the cancer has spread. However, CLL does not typically form tumors and has usually spread via the blood by the time it gets diagnosed. Therefore, a different type of staging system is required for CLL.

The three commonly accepted staging systems for CLL include:

  • Rai system – widely used in the United States
  • Binet system – commonly used in Europe

“[My doctor said] If your white count is elevated and you have no reason like an infection, it could be leukemia.”

Andrew S | Read More

Rai Staging System for CLL

The Rai staging system requires a patient to have lymphocytosis to be diagnosed with CLL. Lymphocytosis is a symptom in which a person has a high number of lymphocytes (a form of white blood cells) in their blood and bone marrow that is not linked with another cause. 

A patient must have at least 5,000/mm3 monoclonal lymphocytes to be diagnosed with CLL under the Rai staging system. A monoclonal lymphocyte means that all the lymphocytes can be traced back to one original cell due to their chemical pattern.

In all stages of the Rai Staging System, a patient has lymphocytosis. In addition, the stages differ by the following symptoms:

  • Stage 0 – No additional symptoms other than lymphocytosis
  • Stage I – Enlarged lymph nodes and lymphocytosis, but no other symptoms.
  • Stage II – Enlarged spleen, potentially an enlarged liver and lymph nodes, and lymphocytosis.
  • Stage III – Red blood cell counts are low (anemia). In addition, the spleen, liver, and liver may be enlarged, and lymphocytosis.
  • Stage IV – Platelet counts are low (thrombocytopenia), and you may have the symptoms from the other stages.

When determining treatment options for CLL, a doctor will use your stage to determine the risk that CLL poses to your health. The stages are broken into three risk levels:

  • Low risk – Stage 0
  • Intermediate risk – Stages I & II
  • High risk – Stages III & IV

The higher the risk, the more intensive the treatment plan will be. However, all stages of CLL should be monitored closely and treated when appropriate.

woman hiking with scraped leg and arm

Because CLL is usually slow-growing, it was a watch and wait situation. I had labs and saw my oncologist every 3 months to see if any lymph nodes were enlarging, talk about any symptoms and look at my lab work. 

Lacey B | Read More

Binet Staging System for CLL

The Binet Staging System is more commonly used to assess the progress of CLL in Europe. The system looks at how many different tissues containing lymph nodes have been impacted and if a patient has a low red blood cell or platelet count. 

The Binet Staging System is broken into three stages:

  • Stage A – Less than three areas with lymphoid tissue are enlarged, and red blood cell and platelet count are normal.
  • Stage B – Three or more areas with lymphoid tissues are enlarged, and red blood cell count and platelet count are average.
  • Stage C – red blood cell or platelet count is low, and one or more areas of lymphoid tissue are enlarged.

As you may have noticed, the main difference between the two staging systems is that the Binet System evaluates progression by the number of areas with lymphoid tissues that are enlarged. At the same time, the Rai System focuses on which of the tissues have been impacted. Both view low platelet and red blood count as the furthest progression of CLL.

“Everybody’s a little bit different. You’ll see some people diagnosed with CLL, and they’ve had it longer than I’ve been a doctor, and they’re doing fine with it. Their white count is the same as it has been for the last couple of decades.”

Dr. Kerry Rogers | Read More

CLL International Prognostic Index (CLL-IPI)

The CLL International Prognostic Index was released by a large working group in 2016. While the index isn’t a staging system, it is a prognostic tool that helps guide doctors on how to proceed with treatment. 

The index uses genetic, biochemical, and clinical parameters to form a scoring system that breaks patients into four prognostic categories. The prognostic factors include:

  • TP53 deleted or mutated =4 points
  • Unmutated IGHV = 2 points
  • Serum beta-2 microglobulin concentration > 3.5 mg/L = 2 points
  • Rai Stage I – V or Binet Stage B – C = 1 point
  • Patient age > 65 years = 1 point

A patient receives the corresponding points for each prognostic factor they have, and they are then totaled up to fall into one of the four risk categories. The risk categories and their associated treatment recommendations include:

  • Low Risk (0-1) – do not treat
  • Intermediate Risk (2-3) – only treat if the patient is highly symptomatic
  • High Risk (4-6) – treat unless the patient is asymptomatic
  • Very High Risk (7-10) – use novel agents or treatment in a clinical trial rather than chemotherapy

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