Chronic Lymphocytic Leukemia (CLL)

Chronic lymphocytic leukemia (CLL) is a type of cancer that starts from cells that become certain white blood cells (called lymphocytes) in the bone marrow. The cancer (leukemia) cells start in the bone marrow but then go into the blood. In CLL, the leukemia cells often build up slowly over time, and many people don't have any symptoms for at least a few years. In time, the cells can spread to other parts of the body, including the lymph nodes, liver, and spleen.

CLL types

Doctors have found that there seem to be 2 different kinds of CLL

• One kind of CLL grows very slowly and so it may take a long time before the patient needs treatment.
• The other kind of CLL grows faster and is a more serious disease.

The leukemia cells from these 2 types look alike, but lab tests can tell the difference between them. The tests look for proteins called ZAP-70 and CD38. If the CLL cells contain low amounts of these proteins, the leukemia tends to grow more slowly

Diagnostics

A full blood count with morphological examination of the peripheral blood film is essential. An immunophenotype of CD20, CD19, and CD5 positivity (usually also with CD23 positivity) to document the characteristic CLL phenotype by flow cytometry, is also required to differentiate CLL from other lymphoproliferative disorders. A bone marrow is only performed to assess marrow reserves and genetic analysis prior to therapy, and after treatment completion to assess response. After initial therapy, patients in remission but with detectable minimal residual disease by flow cytometry in marrow or blood predicts earlier relapse and shorter progression free and overall survival. Flow cytometry requires a significant skill set and training.

Causes

In most cases, doctors don't know what causes CLL. You're more likely to get it if:

• You have a parent, sibling, or child who has CLL.
• You're middle-aged or older.
• You're a white man.
• You have relatives who are either Eastern European or Russian Jews.
• If you were exposed to Agent Orange, an herbicide widely used during the Vietnam War, your chances of getting CLL may also be higher.

Symptoms

You may have no symptoms for a while. Over time, you may have:

• Swollen lymph nodes in your neck, armpits, stomach, or groin. Lymph nodes are pea-sized glands in these and other areas of your body.
• Shortness of breath
• Pain or fullness in your stomach, which may be because the disease has made your spleen bigger
• Fatigue
• Night sweats
• Fever and infections
• Loss of appetite and weight

Treatment

If you have early stage CLL, you probably don't need treatment. Studies show that it doesn't help, as with some other cancers. Even so, you should keep up with all your doctor visits. Your doctor will closely check to make sure your condition hasn't changed. You may start treatment if your doctor notices a change, like the number of lymphocytes in your blood growing quickly, a decline in the number of your red blood cells, or swelling in a lymph node gets bigger.

Your treatment may include:

Chemotherapy

You may get this by pill, shot, or IV. Fludara (fludarabine) tends to work best to treat CLL. But doctors often combine two or more drugs that work in different ways to kill the cancer cells.

Immunotherapy

This uses lab-made antibodies, disease fighters, to attack the cancer without harming normal cells. It's often used with chemotherapy as a first treatment.

Radiation therapy

This type of treatment uses high-energy rays, such as X-rays, to destroy cancer cells. You probably won't need it, unless your doctor recommends it to shrink swelling in a lymph node, spleen, or other organ that's causing pain.

Second-line therapy

Venetoclax (Venclexta) is given to adults with a 17p deletion who have received at least one previous treatment for CLL.

Surgery

It's rare, but if chemotherapy or radiation doesn't reduce an enlarged spleen, doctors recommend surgery to take it out.

Researchers are studying new combinations of drugs and ways of treating CLL to help people stay disease-free longer. One such treatment combines chemotherapy with a stem cell transplant. Chemotherapy destroys cancer cells but also damages some healthy cells in the bone marrow. The stem cell transplant supplies healthy young cells to help rebuild your immune system. These aren't the "embryonic" stem cells that you may have heard about. They come from a donor's bone marrow.