Chronic Lymphocytic Leukemia

Overview

Getting Started

Chronic lymphocytic leukemia (CLL) is a type of hematologic (blood) cancer. It is usually slow-growing and, at this time, is a condition that is managed, not cured. Though a CLL diagnosis is life-changing, the landscape of CLL research is very promising. More effective drugs and combination treatments are now available for this chronic blood cancer.

Moving forward with confidence is easier with a plan. You may consider choosing a hematologist with expertise in treating CLL. Specialists better understand CLL and how it responds to treatment. They can offer treatment plans that help many people live longer and with a better quality of life.

Your diagnosing physician should be able to give you a referral. If you are not located near a CLL specialist and traveling for treatment is not an option, look for a CLL specialist who will consult with your doctor and offer a second opinion. This can confirm the suggested treatment plan or add other options to consider.

Commit to taking an active role in your care. While your doctor monitors your test results, you will need to communicate honestly about symptoms and treatment-related side effects. It will also be up to you to keep in-person and telehealth appointments and make wise nutrition and lifestyle choices.

Explaining CLL

Leukemia begins in the blood and bone marrow (the soft, spongy center of some bones). It occurs when the bone marrow makes too many white blood cells. These white blood cells don’t fight infection like healthy white blood cells do, which makes an individual susceptible to repeated infections.

CLL develops from a type of white blood cell known as a lymphocyte. Lymphocytes make up lymphoid tissue, which is found in the lymph nodes, thymus, spleen, tonsils and other parts of the body. They are a part of the immune system.

CLL begins when mature lymphocytes change and multiply uncontrollably. They grow at a faster rate than usual, and they do not die when they should, causing them to build up in the blood or bone marrow. This leads to an accumulation of them in the blood, bone marrow, lymph nodes and spleen that interferes with the normal production of healthy cells, including red blood cells that carry oxygen; white blood cells that fight infection; and platelets that help blood to clot.

Except for lymphomas, blood cancers typically do not grow into tumors. Instead, abnormal lymphocytes build up to become CLL cancer cells that circulate in the bloodstream and can spread through the lymphatic system to other parts of the body. However, patients with CLL often have larger than normal lymph nodes that can appear as lumps in the neck, armpits or groin regions.

Diagnosing & Planning

Your doctor will perform a physical examination to check for pain, enlarged lymph nodes and size irregularity in your spleen and other organs. Blood test results help determine your prognosis (outlook).

To identify your CLL subtype, your doctor will order genetic testing, also called molecular profiling, on a small sample of fluid or tissue. The results of these tests, including a fluorescence in situ hybridization (FISH) test, your TP53 gene mutation status, immunoglobulin heavy chain variable (IGHV) status, and serum B2-microglobulin concentration, help identify specific gene abnormalities or mutations, proteins and changes in chromosomes.

The Modified Rai Staging System and the Chronic Lymphocytic Leukemia International Prognostic Index (CLL-IPI) further assist your doctor (see Tables 1 and 2). Modified Rai divides CLL into five stages according to the number of lymphocytes and other cells in the blood and the presence of certain abnormalities noticed on the physical examination. The “risk” characteristic assigned in staging is associated with the predicted rate of growth for the disease. This offers information about how CLL may progress. CLL-IPI is used to predict the risk of disease recurrence and overall survival and incorporates the Rai stage.

Your doctor will also consider the clinical stage, your age and other criteria.

This information will help determine the treatment that will be most effective.



Table 1. Modified Rai Staging System

Stage Risk Description
Stage 0 Low Lymphocytosis only (the blood contains a significant number of lymphocytes).
Stage I Intermediate + Adenopathy (the blood contains a significant number of lymphocytes, and lymph nodes are enlarged).
Stage II Intermediate + Enlarged spleen and/or liver (the blood contains a significant number of lymphocytes, the spleen and/or liver is enlarged, and the lymph nodes may or may not be enlarged).
Stage III High Lymphocytosis + Hgb < 11g/dL (the blood contains a significant number of lymphocytes, and the hemoglobin is less than 11 grams per deciliter).
Stage IV High Lymphocytosis + Plt < 100,000/?L (the blood contains a significant number of lymphocytes, and the platelet count is less than 100,000 microliters).


Table 2. Chronic Lymphocytic Leukemia International Prognostic Index (CLL-IPI)

The CLL-IPI assigns weighted values for each of the following risk factors.

Risk factors Points
TP53 mutation 4
IGHV unmutated 2
Serum B2-microglobulin more than 3.5 mg/L 2
Rai Stages I-IV --
Age older than 65 years 1


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