Review Article

Scientific Advances in the Diagnosis and Risk Stratification of Chronic Lymphocytic Leukemia

Amy Goodrich, MSN, CRNP, and Nina Wagner-Johnston, MD

Johns Hopkins School of Medicine and The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, Maryland

Amy Goodrich, MSN, CRNP, Johns Hopkins School of Medicine, Kimmel Cancer Center, 550 North Broadway, Suite 1003 Baltimore, MD 21205. E-mail: goodram@jhmi.edu


J Adv Pract Oncol 2017;8:17–28 C | https://doi.org/10.6004/jadpro.2017.8.7.14 | © 2017 Harborside™


  

ABSTRACT

Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) are a group of heterogeneous indolent B-cell malignancies that are largely incurable and occur most commonly in older adults. Chronic lymphocytic leukemia is the most prevalent leukemia in the Western world. An incidental finding of lymphocytosis on routine laboratory evaluations or the presence of enlarged lymph nodes are the most common findings that lead to a CLL diagnosis, and up to 50% of newly diagnosed patients will be completely asymptomatic. The diagnosis can be established by peripheral blood flow cytometry with the typical immunophenotype being CD5+, CD23+, CD43+/–, CD10–, CD19+, CD20 dim+, slg dim+, and cyclin D1–. Workup parameters include a comprehensive history and physical exam and extensive laboratory evaluation, at a minimum. Staging by Rai or Binet staging systems utilize a combination of level of lymphocytosis, extent of lymphadenopathy, presence of organomegaly and cytopenias to both stage and project risk status and median survival. Staging parameters, along with immunoglobulin heavy-chain variable region gene (IgHV) mutational status, cytogenetics by florescence in situ hybridization (FISH), beta2-microglobulin, TP53 status, gender, and age at diagnosis are key variables in current prognostic indices, to project 5-year and 10-year overall survival. Response criteria were updated in 2008 to reflect newer prognostic markers, diagnostic parameters and treatment. Advanced practitioners play a critical role in the diagnostic and prognostic workup of patients with CLL, as well as in monitoring changes in prognostic parameters over time. They are also key to educating patients and families on the complexities of this disease at diagnosis and throughout their disease course.




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