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How Chronic Lymphocytic Leukemia Stages Are Diagnosed

Updated on July 01, 2022
Medically reviewed by
Todd Gersten, M.D.
Article written by
Emily Wagner, M.S.

  • Chronic lymphocytic leukemia (CLL) is staged based on many factors including blood cell counts and whether lymph nodes, spleen, and other tissues are enlarged.
  • Genetic mutations and other abnormalities in cancer cells help doctors predict prognosis.
  • Based on your risk factors, your doctors will determine which treatment options to recommend.

When you’re first diagnosed with chronic lymphocytic leukemia, your doctor will run several tests to determine the stage of your cancer. Knowing the stage helps determine what kind of treatment you will receive and the outlook for your diagnosis.

Traditionally, cancers are staged by the size of the tumor and how much the cancer has spread throughout the body. However, CLL is a blood cancer and does not usually form tumors. Instead, the leukemia cells can be found in the blood-forming cells of the bone marrow. It can also spread to the liver, lymph nodes, and spleen.

Staging Systems for CLL

Staging systems are standardized ways for doctors to describe cancer and make a diagnosis. When diagnosing CLL, doctors can use two staging systems. The Rai system is commonly used in the United States. The Binet system is commonly used in Europe.

Rai Staging System

The Rai staging system divides CLL cases into five stages based on high white blood cell (WBC) counts and other factors. Lymphocytes are WBCs of the immune system that help fight infection. In leukemia and other health conditions (such as arthritis or lymphoma), the body makes too many lymphocytes. This can lead to lymphocytosis, a high WBC count.

When diagnosing CLL, a health care specialist must observe a lymphocyte count elevated to a certain number of monoclonal B cells. “Monoclonal” describes cancer cells that all came from one original cell. B cells are specialized immune cells that are responsible for making antibodies. Having too many monoclonal B cells is called monoclonal B-cell lymphocytosis.

The Rai staging system divides CLL into five stages, 0 through 4, based on many factors. These include the presence of:

  • Lymphocytosis
  • Enlarged liver (hepatomegaly)
  • Enlarged spleen (splenomegaly)
  • Enlarged lymph nodes
  • Decreased number of platelets (thrombocytopenia)
  • Decreased red blood cell (RBC) count (anemia)

The stages can also be broken down into three risk groups: low-risk, intermediate-risk, and high-risk.

Stage 0

In stage 0 CLL, there is lymphocytosis and no enlargement of the liver, spleen, or lymph nodes. Blood test results show that RBC and platelet counts are near normal. Stage 0 is low-risk.

Stage 1

In stage 1 CLL, there is lymphocytosis, and the lymph nodes are enlarged. The liver and spleen are not enlarged, and blood test results show that RBC and platelet counts are near normal. Stage 1 is intermediate-risk.

Stage 2

In stage 2 CLL, there is lymphocytosis and the spleen is enlarged. The liver and lymph nodes may or may not be enlarged, and blood test results show that RBC and platelet counts are near normal. Stage 2 is also intermediate-risk.

Stage 3

In stage 3 CLL, there is lymphocytosis, and the liver, spleen, and lymph nodes may or may not be enlarged. Blood test results show that RBC counts are low, indicating anemia, and platelet counts are near normal. Stage 3 is high-risk.

Stage 4

In stage 4 CLL, there is lymphocytosis and the liver, spleen, and lymph nodes are enlarged. Blood test results show that RBC counts may be low or near normal, and platelet counts are low, indicating thrombocytopenia. Stage 4 is high-risk.

Binet Staging System

The Binet staging system divides CLL into three stages — A, B, and C — based on how many different tissues containing lymph nodes (found in the groin, neck, underarms, liver, and spleen) are affected, and whether the person has low RBC or platelet levels.

Stage A

Stage A CLL is diagnosed when less than three areas of lymphoid tissue are enlarged and there is no anemia or thrombocytopenia.

Stage B

Stage B CLL is diagnosed when three or more areas of lymphoid tissue are enlarged and there is no anemia or thrombocytopenia.

Stage C

Stage C CLL is diagnosed when there is anemia or thrombocytopenia in addition to any number of lymphoid tissue areas being enlarged.

Do you know what stage of chronic lymphocytic leukemia you have?
Click
here to share your experience in the comments below.

CLL International Prognostic Index

The CLL International Prognostic Index (CLL-IPI) can be used to help predict a person’s disease prognosis (outlook). These factors are not taken into consideration during traditional staging, but they can be used to determine the right treatment options. Depending on the specific characteristics of your leukemia, existing CLL treatments or clinical trials might provide the most promising treatment.

There are two types of factors used in the CLL-IPI: adverse prognostic factors and favorable prognostic factors. Adverse factors are generally associated with shorter survival time, and favorable factors are associated with longer survival.

Adverse prognostic factors include:

  • Older age (65 and up)
  • Three copies of chromosome 12 in CLL cells (known as trisomy 12)
  • Missing pieces of chromosomes 11 or 17
  • High levels of certain substances in the blood, such as beta-2 microglobulin
  • CLL cells that are missing the TP53 gene
  • Widespread replacement of healthy bone marrow cells with CLL cells (known as diffuse pattern of bone marrow involvement)
  • Increased number of an early form of lymphocytes (known as prolymphocytes) found in the blood
  • Large number of CLL cells with increased amounts of the proteins ZAP70 or CD38
  • CLL cells with an unmutated gene for an immune-system protein called immunoglobulin heavy chain variable region (IGHV)

Favorable prognostic factors include:

  • Small number of cells that have increased amounts of ZAP70 or CD38
  • CLL cells with a mutation in the IGHV gene
  • Missing pieces of chromosome 13
  • Localized replacement of bone marrow cells with CLL cells (known as nondiffuse pattern of bone marrow involvement)

Some factors, like ZAP70, CD38, and IGHV mutations, can help doctors divide CLL into slow-growing or fast-growing cases. People with early-stage, slow-growing CLL may be able to delay treatment and go on “watchful waiting” with regular monitoring. Slow-growing CLL tends to have a better prognosis, and people tend to live longer.

Talk With Others Who Understand

MyLeukemiaTeam is the social network for people with leukemia and their loved ones. Every day, more than 11,000 members come together to ask questions, give advice, and share their stories with others who understand life with leukemia.

Do you know what stage of chronic lymphocytic leukemia you have? Share your experience in the comments below, or start a conversation by posting in Activities.

All updates must be accompanied by text or a picture.
Todd Gersten, M.D. is a hematologist-oncologist at the Florida Cancer Specialists & Research Institute in Wellington, Florida. Review provided by VeriMed Healthcare Network. Learn more about him here.
Emily Wagner, M.S. holds a Master of Science in biomedical sciences with a focus in pharmacology. She is passionate about immunology, cancer biology, and molecular biology. Learn more about her here.

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