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For most cancers, staging is the process of finding out how far the cancer has spread. Stages are often useful because they can help guide treatment and determine a person's outlook. Most types of cancer are staged based on the size of the tumor and how far the cancer has spread.
Chronic lymphocytic leukemia (CLL), on the other hand, does not usually form tumors. It's generally in the bone marrow and blood. And, in many cases, it has spread to other organs such as the spleen, liver, and lymph nodes by the time it's found. The outlook for a person with CLL depends on other information, such as the results of lab test and imaging tests.
A staging system is a standard way for the cancer care team to describe cancer. There are 2 different systems for staging CLL:
Both of these staging systems are helpful and have been in use for many years.
The Rai system is based on lymphocytosis. The patient must have a high number of lymphocytes in their blood and bone marrow that isn't linked to any other cause (like infection).
For a diagnosis of CLL, the overall lymphocyte count does not have to be high, but the patient must have at least 5,000/mm3 monoclonal lymphocytes (sometimes called a monoclonal lymphocytosis). Monoclonal means that the cancer cells all came from one original cell. This causes them to have the same chemical pattern which can be seen with special testing.
This system divides CLL into 5 stages based on the results of blood tests and a physical exam:
Doctors separate the Rai stages into low-, intermediate-, and high-risk groups when determining treatment options.
These risk groups are used later in Treatment of Chronic Lymphocytic Leukemia.
In the Binet staging system, CLL is classified by the number of affected lymphoid tissue groups (neck lymph nodes, groin lymph nodes, underarm lymph nodes, spleen, and liver) and by whether or not the patient has anemia (too few red blood cells) or thrombocytopenia (too few blood platelets).
Along with the stage, there are other factors that help predict a person's outlook. These factors are not part of formal staging systems (at least at this time), but are often taken into account when looking at possible treatment options.
Certain prognostic factors such as the presence or absence of ZAP-70, CD38, and a mutated gene for IGHV help divide cases of CLL into 2 groups, slow growing and fast growing. People with the slower growing kind of CLL tend to live longer and may be able to delay treatment longer as well.
There is no standard staging system for hairy cell leukemia.
Some people have monoclonal lymphocytes in their blood, but not enough to make the diagnosis of CLL. If someone has less than 5,000 monoclonal lymphocytes (per mm3), normal counts of red blood cells and platelets, and no enlarged lymph nodes (or enlarged spleen), they have a condition called monoclonal B-lymphocytosis (MBL). MBL doesn’t need to be treated, but about one patient of every 100 with this condition will go on to need treatment for CLL.
The cancer cells of small lymphocytic lymphoma (SLL) and CLL look the same under the microscope and have the same marker proteins on the surface of the cells. Whether someone is diagnosed with SLL or CLL depends largely on the number of lymphocytes in the blood. To be diagnosed with CLL, there must be at least 5,000 monoclonal lymphocytes (per mm3) in the blood. For it to be called SLL, the patient must have enlarged lymph nodes or an enlarged spleen with fewer than 5 ,000 lymphocytes (per mm3) in the blood. Still, since SLL and CLL can be treated the same, the difference between them isn't really important.
Our team is made up of doctors and oncology certified nurses with deep knowledge of cancer care as well as journalists, editors, and translators with extensive experience in medical writing.
Boddu P, Ferrajoli A. Prognostic Factors in the Era of Targeted Therapies in CLL. Curr Hematol Malig Rep. 2018;13(2):78-90.
CLL Society, Inc. Prognostic Factors in CLL. Accessed at https://cllsociety.org/2017/09/prognostic-factors-cll/ on April 13, 2018.
CLL Society, Inc. Rai Staging of CLL (chronic lymphocytic leukemia). 2/1/16. Accessed at https://cllsociety.org/2016/03/rai-staging-cll-chronic-lymphocytic-leukemia/ on April 13, 2018.
Leukemia & Lymphoma Society. Chronic lymphocytic leukemia Disease Complications. Accessed at www.lls.org/leukemia/chronic-lymphocytic-leukemia/disease-complications on April 13, 2018.
National Cancer Institute. Chronic Lymphocytic Leukemia Treatment (PDQ®)–Health Professional Version. February 7, 2018. Accessed at www.cancer.gov/types/leukemia/hp/cll-treatment-pdq on April 13, 2018.
National Cancer Institute. Hairy Cell Leukemia Treatment (PDQ®)–Patient Version. April 6, 2016. Accessed at www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq#section/_1 on April 13, 2018.
National Comprehensive Cancer Network, Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma, Version 5.2018 -- March 26, 2018. Accessed at www.nccn.org/professionals/physician_gls/pdf/cll.pdf on April 13, 2018.
Radiopaedia.org. Binet staging system for chronic lymphocytic leukaemia. Accessed at https://radiopaedia.org/articles/binet-staging-system-for-chronic-lymphocytic-leukaemia on April 13, 2018.
Strati P, Shanafelt TD. Monoclonal B-cell lymphocytosis and early-stage chronic lymphocytic leukemia: diagnosis, natural history, and risk stratification. Blood. 2015;126:454-462.
Last Revised: May 10, 2018