Chronic Lymphocytic Leukemia
Best Practices for Routine Follow-up Visits in Chronic Lymphocytic Leukemia Managed Initially by Watch and Wait
Patients with chronic lymphocytic leukemia (CLL) who are treated initially by watch and wait benefit from receiving appropriate vaccinations and cancer screenings. Such patients may also benefit from a more thorough risk stratification at diagnosis, as well as counseling and reassurance to address fears about their disease.
Director, Center for Chronic Lymphocytic Leukemia
“It is important to be aggressive in helping patients with low-risk disease to understand that we usually do not treat immediately after diagnosis, and that we may not treat them for many years.”
There are 2 things that I discuss with all of my newly diagnosed patients with CLL, regardless of age: the importance of receiving all of their vaccinations and the need for routine cancer screenings. I make sure that they are up to date with all of their vaccinations, including the pneumococcal vaccine, PCV13, typically followed by PPSV23 2 months later and again every 5 years. For the prevention of shingles, we also vaccinate with Shingrix (zoster vaccine, recombinant adjuvanted), which is not a live vaccine and is safe in this population. Additionally, cancer screening is very important since patients with CLL are at risk for a second malignancy. I always instruct patients to see their dermatologist if they have not already done so, and we perform all of the other recommended cancer screenings (eg, mammograms, colonoscopies, Pap smears, prostate examinations), even in older patients. I have seen a number of cases of human papillomavirus reactivation in women whose exposure was from 20 or 30 years prior.
Patients who are not being treated also need to understand the validity of watch and wait; it is important to be aggressive in helping patients with low-risk disease to understand that we usually do not treat immediately after diagnosis, and that we may not treat them for many years. Less aggressive approaches can lead to treatment earlier than is needed (eg, based on patient or provider anxiety), which does not improve outcomes, so it is important to adhere to the International Workshop on Chronic Lymphocytic Leukemia criteria.
Providing counseling to help ease a patient’s fear and anxiety is also important. It is helpful for patients to understand and for us to provide reassurance that every patient with CLL experiences nonspecific symptoms on occasion, and that such symptoms that arise in an individual who has been recently diagnosed with CLL are most typically not related to the CLL and that this can remain true even years after diagnosis. A more thorough risk stratification at diagnosis can also help to provide peace of mind to patients, so that they see what low-risk disease really means. It is fine to perform fluorescence in situ hybridization for 17p deletion, but then, if the cytogenetics are more favorable, an IGHV mutation analysis really becomes the most important test for counseling patients, as mutated IGHV can be quite reassuring. The median overall survival for IGHV-mutated patients is in excess of 25 years (based on data from the 1990s), and this is helpful for patients to hear. Risk stratification is also important as relates to establishing an ideal frequency for monitoring and follow-up. In general, I think that less follow-up is indicated for low-risk patients, whom I typically see every 6 months initially, increasing to every 12 months if they are stable. Computed tomography scans and labs are also obtained too frequently; the former is not indicated until the time of treatment, at the earliest.
Flowers CR, Nabhan C, Kay NE, et al. Reasons for initiation of treatment and predictors of response for patients with Rai stage 0/1 chronic lymphocytic leukemia (CLL) receiving first-line therapy: an analysis of the Connect® CLL cohort study. Leuk Lymphoma. 2018;59(10):2327-2335.
Hallek M, Cheson BD, Catovsky D, et al. iwCLL guidelines for diagnosis, indications for treatment, response assessment, and supportive management of CLL. Blood. 2018;131(25):2745-2760.
Nørgaard CH, Søgaard NB, Biccler JL, et al. Limited value of routine follow-up visits in chronic lymphocytic leukemia managed initially by watch and wait: a North Denmark population-based study. PLoS One. 2018;13(12):e0208180.
Visentin A, Facco M, Gurrieri C, et al. Prognostic and predictive effect of IGHV mutational status and load in chronic lymphocytic leukemia: focus on FCR and BR treatments. Clin Lymphoma Myeloma Leuk. 2019 Mar 11. pii: S2152-2650(18)31763-4. doi: 10.1016/j.clml.2019.03.002. [Epub ahead of print]