Chronic lymphocytic leukemia (CLL) is a slow-growing cancer that usually takes hold in older adults when it hijacks the cells that will become white blood cells. The bone marrow creates ineffective lymphocytes that multiply and spread throughout the body. However, it can take some time for these cancer cells to proliferate and build up enough to start causing symptoms.
“The way CLL is diagnosed has changed in recent years,” Sameer Parikh, M.D., an assistant professor of medicine and oncology at the Mayo Clinic College of Medicine, said in a phone call with HealthCentral. “The vast majority, about three-quarters, of patients are found incidentally through bloodwork done during routine physicals. The other quarter have enlarged nodes in their neck or armpit area, or constitutional symptoms of weight loss, fatigue, or night sweats.”
Dr. Parikh continues on to say that most CLL patients who are diagnosed through routine bloodwork don’t need treatment right away.
“Treatment of asymptomatic CLL hasn’t translated into better outcomes,” Dr. Parikh said. “This is in contrast to what we’re told about cancer – find it and treat it early. But for CLL, this treatment may be more harmful than observation.”
According to Mazyar Shadman, M.D., attending physician at Seattle Cancer Care Alliance, assistant professor at University of Washington, and assistant member of Fred Hutch Cancer Research Center, in addition to the symptoms Dr. Parikh noted, other early CLL indicators often include frequent infections, fatigue and anemia, and trouble with blood clotting.
“Basically if a CLL patient has any of these symptoms or problems,” Dr. Shadman said in a phone call with HealthCentral, “that would be an indication for treatment.”
Each patient’s specific regimen depends on the characteristics of their cancer and the symptoms that need to be alleviated. The most common CLL complications include:
Frequent Infections – Because these white blood cells are ineffective and unable to create antibodies, infections can thrive in patients with CLL. Chemotherapy that reduces blood cell production may complicate problems by further reducing the number of white blood cells. Infections are most commonly found in the upper or lower respiratory tract.
Because of this increased risk of infection, doctors often recommend certain vaccines. Sometimes antibiotic therapy is necessary to treat bacterial or fungal infections. Some patients may receive immunoglobulin injections (a protein made by white blood cells that helps fight infection) and antibody therapy.
Increased Risk of Other Cancers – Some CLL patients develop secondary cancers. The most commonly seen among CLL patients are acute myeloid leukemia, myelodysplastic syndromes, melanoma, gastrointestinal cancer, breast cancer, and lung cancer.
Somewhere between 2 and 10 percent of people with CLL develop a more aggressive cancer called diffuse large B-cell lymphoma (DLBCL) or Richter’s syndrome. Symptoms include significantly enlarged lymph nodes, fever and/or weight loss. These patients should be treated with chemotherapy regimens designed to treat DLBCL. An allogenic stem cell transplant may also be considered.
Immune System Problems – Low blood cell counts are common among CLL patients and treatment may include giving patients white blood cell growth factors like Neupogen or Leukine.
Some patients develop a condition called autoimmune cytopenia where their white blood cells mistakenly attack platelets or red blood cells. Prednisone or other cortisone-like steroids that reduce inflammation are often prescribed. And removal of the spleen may be considered for patients who do not respond to steroid therapy.
Tumor Flare – These reactions include painfully enlarged lymph nodes, elevated lymphocyte counts, enlarged spleen, low-grade fever, rash, and bone pain. Doctors often recommend steroids and antihistamines to manage the inflammation and rash.
While no one is sure what starts or stops the process of CLL, both Dr. Shadman and Dr. Parikh mentioned that chemotherapy agents are being used less in the treatment of CLL as new, novel treatments are showing greater results.
“This is truly a transformative time in treatment for CLL,” Dr. Parikh says. “We can deliver more effective treatment orally without complications. Current patients should be enrolled in clinical trials or with major medical centers where they have the latest options.”
As protocols for CLL transition away from chemotherapy, the questions and challenges that doctor’s face also change.
“Overall, we're moving to the novel agent’s era,” Dr. Shadman says. “And the big question in the field is, does it make sense to combine these good drugs together and obtain a very deep and very high quality remission in patients? Or is it better to go with one drug and, as long as the patient is tolerating it and responding to it, continue until it fails and then go to the next drug?”