Knowing the stage of your cancer after a diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) helps your healthcare team decide on the best treatment options and the likelihood that treatment will work. CLL and SLL are usually considered low-grade forms of non-Hodgkin lymphoma (NHL). Typically, NIH is staged based on how many lymph nodes are affected and where they are in the body.

Because SLL is closely related to CLL, doctors use the same staging systems for both. Keep reading to learn more about how CLL/SLL is staged.

What Is Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma?

CLL and SLL are actually the same disease but in different parts of the body. Both CLL and SLL are indolent (slow-growing) blood cancers that begin when B cells (a type of white blood cell also known as B lymphocytes) begin to grow out of control and build up over time.

When cancerous B cells collect in the blood and bone marrow, the disease is called CLL. This disease is classified as leukemia because it involves leukocytes (also known as white blood cells). When B cells collect in the lymph nodes, the disease is called SLL. SLL is classified as a lymphoma because it involves the lymphatic system (a group of organs, vessels, and tissues that help fight infection).

What Are the Stages of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma?

The stage of CLL or SLL helps doctors understand how far the disease has progressed and guides treatment decisions. The type of staging system used depends on whether you have chronic lymphocytic leukemia or small lymphocytic lymphoma.

There are two main staging systems for CLL — Rai and Binet. The Rai system is the one most used in the United States, while the Binet system is more common in Europe. Both systems rely on a physical exam and blood tests, including a complete blood count, especially to measure the number of lymphocytes.

SLL is staged using the Lugano classification system, which is also used for other types of NHL. This system helps determine how far the cancer has spread in the body. Because SLL mainly affects the lymph nodes, the Lugano classification can be used. However, since CLL primarily affects blood and bone marrow, other systems are more appropriate.

Rai System

The Rai staging system uses five stages (0 through 4) based on the presence or absence of several factors:

• Lymphocytosis — Elevated (high) white blood cell count
• Hepatomegaly — Enlarged liver
• Splenomegaly — Enlarged spleen
• Lymphadenopathy — Enlarged lymph nodes
• Anemia — Low red blood cell (RBC) count or hemoglobin concentration
• Thrombocytopenia — Low platelet count

Stage 0 (Low Risk)

A person with stage 0 CLL has lymphocytosis but no enlargement of the liver, spleen, or lymph nodes. RBC and platelet counts are near normal.

Stage 1 (Intermediate Risk)

In stage 1, lymphocytosis and lymph node enlargement are present. The liver and spleen aren’t enlarged. RBC and platelet counts are near normal.

Stage 2 (Intermediate Risk)

In stage 2, a person has lymphocytosis and an enlarged spleen. The liver and lymph nodes may or may not be enlarged. RBC and platelet counts are near normal.

Stage 3 (High Risk)

Lymphocytosis is present and RBC counts are low in stage 3. The liver, spleen, and lymph nodes may or may not be enlarged, and platelet counts remain near normal.

Stage 4 (High Risk)

Stage 4 involves lymphocytosis and low platelet counts. The liver, spleen, and lymph nodes may be enlarged. RBC counts may be low or near normal.

Binet System

The Binet staging system has three stages (A, B, and C) based on the following factors:

• Number of enlarged lymphoid tissue areas, including lymph nodes in the neck, underarms, or groin, as well as the spleen or liver
• Anemia
• Thrombocytopenia

Stage A

Binet stage A corresponds to Rai stages 0, 1, and 2. Fewer than three areas of lymphoid tissue are enlarged, and RBC and platelet counts are normal.

Stage B

Binet stage B corresponds to Rai stages 1 and 2. Three or more areas of lymphoid tissue are enlarged, but RBC and platelet counts are normal.

Stage C

Binet stage C corresponds to Rai stages 3 and 4. RBC and platelet counts are low, and any number of lymphoid tissue areas are enlarged.

Lugano Classification

SLL is usually staged the same way as other types of NHL, using the Lugano classification system. This system has four stages — 1 through 4, often noted with Roman numerals (I through IV) — based on the number and location of tumors in the lymph nodes and whether tissue outside the lymphatic system is involved.

The body is divided into two areas — above and below the diaphragm (the dome-shaped muscle that separates the chest from the abdomen). If organs other than the lymph nodes, spleen, or tonsils and adenoids are involved, an “E” for “extranodal involvement” is added to the stage number.

Stage 1 (Limited)

In stage 1, either just one group of lymph nodes or one organ outside the lymphatic system is involved (stage 1E).

Stage 2 (Limited)

In stage 2, either two or more groups of lymph nodes on the same side of the diaphragm or one group of lymph nodes and one nearby organ are involved (stage 2E).

Stage 3 (Advanced)

In stage 3, lymph nodes on both sides of the diaphragm or above the diaphragm and the spleen are involved.

Stage 4 (Advanced)

In stage 4 SLL has spread to at least one organ outside the lymphatic system, such as the bone marrow, liver, or lungs.

What Is the Prognosis for Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma?

Staging alone doesn’t provide a full picture of what to expect with CLL/SLL. Doctors also use other important details, called prognostic factors, to help determine the best treatment and estimate how the disease will progress.

CLL International Prognostic Index

The CLL International Prognostic Index (CLL-IPI) combines several key factors to produce a risk score (1-10 points) that categorizes a person’s risk as low, intermediate, high, or very high. The CLL-IPI considers:

• Age over 65 years — 1 point
• Stage (Rai 1 to 4 or Binet B or C) — 1 point
• Beta-2 microglobulin blood level above 3.5 milligrams per liter — 2 points
• Tumor protein 53 (TP53) gene mutation or chromosome 17p deletion — 4 points
• No immunoglobulin heavy chain (IGHV) gene mutation — 2 points

CLL-IPI risk categories are:

• Low risk — 0-1 point
• Intermediate risk — 2-3 points
• High risk — 4-6 points
• Very high risk — 7-10 points

People with low-risk CLL usually do not need treatment right away. Treatment may also be delayed for those in the intermediate- or high-risk groups if they don’t have any symptoms. Instead, doctors may recommend active surveillance (watchful waiting), which includes regular checkups and tests to monitor if CLL is progressing.

People with very-high-risk CLL often start treatment sooner. Targeted therapies, which attack cancer cells in a more precise way than chemotherapy, are usually recommended. These treatments often work better and cause fewer side effects. Some people may choose to join clinical trials to try new treatments.

Other Prognostic Factors

Doctors also look at other factors to help predict how the disease may behave. These include both adverse prognostic factors (linked to faster-growing cancer or shorter survival) and favorable ones (linked to slower-growing cancer or longer survival).

Adverse prognostic factors include:

• Older age (65 and up)
• Cancer cells with certain genetic abnormalities
• No IGHV gene mutation (change)
• SF3B1 gene mutation
• High levels of certain proteins (ZAP-70 and CD38)
• High blood levels of beta-2 microglobulin
• High blood levels of prolymphocytes (immature white blood cells)
• Diffuse (widespread) involvement in the bone marrow
• Lymphocyte doubling time of less than one year

Favorable prognostic factors include:

• Non-diffuse pattern of cancer involvement in bone marrow
• Presence of IGHV gene mutation
• Chromosome 13 deletion
• Low levels of ZAP-70 and CD38 proteins on cancer cells

Talk With Others Who Understand

MyLymphomaTeam is the social network for people with lymphoma and their loved ones. On MyLymphomaTeam, more than 21,000 members come together to ask questions, give advice, and share their stories with others who understand life with CLL/SLL.

Are you or a loved one living with CLL/SLL? Did you feel well informed when learning about your stage and prognosis? Share your experience in the comments below, start a conversation by posting on your Activities page, or connect with like-minded members in Groups.