Abstract
BackgroundChronic lymphocytic leukaemia (CLL) is the most common leukaemia among adults in western countries. Considering the increasing incidence and prevalence of this condition, it is highly relevant to better characterise these patients in Portugal, where data is still scarce.MethodsTo determine incidence, clinical presentation, survival and second malignancies, a population-based historical cohort study was conducted. Cases of interest were identified through the South Region Cancer Registry database and additional data sources. Patients aged ≥18 years, with a confirmed diagnosis of CLL or small lymphocytic lymphoma between January 1st, 2013 and December 31st, 2014 were included. Patients were followed‐up until death or cut-off date (December 31st, 2019).ResultsA total of 496 patients were included and median follow-up time was 5.46 years. Crude incidence rates were 5.03 and 5.22 per 100,000 inhabitants for 2013 and 2014, respectively, and age-adjusted incidence rates were 3.18:100,000 European population for 2013 and 3.35:100,000 European population for 2014. Median age at diagnosis was 71 years and the male/female ratio was 1.40. The majority of patients had leukemic presentation of the disease (86.09%), was diagnosed in Binet stage A (75.58%) and did not present B symptoms (84.01%), anaemia (haemoglobin ≤10g/dL; 90.63%) nor thrombocytopenia (platelet count ≤100 000/μL; 91.73%). Five-year overall survival (OS) rate was 70.53% (95%CI 66.31–74.34) and age, lactate dehydrogenase, Binet stage and a ≥5 Charlson comorbidity index score were independently associated with OS. Standardised-incidence ratios for any second malignancy and cutaneous squamous cell carcinoma were 1.59 (95%CI 1.19–2.08) and 10.15 (95%CI 6.28–15.51), respectively.ConclusionIncidence, clinical presentation and survival of CLL Portuguese patients are similar to those reported for other western countries. The increased risk of second malignancies raises concerns and needs adequate clinical watchfulness.
Highlights
Chronic lymphocytic leukaemia (CLL) is an indolent lymphoproliferative malignancy of CD5+ mature B cells involving peripheral blood, bone marrow and lymphoid organs
Five-year overall survival (OS) rate was 70.53% (95%confidence interval (CI) 66.31– 74.34) and age, lactate dehydrogenase, Binet stage and a 5 Charlson comorbidity index score were independently associated with OS
The diagnosis requires a minimum of 5000 B lymphocytes/μL in the peripheral blood and its clonality needs to be confirmed by flow cytometry [5,6,7]
Summary
Chronic lymphocytic leukaemia (CLL) is an indolent lymphoproliferative malignancy of CD5+ mature B cells involving peripheral blood, bone marrow and lymphoid organs. It is the most common leukaemia among adults in western countries, with incidence rates ranging between 4 and 6 per 100,000 people per year; in contrast, it is rare in Asia, in Japan and Korea. Small lymphocytic lymphoma (SLL) represents a different clinical presentation of the same disease and follows the same management guidelines [3] This entity is characterised by the presence of lymphadenopathy and/or organomegaly with less than 5000 B lymphocytes/μL in the peripheral blood with a CLL phenotype [5,6,7]. Considering the increasing incidence and prevalence of this condition, it is highly relevant to better characterise these patients in Portugal, where data is still scarce
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
