LLS | Holding onto hope: Living with chronic lymphocytic leukemia

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Coping with chronic lymphocytic leukemia (CLL) can be a challenging and deeply emotional experience—both for the person diagnosed and for the friends or family members providing support. Recognizing how CLL progresses and understanding the symptoms that may appear as the disease advances can offer a sense of preparedness. At the same time, learning about available treatments, diagnostic procedures, and avenues for support can bring hope and a clearer path forward.

What is CLL?

Chronic lymphocytic leukemia (CLL) is a type of leukemia affecting white blood cells known as lymphocytes, which are crucial to the immune system, fighting off infections and diseases. In CLL, a genetic mutation occurs in a single lymphocyte, causing it to behave abnormally. Instead of maturing and functioning normally, the mutated cell begins to multiply uncontrollably, producing large numbers of abnormal lymphocytes. Over time, these cells accumulate in the blood and bone marrow, crowding out healthy cells and weakening the body’s ability to fight infections.

Unlike acute leukemias, which progress rapidly, CLL usually unfolds more slowly. Some people may not notice any problems at first and can feel reasonably well for years before requiring active treatment. For others, the disease can take a more active course (Eichhorst et al. 2020).

CLL stages

Researchers and healthcare professionals have long used specific systems to define CLL progression so they can better determine treatment strategies. The Rai system, popular in the United States, classifies CLL into five risk categories (Rai 0 through IV) based on factors like lymph node enlargement, spleen size, and whether anemia or low platelet counts are present. Lower stages typically involve fewer symptoms and are considered lower risk, while higher stages may indicate more significant blood cell count issues and greater disease burden (Rai et al. 1975).

Meanwhile, the Binet system, commonly used in Europe, divides CLL into stages A, B, and C. This classification hinges on the number of areas with enlarged lymphoid tissue (for example, lymph nodes or the spleen) and whether the patient exhibits anemia or low platelet counts. Stage A usually involves up to three areas of lymphoid enlargement with no anemia or low platelets; Stage B indicates more than three areas of enlargement; and Stage C includes anemia or low platelet counts (Binet et al. 1981). In 2016, experts introduced a new tool called the CLL International Prognostic Index (CLL-IPI). This model considers various biological and clinical factors—such as genetic changes in leukemia cells, certain gene mutations, and overall patient health—to better predict how the disease might progress (Molica et al. 2016).

CLL symptoms

One unique aspect of CLL is that it’s often discovered accidentally. Many individuals are diagnosed after a routine blood test for an unrelated issue reveals an elevated lymphocyte count. In earlier stages, people might notice few or no symptoms of illness. As CLL progresses, however, common symptoms can include:

Swollen lymph nodes in the neck, underarms, or groin
A lingering feeling of fullness beneath the ribs, sometimes due to an enlarged spleen
Persistent tiredness or fatigue
Recurring infections, fevers, or chills
Night sweats or unexplained weight loss

For those in advanced stages of CLL or nearing the end of life, additional symptoms may appear. These can include:

Decreased or darker urine output
Heart rate fluctuations
Cold or blotchy hands and feet
Changes in breathing patterns or shortness of breath
Drops in blood pressure
Unresponsiveness to others or delirium
Difficulty swallowing
Muscle jerks
Excessive bleeding (hemorrhage)

CLL testing and diagnosis

Before a treatment plan is decided, doctors conduct comprehensive evaluations to diagnose CLL. These can involve:

Health history and physical exams
Complete blood count (CBC)
Blood chemistry tests, bleeding, and clotting factors
Imaging (chest x-ray, CT scan, MRI, ultrasound)
Special laboratory tests to identify unique features of CLL cells, which look for specific proteins or markers on the cells and use certain dyes to highlight important details
Flow cytometry, which analyzes cells’ surface proteins to confirm the presence of CLL
Cytogenetic and molecular studies, which look for genetic abnormalities or mutations that can influence prognosis
Lymph node biopsy, which involves removing a small sample of lymph node tissue to confirm a diagnosis or clarify how far the disease has progressed

CLL treatment

Treatments for CLL depend on the stage of the disease, overall health, and a patient’s specific risk factors. A few primary approaches include:

Chemotherapy: Involves using drugs that travel throughout the body to kill or slow the growth of cancer cells. Combination chemotherapy regimens may be used for certain patients, while others might receive a gentler single-drug protocol.
Immunotherapy: Harnesses the power of the body’s own immune system. For instance, monoclonal antibodies latch onto specific markers on CLL cells, effectively flagging them for destruction by the immune system.
Targeted Drug Therapy: Uses medications designed to pinpoint certain vulnerabilities in leukemia cells—like proteins or enzymes the cancer cells need to survive. This approach can spare more healthy cells compared to some forms of chemotherapy.
Stem Cell Transplant: Also called a bone marrow transplant, this technique aims to replace diseased bone marrow with healthy stem cells. It can be an intense process, often reserved for younger or relatively fit individuals.
Radiation and Surgery: These methods are typically used in more specific circumstances, such as treating a localized mass or alleviating discomfort from enlarged lymph nodes or the spleen. Radiation uses high-energy beams to target affected areas, while surgery (like spleen removal) might be considered if it helps improve blood cell counts or reduce certain complications.

Prognosis and survival for CLL

People with CLL can have widely different experiences due to a number of factors, both prognostic (overall outcome) and predictive (effects from specific treatments). These include:

Age and stage: Older patients and those at higher Rai or Binet stages may face greater challenges, but individuals at any age can respond differently to treatments.
Sex: Biological males can sometimes have a slightly worse outcome compared to biological females.
Lymphocyte doubling time: This measures how fast the count of lymphocytes doubles, indicating how quickly the disease may be advancing.
Chromosome changes: Certain genetic deletions or abnormalities, such as deletion 17p or 11q, can suggest a more aggressive disease.
TP53 gene mutation: This plays a significant role in how CLL cells respond to therapy.
IGHV gene mutation: Generally, a mutated IGHV status is linked with a better prognosis than an unmutated IGHV.
Protein levels: Levels of proteins in the blood or on the surface of CLL cells can help doctors gauge disease aggressiveness.

LLS blood cancer research impact

The Leukemia & Lymphoma Society (LLS) supports people affected by blood cancers through research funding, patient services, and advocacy.

Advancing research: As the largest nonprofit funder of blood cancer research, LLS supports a wide spectrum of scientific efforts aimed at finding better treatments and cures. Their investments focus on breakthroughs that can significantly extend and improve patient lives.
Offering support: LLS is there for patients and families when they need help the most—providing financial assistance, guidance on clinical trial options, and one-on-one support to help navigate diagnoses and treatment plans.
Policy and advocacy: Through its Office of Public Policy (OPP), LLS advocates for faster development of new therapies and works to eliminate barriers that prevent patients from getting timely and affordable care.
Educating and empowering: LLS works to raise awareness about blood cancers and make trusted information accessible so patients, caregivers, and communities can make informed decisions with confidence.

Your generosity powers LLS’s mission. Every donation helps drive forward new discoveries and brings us closer to a future without blood cancer.

Conclusion

Living with CLL or caring for someone who has it can evoke worry and a desire for reliable information. Understanding how CLL is staged, learning to recognize symptoms, and exploring different treatment pathways can lessen uncertainty. With developments like the CLL-IPI bringing precision to diagnosis and treatment and ongoing research propelled by organizations such as LLS, there is a growing network of care and expertise to lean on.

About the author: Dr. Ali is a medical journalist and copywriter.

Sources

Eichhorst, B., T. Robak, E. Montserrat, P. Ghia, C.U. Niemann, A.P. Kater, M. Gregor, et al. 2020. “Chronic Lymphocytic Leukaemia: ESMO Clinical Practice Guidelines for Diagnosis, Treatment and Follow-up.” Annals of Oncology 32 (1): 23–33. https://doi.org/10.1016/j.annonc.2020.09.019.

Rai, K. R., A. Sawitsky, E. P. Cronkite, A. D. Chanana, R. N. Levy, and B. S. Pasternack. 1975. “Clinical Staging of Chronic Lymphocytic Leukemia.” Blood 46 (2): 219–34. https://doi.org/10.1182/blood.v46.2.219.219.

Binet, J. L., A. Auquier, G. Dighiero, C. Chastang, H. Piguet, J. Goasguen, G. Vaugier, et al. 1981. “A New Prognostic Classification of Chronic Lymphocytic Leukemia Derived From a Multivariate Survival Analysis.” Cancer 48 (1): 198–206. https://doi.org/10.1002/1097-0142(19810701)48:1.

Molica, Stefano, Tait D. Shanafelt, Diana Giannarelli, Massimo Gentile, Rosanna Mirabelli, Giovanna Cutrona, Luciano Levato, et al. 2016. “The Chronic Lymphocytic Leukemia International Prognostic Index Predicts Time to First Treatment in Early CLL: Independent Validation in a Prospective Cohort of Early Stage Patients.” American Journal of Hematology 91 (11): 1090–95. https://doi.org/10.1002/ajh.24493.

Holding onto hope: Living with chronic lymphocytic leukemia