The Clinical Presentation of CLL
This chapter deals with the basic demographics and the presenting features of CLL and how to assess patients once a diagnosis has been made. CLL is predominantly a disease of the elderly with a preponderance of male patients. New evidence suggests that there are variations in gender incidence according to the clinical status of patients, with a higher male:female ratio in the groups with worse prognosis.
Full blood counts and a physical examination are the basis of the existing staging systems of Rai and Binet. Establishing the patient’s clinical stage sets the scene for the frequency of follow-up, the possible need for therapy and the need for cytogenetic and molecular investigations. Examination of blood films is still important, to identify the presence of prolymphocytes and to consider alternative diagnostic possibilities. In addition, biochemical tests such as beta-2 microglobulin and lactate dehydrogenase are a valuable part of the prognostic evaluation.
Patients may need support to deal with the psychological and quality of life issues arising from their disease.
KeywordsCLL Incidence Clinical presentation Second malignancies Age at diagnosis Gender Rai stage Binet stage
- 1.Howlader N, Noone AM, Krapcho M, Miller D, Bishop K, Altekruse SF, et al., editors. SEER cancer statistics review, 1975–2013. Bethesda: National Cancer Institute; 2016. https://seer.cancer.gov/statfacts/html/clyl.html.
- 2.Lifetime risk estimates calculated by the Statistical Information Team at Cancer Research UK. Based on data provided by the Office of National Statistics, ISD Scotland, the Welsh Cancer Intelligence and Surveillance Unit and the Northern Ireland Cancer Registry, on request. http://www.cancerresearchuk.org/health-professional/cancer-statistics/statistics-by-cancer-type/leukaemia-cll/incidence.
- 10.Al-Sawaf O, Robrecht S, Bahlo J, Fink AM, Cramer P, von Tresckow J, et al. Impact of gender on outcome after chemoimmunotherapy in patients with chronic lymphocytic leukemia: a meta-analysis by the German CLL study group. Leukemia. 2017;31(10):2251–3. https://doi.org/10.1038/leu.2017.221.CrossRefPubMedGoogle Scholar
- 12.Stauder R, Eichhorst B, Hamaker ME, Kaplanov K, Morrison VA, Österborg A, et al. Management of chronic lymphocytic leukemia (CLL) in the elderly: a position paper from an international Society of Geriatric Oncology (SIOG) Task Force. Ann Oncol. 2017;28(2):218–27. https://doi.org/10.1093/annonc/mdw547.CrossRefPubMedGoogle Scholar
- 18.Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Döhner H, et al. International Workshop on Chronic Lymphocytic Leukemia. 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;111:5446–56.CrossRefGoogle Scholar
- 24.Kleinstern G, Rishi A, Achenbach SJ, Chaffee KR, Kay NE, Shanafelt TD, et al. Skin cancers among chronic lymphocytic leukemia (CLL) patients - the effect of UV radiation and CLL clinical characteristics. Blood. 2016;128:4772.Google Scholar
- 26.Autore F, Strati P, Innocenti I, Corrente F, Trentin L, Cortelezzi A, et al. LDH levels predict progression-free survival in treatment-NaÏVe patients with trisomy 12 chronic lymphocytic leukemia. Blood. 2016;128:3211. Abstract.Google Scholar
- 27.Giné E, Martinez A, Villamor N, López-Guillermo A, Camos M, Martinez D, et al. Expanded and highly active proliferation centers identify a histological subtype of chronic lymphocytic leukemia (“accelerated” chronic lymphocytic leukemia) with aggressive clinical behavior. Haematologica. 2010;95:1526–33.CrossRefGoogle Scholar
- 28.Dearden C, Wade R, Else M, Richards S, Milligan D, Hamblin T, et al. The prognostic significance of a positive direct antiglobulin test in chronic lymphocytic leukemia: a beneficial effect of the combination of fludarabine and cyclophosphamide on the incidence of hemolytic anemia. Blood. 2008;111:1820–6.CrossRefGoogle Scholar
- 31.Rai KR. A critical analysis of staging in CLL. In: Gale RP, Rai KR, editors. Chronic lymphocytic leukemia: recent progress and future directions. New York: Alan R. Liss; 1987. p. 253–64.Google Scholar