Chronic lymphocytic leukemia (CLL) is the most common leukemia, accounting for about 15,000 new patients every year in the United States. CLL is a condition that people often live with for many years, so the prevalence is much higher. The disease is different from acute leukemias in that the indolent rate of progression offers most patients a favorable prognosis, and many patients will have normal life expectancy. That said, a small proportion CLL cases will progress rapidly and be life-threatening. As diagnostics have improved, clinicians are more commonly diagnosing CLL in younger patients and catching it in its earliest stages.
The goal when managing CLL is to employ treatment as needed to ensure that overall survival and quality of life are optimized. The initial question to consider after diagnosis is whether or not patients require treatment. Asymptomatic patients who do not have significant cytopenias or organ dysfunction do not appear to benefit from earlier treatment. These CLL patients can be observed, sometimes lifelong, without chemotherapy or immunotherapy. If treatment is required, there are many new therapies that have emerged in recent years to effectively combat the disease.
A Shift in Treatment
CLL therapies have evolved significantly over time, causing a paradigm shift in treatment. Previously, CLL therapies could be used to improve symptoms but these agents did not frequently induce complete remission or improve survival. With the emergence and evolution of newer drugs, the durations of remission and survival rates have improved considerably. We now have therapies—used alone or in combination—that improve morbidity and even mortality associated with CLL. These therapies, along with the fact that CLL is being diagnosed earlier in asymptomatic patients, have helped change the natural history of the disease, allowing patients to live longer than ever before. We are also better at predicting prognoses for individual patients with CLL based on a variety of clinical, pathologic, and genetic features that improve risk stratification and, in some cases, assist with treatment selection.
It should be noted that any chronic leukemia that recurs after initial chemotherapy will be less responsive to chemotherapy at the time of relapse, highlighting the need for careful consideration of when to initiate therapy and the choice of therapy. An exciting change is that the growing armamentarium of novel treatment options in CLL has enhanced clinicians’ ability to induce and maintain remission.
Tackling Supportive Care Issues
There are a number of supportive care issues among patients with CLL to consider, even among individuals not receiving treatment. These patients have defective immune systems before, during, and after treatment. They have less effective cell-mediated and humoral immunity, putting CLL patients at increased risk for multiple infections. These infectious risks become magnified during and after treatment due to worsened immunosuppression.
There are several actions physicians should consider when providing supportive care. These include treatment and prophylaxis for specific infections as needed, and infusions of IV immunoglobulin in the setting of hypogammaglobulinemia and recurrent infections. Complications such as autoimmune hemolytic anemia or autoimmune thrombocytopenia are also common in CLL, but are treatable when identified. Therapies may be used to increase red blood cell counts and white blood cell counts during therapy in certain instances. The key for clinicians is to monitor all CLL patients closely, especially when therapy is used, to ensure optimal long-term outcomes.
Readings & Resources (click to view)
Leukemia & Lymphoma Society: Chronic Lymphocytic Leukemia. Available at: http://www.leukemia-lymphoma.org/all_page?item_id=7059. Accessed January 21, 2010.
Hallek M, Cheson BD, Catovsky D, et al. Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines. Blood. 2008,111:5446-5456. Available at: http://bloodjournal.hematologylibrary.org/cgi/content/full/111/12/5446. Accessed January 21, 2010.
Ozer H, Armitage JO, Bennett CL, et al. 2000 update of recommendations for the use of hematopoietic colony-stimulating factors: evidence-based, clinical practice guidelines. American Society of Clinical Oncology Growth Factors Expert Panel. J Clin Oncol. 2000;18:3558-3585.
Ludwig H, Rai K, Blade J, et al. Management of disease-related anemia in patients with multiple myeloma or chronic lymphocytic leukemia: epoetin treatment recommendations. Hematol J. 2002;3:121-130.